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Sample records for slow-growing craniopharyngioma masquarading

  1. Slow-growing craniopharyngioma masquarading as early-onset eating disorder: Two cases

    DEFF Research Database (Denmark)

    Vad Winkler, Laura; Andersen, Marianne; Hørder, Kirsten

    2009-01-01

    BACKGROUND:: Craniopharyngiomas are slow-growing tumors, which can either be asymptomatic or present themselves with visual, neuropsychiatric or endocrine disturbances. Eating disorders (EDs) are syndromes with unknown etiology, associated with multiple endocrine abnormalities. In pediatric cases...... the possibility of an as yet unidentified structural hypothalamic disorder to be implicated in the etiopathogeny of ED. (c) 2008 by Wiley Periodicals, Inc. Int J Eat Disord, 2009....

  2. Craniopharyngioma

    OpenAIRE

    Grill Jacques; Puget Stéphanie; Garnett Matthew R; Sainte-Rose Christian

    2007-01-01

    Abstract Craniopharyngiomas are benign slow growing tumours that are located within the sellar and para sellar region of the central nervous system. The point prevalence of this tumour is approximately 2/100,000. The onset of symptoms is normally insidious with most patients at diagnosis having neurological (headaches, visual disturbances) and endocrine (growth retardation, delayed puberty) dysfunctions. Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal ...

  3. Craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Grill Jacques

    2007-04-01

    Full Text Available Abstract Craniopharyngiomas are benign slow growing tumours that are located within the sellar and para sellar region of the central nervous system. The point prevalence of this tumour is approximately 2/100,000. The onset of symptoms is normally insidious with most patients at diagnosis having neurological (headaches, visual disturbances and endocrine (growth retardation, delayed puberty dysfunctions. Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch (adamantinomatous type or from metaplasia of squamous epithelial cell rests that are remnants of the part of the stomadeum that contributed to the buccal mucosa (squamous papillary type. The neuroradiological diagnosis is mainly based on the three components of the tumour (cystic, solid and calcified in the characteristic sellar/para sellar location. Definitive diagnosis is made following histological examination of a surgical specimen. The differential diagnosis includes other tumours in this region (pituitary adenoma, infectious or inflammatory processes (eosinophilic granuloma, vascular malformations (aneurysm and congenital anomalies (Rathke's cleft cyst. The current treatment is gross total excision of the tumour, if there is no hypothalamic invasion or, in the presence of hypothalamic invasion, a sub-total resection with post-operative radiotherapy. Endocrine disturbances are normally permanent and need careful replacement. Overall, there is an 80% 5 year survival, though this can be associated with marked morbidity (hypothalamic dysfunction, altered neuropsychological profile.

  4. Craniopharyngioma

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000345.htm Craniopharyngioma To use the sharing features on this page, please enable JavaScript. A craniopharyngioma is a noncancerous (benign) tumor that develops at ...

  5. Craniopharyngioma

    International Nuclear Information System (INIS)

    Lingood, R.M.; Urie, M.M.; Munzenrider, J.E.; Austin-Seymour, M.

    1991-01-01

    This paper compares five patients treated with 10-MV photons with 12 patients treated with a combination of 10-MV photons and 160-MV protons. From 1978 to 1986, 17 patients with craniopharyngioma have ben treated with surgery and radiation therapy. Comparison is made of dose distribution and does volume histograms for these patients treated with different techniques. Four of five patients treated with photons alone are disease free 4-13 years after treatment and one died of recurrent tumor at 6 years. Patients receiving protons have been followed up for 39-90 months; eight have tumor control, and one patient with cystic tumor, which required drainage 4 months after treatment, remains well at 47 months. One patient with substantial residual tumor who received 54 CGE developed bilateral visual loss 5 months later, but this condition improved markedly after reoperation. One patient with a large tumor who received 61.88 CGE had tumor control but severe short-term memory loss and required nursing care

  6. Craniopharyngioma

    International Nuclear Information System (INIS)

    Resch, R.; Haas, H.; Schwarz, S.; Mayr, U.; Twerdy, K.; Huettenberger, H.

    1981-01-01

    Craniopharyngioma, primarily a benign tumour, is associated with a variety of serious problems due to tumour localization. Access to the tumour during surgery is difficult and total resection can rarely be achieved. Growth of the remaining tumour continues and symptoms of increased intracranial pressure, further visual field loss and hormonal disturbances reappear. However, conservative operating in conjunction with radical irradiation shows good results as regards tumour regression and survival with satisfactory quality of life. Irradiation with 50 Gy on average was performed in 5 out of 6 children with remaining tumour or tumour recurrence, and satisfactory results were obtained. In all cases tumour size regressed. Endocrine disturbances could be ascertained by the combined LHRH-TRH-insulin test and the clinical picture. In nearly every case there was finally a complete lack of hormones of the pituitary-hypothalamic axis. Substitution of various hormones became necessary in all cases. Meticulous surveillance of children still undergoing developmental maturation is mandatory. All six children are well and lead a nearly normal life. (orig.) [de

  7. Craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Resch, R.; Haas, H.; Schwarz, S.; Mayr, U.; Twerdy, K.; Huettenberger, H.

    1981-11-06

    Craniopharyngioma, primarily a benign tumour, is associated with a variety of serious problems due to tumour localization. Access to the tumour during surgery is difficult and total resection can rarely be achieved. Growth of the remaining tumour continues and symptoms of increased intracranial pressure, further visual field loss and hormonal disturbances reappear. However, conservative operating in conjunction with radical irradiation shows good results as regards tumour regression and survival with satisfactory quality of life. Irradiation with 50 Gy on average was performed in 5 out of 6 children with remaining tumour or tumour recurrence, and satisfactory results were obtained. In all cases tumour size regressed. Endocrine disturbances could be ascertained by the combined LHRH-TRH-insulin test and the clinical picture. In nearly every case there was finally a complete lack of hormones of the pituitary-hypothalamic axis. Substitution of various hormones became necessary in all cases. Meticulous surveillance of children still undergoing developmental maturation is mandatory. All six children are well and lead a nearly normal life.

  8. Preliminary characterization of slow growing rhizobial strains ...

    African Journals Online (AJOL)

    In this paper, we did some preliminary characterization of six slow growing rhizobial strains, isolated from Retama monosperma (L.) Boiss. root nodules sampled from 3 sites along the coast of Oran (CapeFalcon, Bousfer and MersElHadjadj) in Northwestern Algeria. Results of this study showed that all strains had a very ...

  9. Craniopharyngioma - Transnasal Endoscopic Approach

    Directory of Open Access Journals (Sweden)

    Sanjeev Bhagat,

    2011-01-01

    Full Text Available Craniopharyngiomas are slow growing tumours arising from remnants of the craniopharyngeal duct and occupy the sellar region. The patients may remain asymptomatic for long duration or present with headache or visual disturbances. Surgery is the mainstay of the treatment. Traditionally these tumours have been removed by neurosurgeons through the cranial approach but the advent of nasal endoscopes has opened new avenues for ENT surgeons to treat such patients. We hereby present a case of craniopharyngioma who was successfully treated by Trans-nasal Hypophysectomy.

  10. MR findings of craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sung Moon; Woo, Young Hoon; Joo, Yang Goo; Suh, Soo Jhi [College of Medicine, Keimyung University, Daegu (Korea, Republic of)

    1992-11-15

    Craniopharyngioma is a benign, slow-growing tumor that constitues 3-9% of all intracranial tumors, and arises from epithelial remnants of the Rathke's pouch. We analyzed MR (2.0T) findings of ten cases with surgically proved craniopharyngioma retrospectively. CT was available in five cases, and Gd-DTPA was used in six cases. Characteristic findings of craniopharyngioma in MRI included multilocularity and variable signal intensities within each loculus that were more prominent in T1WI. Detection rate of calcification in MR was 60%. Six cases with Gd-DTPA enhancement revealed irregular or rim-like enhancement. MRI provides useful information regarding the location, extent and biochemical characteristics of the oraniopharyngioma as well as its relationship to the neighboring structures which will be valuable in planning surgical resection.

  11. MR findings of craniopharyngioma

    International Nuclear Information System (INIS)

    Lee, Sung Moon; Woo, Young Hoon; Joo, Yang Goo; Suh, Soo Jhi

    1992-01-01

    Craniopharyngioma is a benign, slow-growing tumor that constitues 3-9% of all intracranial tumors, and arises from epithelial remnants of the Rathke's pouch. We analyzed MR (2.0T) findings of ten cases with surgically proved craniopharyngioma retrospectively. CT was available in five cases, and Gd-DTPA was used in six cases. Characteristic findings of craniopharyngioma in MRI included multilocularity and variable signal intensities within each loculus that were more prominent in T1WI. Detection rate of calcification in MR was 60%. Six cases with Gd-DTPA enhancement revealed irregular or rim-like enhancement. MRI provides useful information regarding the location, extent and biochemical characteristics of the oraniopharyngioma as well as its relationship to the neighboring structures which will be valuable in planning surgical resection

  12. Minichromosome Maintenance Expression Defines Slow-Growing Gastroenteropancreatic Neuroendocrine Neoplasms

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    Simon Schimmack

    2016-10-01

    Full Text Available BACKGROUND: Small intestinal neuroendocrine neoplasm (SI-NEN proliferation is quantified by Ki67 measurements which capture G1-G2M phases of the cell cycle. G0 and early G1 phases, typical of slow-growing cells, can be detected by minichromosome maintenance protein (MCM expression. We hypothesized that these replication licensing markers may provide clinically relevant information to augment Ki67 in low-grade neuroendocrine neoplasia. METHODS: Immunohistochemical staining (IHC, Western blot analysis, quantitative polymerase chain reaction, and copy number variations of MCM2, MCM3, and Ki67 were undertaken in SI-NENs (n = 22. MCM and Ki67 expression was compared by Kaplan-Meier survival analysis (tissue microarray, independent set [n = 55]. Forty-three pancreatic NENs and 14 normal tissues were included as controls. RESULTS: In SI-NENs, MCM2 (mean: 21.2%: range: 16%-25% and MCM3 (28.7%: 22%-34% were detected in significantly more cells than Ki67 (2.3%: 0%-7%, P < .01. MCM2 mRNA correlated with Ki67 IHC (P < .05. MCM3 protein expression was higher in metastases (38-fold than in normal small intestine (P = .06 and was largely absent in normal neuroendocrine cells. There was considerable variation at the MCM copy number level (0-4 copies. MCM3 expression in proliferating cells significantly predicted overall survival (P < .002. Combinations of Ki67 and MCM2/3 in algorithms differentiated low and higher proliferative lesions (overall survival: 12 vs 6.1 years, P = .06. MCM expression was not informative in pancreatic NENs. CONCLUSION: MCMs are expressed in a higher proportion of NEN cells than Ki67 in slow-growing small intestinal lesions and correlate with survival. Assessment can be used to augment Ki67 to improve prognostic classification in these low-grade tumors.

  13. Craniopharyngioma in adults

    Directory of Open Access Journals (Sweden)

    Flavius eZoicas

    2012-03-01

    Full Text Available Craniopharyngiomas are slow growing benign tumors of the sellar and parasellar region with an overall incidence rate of approximately 1.3 per million. During adulthood there is a peak incidence between 40 and 44 years. There are two histopathological types, the adamantinomatous and the papillary type. The later type occurs almost exclusively in adult patients. The presenting symptoms develop over years and display a wide spectrum comprising visual, endocrine, hypothalamic, neurological and neuropsychological manifestations. Currently, the main treatment option consists in surgical excision followed by radiation therapy in case of residual tumor. Whether gross total or partial resection should be preferred has to be balanced on an individual basis considering the extent of the tumor (e.g. hypothalamic invasion. Although the overall long-term survival is good it is often associated with substantial morbidity. Preexisting disorders are often permanent or even exacerbated by treatment. Endocrine disturbances need careful replacement and metabolic sequelae should be effectively treated. Regular follow-up by a multidisciplinary team is a prerequisite for optimal outcome of these patients.

  14. Infrasellar craniopharyngioma

    International Nuclear Information System (INIS)

    Akimura, T.; Kameda, H.; Abiko, S.; Aoki, H.; Kido, T.

    1989-01-01

    A rare case of infrasellar craniopharyngioma mainly occupying the paranasal sinuses, the infratemporal fossa and the skull base is presented. The patient had been treated by an otolaryngologist as a case of mucocele of the sphenoid and ethmoid sinuses because the biopsy specimens obtained at endonasal surgery failed to confirm the true diagnosis. Computed tomography (CT) scan showed multilobulated lowdensity cysts in the paranasal sinuses, infratemporal fossa and skull base, and the cyst wall was enhanced. Magnetic resonance Imaging (MRI) revealed the cysts to have high intensity in both T1- and T2-weighted images, and excellent three-dimensional visualization was obtained. Characteristic motor-oil-like fluid was aspirated during transnasal surgery and the diagnosis of craniopharyngioma was confirmed by histological examination. (orig.)

  15. Infrasellar craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Akimura, T.; Kameda, H.; Abiko, S.; Aoki, H.; Kido, T.

    1989-05-01

    A rare case of infrasellar craniopharyngioma mainly occupying the paranasal sinuses, the infratemporal fossa and the skull base is presented. The patient had been treated by an otolaryngologist as a case of mucocele of the sphenoid and ethmoid sinuses because the biopsy specimens obtained at endonasal surgery failed to confirm the true diagnosis. Computed tomography (CT) scan showed multilobulated lowdensity cysts in the paranasal sinuses, infratemporal fossa and skull base, and the cyst wall was enhanced. Magnetic resonance Imaging (MRI) revealed the cysts to have high intensity in both T1- and T2-weighted images, and excellent three-dimensional visualization was obtained. Characteristic motor-oil-like fluid was aspirated during transnasal surgery and the diagnosis of craniopharyngioma was confirmed by histological examination.

  16. Gene expression profiling of fast- and slow- growing gonadotroph non-functioning pituitary adenomas

    DEFF Research Database (Denmark)

    Falch, Camilla Maria; Sundaram, Arvind Y M; Øystese, Kristin Astrid

    2018-01-01

    Objective Reliable biomarkers associated with aggressiveness of non-functioning gonadotroph adenomas (GAs) are lacking. As the growth of tumor remnants is highly variable, molecular markers for growth potential prediction are necessary. We hypothesized that fast- and slow - growing GAs present......, GPM6A and six EMT-related genes (SPAG9, SKIL, MTDH, HOOK1, CNOT6L and PRKACB). MTDH, but not EMCN, demonstrated involvement in cell migration and association with EMT-markers. Conclusions Fast- and slow- growing GAs present different gene expression profiles and genes related to EMT have higher...... expression in fast-growing tumors. In addition to MTDH, identified as an important contributor to aggressiveness, the other genes might represent markers for tumor growth potential and possible targets for drug therapy. ....

  17. Carcass traits and meat quality of different slow growing and fast growing broiler chickens

    Directory of Open Access Journals (Sweden)

    M. Oblakova

    2017-12-01

    Full Text Available Abstract. The experiment was conducted in the breeder farm of department Population genetics, reproduction and technologies of poultry and rabbits at the Agriculture Institute of Stara Zagora. Five lines from the National Gene Pool of Bulgaria: line Ss (Sussex, line E (Barred Plymouth Rock, line NG (New Hampshire, line F (NG x Red Rhode Island, line L (White Plymouth Rock were used as maternal forms in the crossing schedule and line M (Cornish as a paternal form for production of slow-growing broilers. The birds were grown to 84 days. Feeding was done with compound feeds according to the age: starter (1/14 days of age, grower (14/28 days of age, finisher (28/84 days of age. By the end of the experiment, slaughter analysis was performed with 3 female and 3 male broiler chickens per group with live weight corresponding to the average of each genotype. The live weight was determined, as well as the grill weight, the weights of cuts (breast, thighs, wings, edible offal (heart, liver, gizzard and abdominal fat. On the basis of these data, the slaughter yield and body parts ratios were calculated. The data for the live body weights of birds at slaughtering indicated the highest values for group V – 4040g, followed by groups ІV and ІІ – 3271.67g and 3186.67g, respectively (p<0.05. There was a statistically significant difference between the sexes with superiority of male birds (p<0.001. In the other 3 groups, breast meat percentage ranged from 19.48 to 19.84%. The share of thighs from the grill was the greatest in slow-growing chickens from group II – 33.01%, followed by group І – 32.35%, group IV – 32.18%, and the lowest- in groups ІІІ and V (31.91% and 31.18%, respectively. The analysis of data exhibited a significant effect of the genotype on water content of breast meat (resp. Dry matter, with lower values in slow-growing birds from group III – 73.19% (p<0.05, whereas in the other groups it ranged between 73.44 and 73.62%. The

  18. Cassava starch factory residues in the diet of slow-growing broilers.

    Science.gov (United States)

    Picoli, Karla Paola; Murakami, Alice Eiko; Nunes, Ricardo Vianna; do Amaral Duarte, Cristiane Regina; Eyng, Cinthia; Ospina-Rojas, Ivan Camilo

    2014-12-01

    The objective of this study was to evaluate the effect of inclusion of dehydrated cassava starch residue (DCSR) on the performance, gastrointestinal tract characteristics and carcass traits of ISA Label JA57 slow-growing broilers. A total of 510 broilers at 21 were distributed in a randomized experimental design with 5 treatments (2, 4, 6, 8, and 10 % DCSR inclusion) and a control group, 5 replicates, and 17 birds per experimental unit. The DCSR inclusion from 21 to 49 days of age negatively influenced (P  0.05) feed conversion in the broilers with increasing of DCSR inclusion. From 21 to 79 days, DCSR inclusion impaired (P litter quality with increasing of DCSR inclusion. The level of blood triglycerides showed a quadratic response (P  0.05) the other parameters of carcass quality and yield, cuts, and percentage of abdominal fat. In conclusion, DCSR inclusion levels above 2 % compromised broiler performance.

  19. Phosphorus dynamics and limitation of fast- and slow-growing temperate seaweeds in Oslofjord, Norway

    DEFF Research Database (Denmark)

    Pedersen, Morten Foldager; Borum, Jens; Fotel, Frank Leck

    2010-01-01

    During coastal eutrophication, fast-growing, ephemeral macroalgae bloom at the expense of slow-growing, perennial macroalgae. This change in community composition has been explained by a differential ability to exploit and utilize inorganic nitrogen among macroalgae with different growth strategies......-growing algae (Ulva and Ceramium) took up dissolved inorganic P (DIP) much faster than thicker, slower growing species (belonging to Fucus, Ascophyllum and Laminaria) but also had much higher P-demands per unit biomass and time. DIP concentrations in the Oslofjord were low from April through August, and fast......-growing species were unable to meet their P-demand from uptake for several months during summer. Hence, Ceramium and Ulva were potentially P-limited during summer, whereas Ascophyllum and Laminaria were able to acquire sufficient external DIP to remain P-replete throughout the year. Storage of P prevented Fucus...

  20. Adult intraventricular craniopharyngioma

    International Nuclear Information System (INIS)

    Chin, H.W.

    1983-01-01

    A case of craniopharyngioma with unusual location confined within the third ventricle is reported. The 56 years old adult presented with symptoms and signs of increased intracranial pressure. There were no characteristic findings of craniopharyngioma in X-ray examinations, but computed tomography scan showed a mass lesion in the third ventricle. Literature survey revealed nine cases of craniopharyngioma developed solely within the third ventricle. (orig.) [de

  1. Adult intraventricular craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Chin, H.W.

    1983-04-01

    A case of craniopharyngioma with unusual location confined within the third ventricle is reported. The 56 years old adult presented with symptoms and signs of increased intracranial pressure. There were no characteristic findings of craniopharyngioma in X-ray examinations, but computed tomography scan showed a mass lesion in the third ventricle. Literature survey revealed nine cases of craniopharyngioma developed solely within the third ventricle.

  2. [Respiratory infections caused by slow-growing bacteria: Nocardia, Actinomyces, Rhodococcus].

    Science.gov (United States)

    Eschapasse, E; Hussenet, C; Bergeron, A; Lebeaux, D

    2017-06-01

    Pneumonia caused by slow-growing bacteria is rare but sometimes severe. These infections share many similarities such as several differential diagnoses, difficulties to identify the pathogen, the importance of involving the microbiologist in the diagnostic investigation and the need for prolonged antibiotic treatment. However, major differences distinguish them: Nocardia and Rhodococcus infect mainly immunocompromised patients while actinomycosis also concerns immunocompetent patients; the severity of nocardioses is related to their hematogenous spread while locoregional extension by contiguity makes the gravity of actinomycosis. For these diseases, molecular diagnostic tools are essential, either to obtain a species identification and guide treatment in the case of nocardiosis or to confirm the diagnosis from a biological sample. Treatment of these infections is complex due to: (1) the limited data in the literature; (2) the need for prolonged treatment of several months; (3) the management of toxicities and drug interactions for the treatment of Nocardia and Rhodococcus. Close cooperation between pneumonologists, infectious disease specialists and microbiologists is essential for the management of these patients. Copyright © 2017 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  3. Effect of dietary restriction and hay inclusion in the diet of slow-growing broilers

    Directory of Open Access Journals (Sweden)

    Karla P. Picoli

    2014-11-01

    Full Text Available The objective of this study was to evaluate the effect of dietary restriction and inclusion of alfalfa (Medicago sativa L. and Bermudagrass (Cynodon dactylon cv Coastal hays in the diets of ISA Label JA57 slow-growing male broilers on performance, gastrointestinal tract characteristics, and economic viability. A total of 272 broilers at 21 days old were distributed in a randomized experimental design with four treatments, four replicates, and 17 birds per experimental unit. The treatments consisted of ad libitum concentrated feed (control intake, feed restriction (80% of the control intake, and feed restrictions with supplementation of alfalfa hay (80% of the control intake+20% alfalfa or Bermudagrass hay (80% control intake+20% Bermuda. Dietary restriction, with and without hay inclusion, negatively affected (P<0.05 the weight gain of the birds; however, feed conversion was improved (P<0.05 for animals that underwent only restricted feeding, which also had the best economic indices. Birds subjected to dietary restriction and inclusion of hays showed changes (P<0.05 in the gastrointestinal organs and intestinal morphology.

  4. Cecum lymph node dendritic cells harbor slow-growing bacteria phenotypically tolerant to antibiotic treatment.

    Directory of Open Access Journals (Sweden)

    Patrick Kaiser

    2014-02-01

    Full Text Available In vivo, antibiotics are often much less efficient than ex vivo and relapses can occur. The reasons for poor in vivo activity are still not completely understood. We have studied the fluoroquinolone antibiotic ciprofloxacin in an animal model for complicated Salmonellosis. High-dose ciprofloxacin treatment efficiently reduced pathogen loads in feces and most organs. However, the cecum draining lymph node (cLN, the gut tissue, and the spleen retained surviving bacteria. In cLN, approximately 10%-20% of the bacteria remained viable. These phenotypically tolerant bacteria lodged mostly within CD103⁺CX₃CR1⁻CD11c⁺ dendritic cells, remained genetically susceptible to ciprofloxacin, were sufficient to reinitiate infection after the end of the therapy, and displayed an extremely slow growth rate, as shown by mathematical analysis of infections with mixed inocula and segregative plasmid experiments. The slow growth was sufficient to explain recalcitrance to antibiotics treatment. Therefore, slow-growing antibiotic-tolerant bacteria lodged within dendritic cells can explain poor in vivo antibiotic activity and relapse. Administration of LPS or CpG, known elicitors of innate immune defense, reduced the loads of tolerant bacteria. Thus, manipulating innate immunity may augment the in vivo activity of antibiotics.

  5. Stoichiometry and kinetics of poly-{beta}-hydroxybutyrate metabolism in aerobic, slow growing, activated sludge cultures

    Energy Technology Data Exchange (ETDEWEB)

    Beun, J.J.; Paletta, F.; Loosdrecht, M.C.M. Van; Heijnen, J.J.

    2000-02-20

    This paper discusses the poly-{beta}-hydroxybutyrate (PHB) metabolism in aerobic, slow growing, activated sludge cultures, based on experimental data and on a metabolic model. The dynamic conditions which occur in activated sludge processes were simulated in a 2-L sequencing batch reactor (SBR) by subjecting a mixed microbial population to successive periods of external substrate availability (feast period) and no external substrate availability (famine period). Under these conditions intracellular storage and consumption of PHB was observed. It appeared that in the feast period, 66% to almost 100% of the substrate consumed is used for storage of PHB, the remainder is used for growth and maintenance processes. Furthermore, it appeared that at high sludge retention time (SRT) the growth rate in the feast and famine periods was the same. With decreasing SRT the growth rate in the feast period increased relative to the growth rate in the famine period. Acetate consumption and PHB production in the feast period both proceeded with a zero-order rate in acetate and PHB concentration respectively. PHB consumption in the famine period could best be described kinetically with a nth order degradation equation in PHB concentration. The obtained results are discussed in the context of the general activated sludge models.

  6. Cushing's disease and craniopharyngioma.

    Science.gov (United States)

    Ackland, F M; Stanhope, R; Preece, M A

    1987-01-01

    A 14-year old girl presented with growth failure and Cushing's disease. Histological examination confirmed a craniopharyngioma but failed to show that the tumour secreted adrenocorticotrophic hormone. We suggest that her Cushing's disease was caused by hypothalamic dysfunction associated with increased corticotrophin-releasing hormone secretion, secondary to the craniopharyngioma. Images Figure PMID:2823728

  7. A huge cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Takamura, Seishi; Fukumura, Akinobu; Ito, Yoshihiro; Itoyama, Yoichi; Matsukado, Yasuhiko.

    1986-01-01

    The findings of computed tomography (CT) of a huge cystic craniopharyngioma in a 57-year-old woman are described. Cyst density varied from low to high levels in a short duration. Follow-up CT scans were regarded as important to diagnose craniopharyngioma. The mechanism of increment of cyst density was discussed. (author)

  8. Gas exchange, heat production and oxidation of fat in chicken embryos from a fast or slow growing line

    DEFF Research Database (Denmark)

    Chwalibog, André; Tauson, Anne-Helene; Ali, Abdalla

    2007-01-01

    The experiment comprised 48 chicken (Gallus gallus) embryos from a modern, fast growing line, Ross 308 (RO) and 48 from a slow growing line, Labresse (LA). The O(2) consumption and CO(2) production were measured in an open-air-circuit respiration unit, and heat production (HE) from embryos was ca...

  9. Infrasellar craniopharyngioma: case report

    Directory of Open Access Journals (Sweden)

    Falavigna Asdrubal

    2001-01-01

    Full Text Available We report a case of infrasellar craniopharyngioma in a 34 year-old woman who presented with progressive headache and diplopia. Computed tomographic and magnetic resonance images showed a heterogeneous tumor originating from the sphenoid bone with ethmoid sinus and sella turcica extension. A sublabial rhinoseptal transsphenoidal surgery was performed. Craniopharyngiomas with infrasellar development are very rare. Infrasellar craniopharyngioma is uncommon, thirty-five cases has been reported in literature. The embryology, clinical features and radiographic investigation of these tumors are discussed.

  10. Primary Ectopic Ethmoidal Craniopharyngioma.

    Science.gov (United States)

    Preti, Andrea; Karligkiotis, Apostolos; Facco, Carla; Ottini, Giorgia; Volpi, Luca; Castelnuovo, Paolo

    2017-06-01

    Craniopharyngiomas are benign but aggressive epithelial tumors usually originating in the anterior lobe of the pituitary gland from squamous remnants of an incompletely involuted craniopharingeal duct developing from the Rathke pouch. To the authors' knowledge only 1 patient of a primary isolated ethmoidal craniopharyngioma has been reported in the literature.The authors report the case of a 17-year-old boy with a primary extracranial ethmoidal craniopharyngioma. An endoscopic endonasal approach was employed to resect the tumor. After 2 years of clinical and radiological follow-up no recurrence of disease was observed.Primary ethmoidal craniopharyngiomas are rare entities and biopsy is necessary for diagnosis. However, a preoperative assessment by means of nasal endoscopy, computed tomography scan, and enhanced magnetic resonance imaging is mandatory to better evaluate the extension and characteristics of the tumor. The endoscopic endonasal technique is a safe and effective approach for the treatment of these lesions.

  11. Pituitary stalk craniopharyngioma

    Science.gov (United States)

    Figueiredo, Eberval Gadelha; Welling, Leonardo Christiaan; de Faria, Jose Weber Vieira; Teixeira, Manoel Jacobsen

    2011-01-01

    Craniopharyngiomas are benign but aggressive neoplasms arising along the craniopharyngeal duct. It is frequently located in the suprasellar region. Primarily pituitary stalk craniopharyngioma is unusual and uncommonly early diagnosed, before it enlarges and extends to supra or parasselar region. This unusual location and the small size pose therapeutic dilemmas, since it has the ability to grow larger. Currently, no consensus exists regarding the optimal management. The authors have recommended complete resection. PMID:22715220

  12. Gamma knife surgery for craniopharyngioma

    International Nuclear Information System (INIS)

    Prasad, D.; Steiner, M.; Steiner, L.

    1995-01-01

    We present our results of Gamma Knife surgery for craniopharyngioma in nine patients. The current status of surgery, radiation therapy, intracavitary instillation of radionuclides and Gamma Knife surgery in the management of craniopharyngiomas is discussed. (author)

  13. Primary ectopic frontotemporal extradural craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Reza Pourkhalili

    2016-01-01

    Full Text Available We present a case of primary ectopic frontotemporal extradural craniopharyngioma. Primary ectopic craniopharyngiomas are very rare and have been reported involving the fourth ventricle, infrasellar region, lateral ventricle, temporal area, cerebellopontine angle, clivus, corpus callosum, and prepontine cistern. There was just 1 case of craniopharyngioma previously presented in the literature, with nearly same location as the presenting case.

  14. Transcranial surgery for craniopharyngiomas

    International Nuclear Information System (INIS)

    Shirane, Reizo; Hayashi, Toshiaki; Tominaga, Teiji

    2007-01-01

    The current treatment of craniopharyngiomas is evolving into a multimodal approach in which the aim is disease control and improved preservation of quality of life (QOL). In this the paper, the transcranial removal of craniopharyngiomas is discussed. Fifty-two patients who were surgically treated for craniopharyngiomas extending outside the sellar-suprasellar region were evaluated. All the patients were operated on mainly by the fronto-basal interhemispheric approach. Multiple surgeries were performed in 15 cases. A total of 10 patients were treated with gamma knife radiosurgery (GKS) after surgical removal. In the immediate postoperative period, major complications, including impairment of the perforating arteries were observed in three cases. They exhibited hyperphagia and obesity due to infarction of the hypothalamic nuclei. In our experience, the cost of aggressive resection is hypothalamic dysfunction and a poor QOL. A good QOL may be achieved by careful total or near total resection followed by reoperation or GKS. The fronto-basal interhemispheric approach is a valid choice for the removal of craniopharyngiomas extending outside the sellar-suprasellar region. Using this approach, tumors can be removed without significant sequelae related to surgical technique due to easy preservation of the pituitary stalk, hypothalamic structures, and perforators. This approach offers a safe and minimally invasive means of treating craniopharyngiomas. (author)

  15. Diagnostic imaging of craniopharyngioma

    International Nuclear Information System (INIS)

    Gradzki, J.; Nowak, S.; Paprzycki, W.

    1993-01-01

    40 patients have been examined with operational and histological confirmation of craniopharyngioma. CT image and X-ray plane of skull were performed in case all of these patients. TMR was conformed to examine 4 patients. X-ray planes was compared to CT. CT permits tumor cyst detection. The efficacy of mentioned above diagnostic techniques was compared with surgical findings. (author)

  16. Isolated petrous apex ectopic craniopharyngioma

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    Julius July

    2015-12-01

    Full Text Available Primary ectopic craniopharyngioma is a rare entity. Isolated petrous apex bone location has not been reported previously. This study reports a case of 26-year-old male with right abducent nerve palsy. CT and MRI imaging reveal right petrous apex cystic lesion. No sellar or suprasellar region involvement was found. Endoscopic endonasal transphenoid approach has been successfully performed. Histopathology examination confirms the diagnosis of adamantinomatous craniopharyngioma. So far, it’s probably the first case report of primary ectopic craniopharyngioma isolated in the petrous apex. This case report supports the premise that primary ectopic craniopharyngioma is a multifactorial process that starts with an error from migrated embryological cells.

  17. Congenital craniopharyngioma treated by radical surgery: case report and review of the literature.

    Science.gov (United States)

    Kageji, Teruyoshi; Miyamoto, Takeshi; Kotani, Yumiko; Kaji, Tsuyoshi; Bando, Yoshimi; Mizobuchi, Yoshifumi; Nakajima, Kohei; Nagahiro, Shinji

    2017-02-01

    Craniopharyngiomas are 5-10 % of all pediatric tumors, but are seldomly encountered in the perinatal period. Only seven instances of a truly antenatal diagnosis of a congenital craniopharyngioma that subsequently underwent radical surgery have been reported. We present the case of a patient who received the diagnosis of a suprasellar tumor during the prenatal period and received radical surgery. We report a case of a neonatal craniopharyngioma treated surgically. The pregnancy progressed uneventfully until a routine ultrasound at 37 weeks of gestation showed a 15 × 15 mm high echoic mass in the center of the fetal head. Neonatal Gd-enhanced T1-weighted MRI at 5 days of life showed a homogenously enhanced mass (16×22×15 mm) in the sellar and suprasellar lesion. As the tumor showed rapid growth at the 3rd month of life, the patient underwent a surgical treatment and the mass was totally removed. Three years later, the physical and mental development of the patient was normal, and Gd-MRI studies showed no tumor recurrence. The present case is the eighth case of a truly antenatal diagnosis of a craniopharyngioma that underwent successful radical surgery. Craniopharyngioma is a benign tumor and thought to be a slow growing tumor in childhood. The results of radical surgery were very poor, and the mortality and morbidity rates were high in the previous reports due to the huge size of tumor at operation. The present case demonstrated the rapid growth in short interval of Gd-MRI. This is the first report of tumor kinetics of congenital craniopharyngioma with previous reports. The calculated tumor doubling time in our case was 37 days.

  18. The neuroradiological study of craniopharyngiomas

    International Nuclear Information System (INIS)

    Oikawa, Susumu; Takemae, Toshiki; Kobayashi, Shigeaki

    1992-01-01

    The neuroradiological manifestations of 10 surgically verified craniopharyngiomas are retrospectively studied and compared with those of 8 non-functioning pituitary macroadenomas. The saucer-like appearance of the sella turcica on a craniogram was noted in 2 of the 10 craniopharyngiomas and in 2 of the 8 pituitary adenomas. A ballooning of the sella turcica was observed in one of the 10 craniopharyngiomas and in 5 of the 8 pituitary adenomas. Calcification on CT scan was presented in only 5 craniopharyngiomas. On a plain CT scan, no craniopharyngioma manifested any high-density mass except calcification. Four pituitary adenomas showed a slightly high density, though. A cystic lesion was revealed in all the craniopharyngiomas and in 4 pituitary adenomas. All the craniopharyngiomas except for one intrasellar type and all the pituitary adenomas were enhanced with a contrast medium. The solid portion in one craniopharyngioma and in one pituitary adenoma was high-intensity on T 1 -weighted MRI. The cystic portion in 4 craniopharyngiomas and in 2 pituitary adenomas manifested a high intensity on a T 1 -weighted image. A normal pituitary gland located on the floor of the sella was disclosed in all the craniopharyngiomas on a sagittal T 1 -weighted image; however in all the pituitary adenomas, no normal pituitary gland was found at least not on the floor of the sella. The presence of a normal pituitary gland on the sellar floor on sagittal T 1 -weighted MRI may be a new differential diagnostic point between craniopharyngioma and pituitary macroadenoma. Calcification, cystic formation, and density on a plain CT scan seem to be useful evidence for diagnosis. (author)

  19. Obesity and craniopharyngioma

    Science.gov (United States)

    2011-01-01

    An epidemic of pediatric obesity has occurred across the world in recent years. There are subgroups within the population at high-risk of becoming obese and especially of having experience of precocious cardiovascular and metabolic co-morbidities of obesity. One of these subgroups comprises patients treated for childhood cancers and namely survivors of craniopharyngioma. The high incidence of obesity in this group makes these patients an important disease model to better understand the metabolic disturbances and the mechanisms of weight gain among cancer survivors. The hypothalamic-pituitary axis damage secondary to cancer therapies or to primary tumor location affect long-term outcomes. Nevertheless, the aetiology of obesity in craniopharyngioma is not yet fully understood. The present review has the aim of summarizing the published data and examining the most accepted mechanisms and main predisposing factors related to weight gain in this particular population. PMID:21846381

  20. Obesity and craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Bruzzi Patrizia

    2011-08-01

    Full Text Available Abstract An epidemic of pediatric obesity has occurred across the world in recent years. There are subgroups within the population at high-risk of becoming obese and especially of having experience of precocious cardiovascular and metabolic co-morbidities of obesity. One of these subgroups comprises patients treated for childhood cancers and namely survivors of craniopharyngioma. The high incidence of obesity in this group makes these patients an important disease model to better understand the metabolic disturbances and the mechanisms of weight gain among cancer survivors. The hypothalamic-pituitary axis damage secondary to cancer therapies or to primary tumor location affect long-term outcomes. Nevertheless, the aetiology of obesity in craniopharyngioma is not yet fully understood. The present review has the aim of summarizing the published data and examining the most accepted mechanisms and main predisposing factors related to weight gain in this particular population.

  1. Giant cystic craniopharyngiomas

    International Nuclear Information System (INIS)

    Young, S.C.; Zimmerman, R.A.; Nowell, M.A.; Bilaniuk, L.T.; Hackney, D.B.; Grossman, R.I.; Goldberg, H.I.

    1987-01-01

    Three cases of giant cystic craniopharyngiomas with large areas of extension beyond the suprasellar area are presented. The magnetic resonance (MR) appearance in one case is described. These giant tumors had large, multilobulated cysts that comprised the bulk of the tumors. In one case, there was an unusual extension of the large tumor cyst into the lateral ventricle. In two cases, the tumors extended to the level of the foramen magnum. On CT, the cyst contents of these two tumors were hyperdense and became hypodense postoperatively. All three tumors harbored calcifications in the form of clumps in the suprasellar region and rim calcifications around the cysts. None of the tumors exhibited contrast enhancement. A literature review of the radiographic features of craniopharyngiomas is discussed. (orig.)

  2. Intracavitary therapy of craniopharyngiomas

    International Nuclear Information System (INIS)

    Shapiro, B.; Fig, L. M.; Gross, M.D.; Ann Arbor Nuclear Medicine Service, Ann Arbor, MI

    1999-01-01

    Craniopharyngiomas are benign cystic para-hypophyseal tumors often associated with hypopituitarism and visual-field abnormalities. Their therapy by surgery and external beam radiotherapy is imperfect. The intracavitary instillation of beta-emitting colloid radiopharmaceuticals into the cysts permits the delivery of far higher radiation doses to the cyst lining than is possible by external beam radiotherapy. This technique permits destruction of the lining epithelium with resultant elimination of cyst fluid formation and cyst shrinkage in up to 80% of cases

  3. Radiosurgery for Craniopharyngioma

    International Nuclear Information System (INIS)

    Niranjan, Ajay; Kano, Hideyuki; Mathieu, David; Kondziolka, Douglas; Flickinger, John C.; Lunsford, L. Dade

    2010-01-01

    Purpose: To analyze the outcomes of gamma knife stereotactic radiosurgery (SRS) for residual or recurrent craniopharyngiomas and evaluate the factors that optimized the tumor control rates. Methods and Materials: A total of 46 patients with craniopharyngiomas underwent 51 SRS procedures at University of Pittsburgh between 1988 and 2007. The median tumor volume was 1.0 cm 3 (range, 0.07-8.0). The median prescription dose delivered to the tumor margin was 13.0 Gy (range, 9-20). The median maximal dose was 26.0 Gy (range, 20-50). The mean follow-up time was 62.2 months (range, 12-232). Results: The overall survival rate after SRS was 97.1% at 5 years. The 3- and 5-year progression-free survival rates (solid tumor control) were both 91.6%. The overall local control rate (for both solid tumor and cyst control) was 91%, 81%, and 68% at 1, 3, and 5 years, respectively. No patients with normal pituitary function developed hypopopituitarism after SRS. Two patients developed homonymous hemianopsia owing to tumor progression after SRS. Among the factors examined, complete radiosurgical coverage was a significant favorable prognostic factor. Conclusion: SRS is a safe and effective minimally invasive option for the management of residual or recurrent craniopharyngiomas. Complete radiosurgical coverage of the tumor was associated with better tumor control.

  4. The Importance of Microtopography and Nurse Canopy for Successful Restoration Planting of the Slow-Growing Conifer Pilgerodendron uviferum

    Directory of Open Access Journals (Sweden)

    Jürgen Bauhus

    2013-01-01

    Full Text Available Recent studies have shown that, owing to a lack of seed trees, the natural rate of recovery of fire-disturbed bog forests previously dominated by the endemic and endangered conifer Pilgerodendron uviferum (D. Don Florin is extremely slow. Hence, increasing the number of seed trees in the landscape through restoration planting could remove the principal biotic filter, limiting recovery of these forests. Here, we analyzed how the success of restoration plantings may be improved through the choice or manipulation of microsites in P. uviferum forests on Chiloé Island in North Patagonia. For this purpose, we manipulated microtopography in water-logged sites in bogs (mounds, flat terrain, mineral soil and changed canopy conditions (gaps, semi-open, closed canopy in upland sites with better drainage. In bogs, there was no significant effect of microtopography on growth and survival of P. uviferum plantings. However, fluorescence measurements indicated lower stress in seedlings established on mounds. Seedlings in upland areas established beneath a nurse canopy had lower mortality and higher relative shoot growth, foliar nutrients, photosynthetic light use efficiency and chlorophyll fluorescence values than those planted in the open. This indicates that seedlings of the slow growing P. uviferum can tolerate extremely wet conditions, yet suffer from stress when grown in the open. Here, the removal of canopy appeared to have also removed or reduced mycorrhizal networks for seedlings, leading to poorer nutrition and growth. Based on these results, recommendations for restoration plantings in highly degraded P. uviferum forests are presented.

  5. Edible Giblets and Bone Mineral Characteristics of Two Slow-Growing Chicken Genotypes Reared in an Organic System

    Directory of Open Access Journals (Sweden)

    E Eleroğlu

    Full Text Available ABSTRACT This study was conducted to compare edible giblets weight, tibial bone mineral density (BMD, and bone mineral content (BMC of two slow-growing broiler genotypes (Hubbard S757; S757 and Hubbard Grey Barred JA; GB-JA reared with outdoor access, and to determine the relationship between these variables. Day-old chicks (straight-run of the genotypes S757 (n=120 and GB-JA (n=120 were housed for 98 days. Each genotype was assigned to six pens of 20 birds each. Birds were reared in indoor floor pens and moving shelters with outdoor access (during daylight hours. Absolute body (BW, heart (HW, spleen (SW, liver (LW, gizzard (GW, and abdominal fat pad (AFW weights of the genotype S757 and male birds were statistically higher than that of the genotype GB-JA and female birds. Genotype statistically affected relative HW, whereas sex affected relative GW. Although BMD values were not influenced by genotype or sex, S757 birds and males presented statistically higher tibial BMC, lean, lean+BMC, total mass values (g and area (cm2 compared with GB-JA birds and females. BW, HW, SW, LW, GW and AFW were positively correlated with BMC obtained by DXA. In conclusion, the measured traits influenced by genetic strain and sex. The use of the Hubbard S757 genotype in organic production systems with outdoor access is recommend.

  6. Aggressive surgical management of craniopharyngiomas

    Directory of Open Access Journals (Sweden)

    Manmohan Singh

    2013-01-01

    Full Text Available Surgical treatment of craniopharyngiomas is challenging and despite advancements it continues to pose a challenge. Proponents of subtotal resection in conjunction with radiotherapy argue that this less aggressive approach can yield appropriate results with the lower morbidity. On the contrary, other argument is that gross total resection is superior. Though surgical management of craniopharyngioma is challenging due to its location and important surrounding neurovascular structures, optimal surgical results can be expected following radical surgical excision. Radical excision of craniopharyngiomas is associated with excellent long-term recurrence free survival. Radiation induced long-term complications can be altogether avoided by excising these tumors completely.

  7. Hypothalamic glioma masquerading as craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Sameer Vyas

    2013-01-01

    Full Text Available Hypothalamic glioma account for 10-15% of supratentorial tumors in children. They usually present earlier (first 5 years of age than craniopharyngioma. Hypothalamic glioma poses a diagnostic dilemma with craniopharyngioma and other hypothalamic region tumors, when they present with atypical clinical or imaging patterns. Neuroimaging modalities especially MRI plays a very important role in scrutinizing the lesions in the hypothalamic region. We report a case of a hypothalamic glioma masquerading as a craniopharyngioma on imaging along with brief review of both the tumors.

  8. Interstitial irradiation for craniopharyngioma

    International Nuclear Information System (INIS)

    Barlas, O.; Bayindir, C.; Can, M.

    2000-01-01

    The results of interstitial irradiation treatment for craniopharyngioma in two patients with six year follow-ups are presented. Stereotactic interstitial irradiation with iodine-125 sources as sole therapy was employed in two adult patients who refused surgical resection. The diagnoses were confirmed by stereotactic biopsy. The first tumour which underwent interstitial irradiation was solid and 4 cm in diameter, and the second, 2.7 cm in diameter, had both cystic and solid components. The implanted iodine-125 seeds delivered 67 Gy and 60 Gy to tumour periphery at the rate of 12 and 14 cGy/h, respectively, were removed at the end of designated radiation periods. Tumour shrinkage and central hypo density, first observed 3 months after irradiation, continued until one tumour shrank to less than 1 cm at 12 months, and the other disappeared completely at 24 months. In both cases functional integrity was restored, and neither radiation induced toxicity nor recurrence has occurred six years after treatment. The results in these two cases suggest that solid craniopharyngiomas are sensitive to interstitial irradiation. (author)

  9. Phylogenetic multilocus sequence analysis of indigenous slow-growing rhizobia nodulating cowpea (Vigna unguiculata L.) in Greece.

    Science.gov (United States)

    Tampakaki, Anastasia P; Fotiadis, Christos T; Ntatsi, Georgia; Savvas, Dimitrios

    2017-04-01

    Cowpea (Vigna unguiculata) is a promiscuous grain legume, capable of establishing efficient symbiosis with diverse symbiotic bacteria, mainly slow-growing rhizobial species belonging to the genus Bradyrhizobium. Although much research has been done on cowpea-nodulating bacteria in various countries around the world, little is known about the genetic and symbiotic diversity of indigenous cowpea rhizobia in European soils. In the present study, the genetic and symbiotic diversity of indigenous rhizobia isolated from field-grown cowpea nodules in three geographically different Greek regions were studied. Forty-five authenticated strains were subjected to a polyphasic approach. ERIC-PCR based fingerprinting analysis grouped the isolates into seven groups and representative strains of each group were further analyzed. The analysis of the rrs gene showed that the strains belong to different species of the genus Bradyrhizobium. The analysis of the 16S-23S IGS region showed that the strains from each geographic region were characterized by distinct IGS types which may represent novel phylogenetic lineages, closely related to the type species of Bradyrhizobium pachyrhizi, Bradyrhizobium ferriligni and Bradyrhizobium liaoningense. MLSA analysis of three housekeeping genes (recA, glnII, and gyrB) showed the close relatedness of our strains with B. pachyrhizi PAC48 T and B. liaoningense USDA 3622 T and confirmed that the B. liaoningense-related isolate VUEP21 may constitute a novel species within Bradyrhizobium. Moreover, symbiotic gene phylogenies, based on nodC and nifH genes, showed that the B. pachyrhizi-related isolates belonged to symbiovar vignae, whereas the B. liaoningense-related isolates may represent a novel symbiovar. Copyright © 2017 Elsevier GmbH. All rights reserved.

  10. Endoscopic Endonasal Management of Craniopharyngioma.

    Science.gov (United States)

    Zacharia, Brad E; Amine, Muhamad; Anand, Vijay; Schwartz, Theodore H

    2016-02-01

    Craniopharyngioma is a rare clinical entity that poses a significant management challenge given their location and propensity to recur. As part of a minimally disruptive treatment paradigm, the expanded endonasal approach has the potential to improve rates of resection, improve postoperative visual recovery, and minimize surgical morbidity. This article updates the otolaryngologic community on the basic principles and techniques regarding the incorporation of the endoscopic, endonasal approach in the management paradigm of craniopharyngioma. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Peculiarities of radiation induced craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Sataev, N.M. (Uzbekskij Nauchno-Issledovatel' skij Inst. Onkologii i Radiologii, Tashkent (USSR))

    1982-03-01

    Due to intracranial implantation of a radiosource in rabbit brain craniopharyngioma appeared. Its specific feature is grandular differentiation of embryonal epithelium of residuals of hypophysical (craniopharyngial) passage and the presence of focuses of blood vessel tumor degeneration of hemangioma type in its stroma. It is suggested that radiation craniopharyngioma is developed along the way of epigenetic changes of cellular elements of embryonal epithelium induced by radiation.

  12. Peculiarities of radiation induced craniopharyngioma

    International Nuclear Information System (INIS)

    Sataev, N.M.

    1982-01-01

    Due to intracranial implantation of a radiosource in rabbit brain craniopharyngioma appeared. Its specific feature is grandular differentiation of embryonal epithelium of residuals of hypophysical (craniopharyngial) passage and the presence of focuses of blood vessel tumor degeneration of hemangioma type in its stroma. It is suggested that radiation craniopharyngioma is developed along the way of epigenetic changes of cellular elements of embryonal epithelium induced by radiation

  13. CCT and sonographic findings in congenital craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Helmke, K.; Hausdorf, G.; Moehrs, D.; Laas, R.

    1984-11-01

    In a case of congenital craniopharyngioma results of the sonographic and CCT examinations are reported and compared with postmortem macroscopic cuts. Comparison is made of clinical data from six cases with congenital craniopharyngioma as reported in the literature.

  14. CCT and sonographic findings in congenital craniopharyngioma

    International Nuclear Information System (INIS)

    Helmke, K.; Hausdorf, G.; Moehrs, D.; Laas, R.

    1984-01-01

    In a case of congenital craniopharyngioma results of the sonographic and CCT examinations are reported and compared with postmortem macroscopic cuts. Comparison is made of clinical data from six cases with congenital craniopharyngioma as reported in the literature. (orig.)

  15. Craniopharyngioma: Survivin expression and ultrastructure

    Science.gov (United States)

    ZHU, JIANG; YOU, CHAO

    2015-01-01

    The aim of the present study was to investigate the significance of survivin protein expression levels in craniopharyngioma. Tumor samples and clinical data were obtained from 50 patients with craniopharyngioma who were admitted to the West China Hospital of Sichuan University (Chengdu, China). The morphology of the craniopharyngioma samples was observed using optical and electron microscopes, and survivin expression was investigated in the samples by immunohistochemical analysis. The immunohistochemical results revealed survivin expression in all of the craniopharyngioma samples, but not in the healthy brain tissue samples. It was identified that survivin was expressed at a higher level in cases of the adamantinomatous type compared with those of the squamous-papillary type, in male patients compared with female patients, in children compared with adults and in recurrent cases compared with non-recurrent cases. Furthermore, no significant difference was detected in survivin expression levels among the tumors of different subtypes and different disease stages. The results of the present study indicate that survivin is significant in the development of craniopharyngioma, and that survivin protein expression levels are a meaningful indicator for assessing craniopharyngioma recurrence. PMID:25435936

  16. Stereotactic Neurosurgical Treatment Options for Childhood Craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Michael eTrippel

    2012-05-01

    Full Text Available Craniopharyngioma are the most common non glial tumors in childhood. The results of different studies indicate that radical excision surgery is not an appropriate treatment strategy for childhood craniopharyngioma with hypothalamic involvement. Stereotactic neurosurgery provides save, minimal invasive and cost efficient options in the treatment of childhood craniopharyngioma. In this review a summary of the contribution of the stereotactic neurosurgery in the interdisciplinary treatment regime of childhood craniopharyngioma will be given and discussed in detail.

  17. Motivation and ability to walk for a food reward in fast- and slow-growing broilers to 12 weeks of age

    NARCIS (Netherlands)

    Bokkers, E.A.M.; Koene, P.

    2004-01-01

    Poor physical abilities of broilers may prevent them from performing behaviours for which they are motivated. The aim of this study was to measure the influence of physical ability and motivation on the performance of broilers in short physical tasks. We tested birds from a fast- and a slow-growing

  18. Antenatal diagnosis of the congenital craniopharyngioma

    International Nuclear Information System (INIS)

    Jurkiewicz, E.; Bekiesinska-Figatowska, M.; Duczkowski, M.; Grajkowska, W.; Roszkowski, M.; Czech-Kowalska, J.; Dobrzanska, A.

    2010-01-01

    Background: Craniopharyngioma is a rare fetal and neonatal tumor. Case Report: We report a case of a congenital craniopharyngioma diagnosed by prenatal magnetic resonance. This diagnosis was confirmed by postnatal MR imaging, neurosurgical treatment and histopathological examination. Conclusions: Outcome of neonatal craniopharyngioma is very poor, even if radical surgery is performed. The main problems are pituitary insufficiency, diabetes insipidus, and visual disturbance. (authors)

  19. Effects of dark brooders and overhangs on free-range use and behaviour of slow-growing broilers.

    Science.gov (United States)

    Stadig, L M; Rodenburg, T B; Reubens, B; Ampe, B; Tuyttens, F A M

    2017-12-04

    , these results could not confirm the hypothesis that dark brooders would decrease fearfulness and thereby increase free-range use. Overhangs also did not improve free-range use, and neither brooders nor overhangs had considerable impact on behaviour of chickens outside. Chickens clearly preferred dense natural vegetation over AS and ranged farther in it, indicating that this type of shelter is more suitable for slow-growing free-range broilers.

  20. MRI of craniopharyngiomas

    International Nuclear Information System (INIS)

    Hashimoto, Hiromi; Takemoto, Kazumasa; Shakudo, Miyuki

    1988-01-01

    Magnetic resonance images of 7 patients with surgically confirmed craniopharyngiomas were evaluated and compared with CT scans. Spin echo (SE) images (SE 600 ∼ 1000/40 and SE 1800 ∼ 2500/120) and inversion recovery (IR) images were obtained using a 0.5 T superconductive MR scanner. All cases except one showed hyperintensity on T2-weighted SE images and hypointensity on IR images. Remaining one case had hyperintensity both on T2-weighted SE and IR images. Calcifications were difficult to be detected in the MR images. Coronal and sagittal IR images demonstrated good anatomical relation between the tumor and adjacent brain tissues such as optic chiasm. SE images with a short echo time were useful in delineating tumor contour. (author)

  1. Surgical strategies in childhood craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Jörg eFlitsch

    2011-12-01

    Full Text Available Craniopharyngiomas are biologically benign lesions (WHO Grade 1 of the sellar and suprasellar region, associated with a serious morbidity. About 50% of these tumors become clinically apparent during childhood. Clinical symptoms include headaches, chiasm syndrome, hydrocephalus, pituitary insufficiencies, and obesity. Growth arrest is a typical symptom in children. The treatment of craniopharyngiomas includes surgery as well as radiotherapy. The goal of surgery varies according to the tumor location and extension and may range from complete resection to biopsy. Surgical complications are well known and cause constant evaluation of surgical strategies. Diencephalic obesity is related to surgical manipulation of hypothalamic tissue. Therefore, a classification system for craniopharyngiomas based on preoperative MRI is suggested by the authors, which may help assess the surgical risk. Recurrences are frequent in craniopharyngiomas, even after complete or gross-total resection. Radiotherapy is therefore recommended to patients with incomplete resections. However, the ideal time for radiotherapy after surgery is under discussion.The treatment of craniopharyngiomas requires an interdisciplinary and multimodal approach. Each patient should receive an individually tailored treatment. Surgically, different approaches as well as different degrees of resection can be considered, depending on tumor location and tumor extension.

  2. Effects of ambient temperature and early open-field response on the behaviour, feed intake and growth of fast- and slow-growing broiler strains.

    Science.gov (United States)

    Nielsen, B L

    2012-09-01

    Increased activity improves broiler leg health, but also increases the heat production of the bird. This experiment investigated the effects of early open-field activity and ambient temperature on the growth and feed intake of two strains of broiler chickens. On the basis of the level of activity in an open-field test on day 3 after hatching, fast-growing Ross 208 and slow-growing i657 chickens were allocated on day 13 to one of the 48 groups. Each group included either six active or six passive birds from each strain and the groups were housed in floor-pens littered with wood chips and fitted with two heat lamps. Each group was fed ad libitum and subjected to one of the three temperature treatments: two (HH; 26°C), one (HC; 16°C to 26°C) or no (CC; 16°C) heat lamps turned on. Production and behavioural data were collected every 2 weeks until day 57. For both strains, early open-field activity had no significant effects on their subsequent behaviour or on any of the production parameters measured, and overall, the slow-growing strain was more active than the fast-growing strain. Ambient temperature had significant effects on production measures for i657 broilers, with CC chickens eating and weighing more, and with a less efficient feed conversion than HH chickens, with HC birds intermediate. A similar effect was found for Ross 208 only for feed intake from 27 to 41 days of age. Ross 208 chickens distributed themselves in the pen with a preference for cooler areas in the hottest ambient temperature treatments. In contrast, the behaviour of the slow-growing strain appeared to be relatively unaffected by the ambient temperature. In conclusion, fast-growing broilers use behavioural changes when trying to adapt to warm environments, whereas slow-growing broilers use metabolic changes to adapt to cooler ambient temperatures.

  3. Prognostic factors of craniopharyngioma with special reference to autocrine/paracrine signaling: underestimated implication of growth hormone receptor.

    Science.gov (United States)

    Ogawa, Yoshikazu; Watanabe, Mika; Tominaga, Teiji

    2015-10-01

    Craniopharyngioma is a slow-growing tumor classified as benign, but tight adhesion and significant local infiltration to the vital structures are common. In spite of improvement of modern microsurgery techniques and precise anatomical understanding not few cases of this tumor recur, and long-term tumor control and maintenance of quality of life are sometimes difficult. However, very little is known about the effects of the molecular characters of craniopharyngioma on the prognosis. Ninety eight cases of craniopharyngioma surgically treated at the Department of Neurosurgery, Tohoku University Hospital and Kohnan Hospital from April 1996 to May 2014, 45 males and 53 females aged from 2 to 80 years (mean, 40.84 years) were retrospectively reviewed, and postoperative outcomes and the possible involvement of the autocrine/paracrine mechanism were investigated. The patients were followed up at intervals of 6 months to assess tumor recurrence, and clinical outcomes were correlated with the findings of immunohistochemical examinations used growth hormone receptor (GHR) and downstream hormones. The follow-up period ranged from 3 to 209 months. Hormone expression was examined in 88 patients, of which 46 specimens (52.3 %) showed high expression of GHR. The GHR high expression group had a significantly shorter duration of postoperative stable disease compared with the low expression group (logrank test, p = 0.007). Simultaneous high expression of growth hormone (GH) and GHR was found in 33 specimens (37.5 %), and the high expression group had a significantly shorter duration of postoperative stable disease compared with the low expression group (logrank test, p = 0.011). No other hormones showed statistically significant differences in outcomes. High expression of GHR is associated with shorter duration of postoperative stable disease in patients with craniopharyngioma. If the surgical specimens were craniopharyngiomas with high GHR expression, GH supplementation

  4. Very long-term sequelae of craniopharyngioma.

    Science.gov (United States)

    Wijnen, Mark; van den Heuvel-Eibrink, Marry M; Janssen, Joseph A M J L; Catsman-Berrevoets, Coriene E; Michiels, Erna M C; van Veelen-Vincent, Marie-Lise C; Dallenga, Alof H G; van den Berge, J Herbert; van Rij, Carolien M; van der Lely, Aart-Jan; Neggers, Sebastian J C M M

    2017-06-01

    Studies investigating long-term health conditions in patients with craniopharyngioma are limited by short follow-up durations and generally do not compare long-term health effects according to initial craniopharyngioma treatment approach. In addition, studies comparing long-term health conditions between patients with childhood- and adult-onset craniopharyngioma report conflicting results. The objective of this study was to analyse a full spectrum of long-term health effects in patients with craniopharyngioma according to initial treatment approach and age group at craniopharyngioma presentation. Cross-sectional study based on retrospective data. We studied a single-centre cohort of 128 patients with craniopharyngioma treated from 1980 onwards (63 patients with childhood-onset disease). Median follow-up since craniopharyngioma presentation was 13 years (interquartile range: 5-23 years). Initial craniopharyngioma treatment approaches included gross total resection ( n  = 25), subtotal resection without radiotherapy ( n  = 44), subtotal resection with radiotherapy ( n  = 25), cyst aspiration without radiotherapy ( n  = 8), and 90 Yttrium brachytherapy ( n  = 21). Pituitary hormone deficiencies (98%), visual disturbances (75%) and obesity (56%) were the most common long-term health conditions observed. Different initial craniopharyngioma treatment approaches resulted in similar long-term health effects. Patients with childhood-onset craniopharyngioma experienced significantly more growth hormone deficiency, diabetes insipidus, panhypopituitarism, morbid obesity, epilepsy and psychiatric conditions compared with patients with adult-onset disease. Recurrence-/progression-free survival was significantly lower after initial craniopharyngioma treatment with cyst aspiration compared with other therapeutic approaches. Survival was similar between patients with childhood- and adult-onset craniopharyngioma. Long-term health conditions were comparable after

  5. Treatment Strategies in Childhood Craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Stephanie ePuget

    2012-06-01

    Full Text Available The surgical management of craniopharyngioma in children has been one of the most controversial topics in pediatric neurosurgery. In theory, based on its benign histology total surgical excision could provide a cure. However, the therapeutic goals for pediatric craniopharyngioma are not only the cure of the disease but also the preservation of function. It has been widely established that in some particular cases total excision could leads to inacceptable damages, especially those linked to hypothalamic functions. During the last 15 years, we observed worldwide a growing advocacy for less-invasive pediatric craniopharyngioma resection supported by international consensus conferences. The state-of-the-art in the surgical management of some craniopharyngioma is now turning to multi-modality treatment strategies (combination surgery and radiotherapy aiming to limit morbidiy. Recent literature and our own experience helped to develop risk-adapted treatment strategies at initial diagnosis, respecting hypothalamic structures to provide optimal quality of life for these children. Following new algorithms of treatment, preliminary results with intention to spare the hypothalamus seem to be encouraging but the long-term clinical outcome in terms of post irradiation complications and relapse management is currently unknown.

  6. CURRENT PRINCIPLES FOR CRANIOPHARYNGIOMA TREATMENT

    Directory of Open Access Journals (Sweden)

    A. N. Konovalov

    2012-01-01

    Full Text Available The paper describes the classification and treatment options of craniopharyngiomas, benign epithelial tumors arising from the cell remains of the Rathke’s pouch. It presents a few types of surgical accesses during surgical treatment for this disease and gives examples of how to place an Ommaya reservoir. 

  7. Craniopharyngioma: How to deal with?

    Directory of Open Access Journals (Sweden)

    Pintea Bianca St.

    2014-03-01

    Full Text Available Craniopharyngiomas are rare, highly complex tumors with bimodal incidence in the pediatric and adult age groups. In our opinion, depending on the means possible, total microscopic ablation offers the best chance of healing, or at least prolongs the time interval of recurrences.

  8. Establishment of primary cultures of craniopharyngioma cells★

    Science.gov (United States)

    Liu, Hao; Liu, Liang; Liu, Zhiyong; Li, Qiang; You, Chao; Xu, Jianguo

    2012-01-01

    Craniopharynigoma samples were collected from 36 patients. Out of the 36 samples, 29 achieved successful sub-culturing, with a success rate of 80.6%. Immunohistochemistry staining showed that cytokeratin-7 was positively expressed in the cytomembrane and cytoplasm of craniopharyngioma cells at 6-8 passages, confirming that all cultured cells were squamous epithelial cells. The doubling time of craniopharyngioma cells was 3 days, as confirmed by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. In this study, craniopharyngioma cells cultured in vitro were established; however, establishment of immortalized craniopharyngioma cell lines requires further research. PMID:25745451

  9. Lipoproteins of slow-growing Mycobacteria carry three fatty acids and are N-acylated by apolipoprotein N-acyltransferase BCG_2070c.

    Science.gov (United States)

    Brülle, Juliane K; Tschumi, Andreas; Sander, Peter

    2013-10-05

    Lipoproteins are virulence factors of Mycobacterium tuberculosis. Bacterial lipoproteins are modified by the consecutive action of preprolipoprotein diacylglyceryl transferase (Lgt), prolipoprotein signal peptidase (LspA) and apolipoprotein N- acyltransferase (Lnt) leading to the formation of mature triacylated lipoproteins. Lnt homologues are found in Gram-negative and high GC-rich Gram-positive, but not in low GC-rich Gram-positive bacteria, although N-acylation is observed. In fast-growing Mycobacterium smegmatis, the molecular structure of the lipid modification of lipoproteins was resolved recently as a diacylglyceryl residue carrying ester-bound palmitic acid and ester-bound tuberculostearic acid and an additional amide-bound palmitic acid. We exploit the vaccine strain Mycobacterium bovis BCG as model organism to investigate lipoprotein modifications in slow-growing mycobacteria. Using Escherichia coli Lnt as a query in BLASTp search, we identified BCG_2070c and BCG_2279c as putative lnt genes in M. bovis BCG. Lipoproteins LprF, LpqH, LpqL and LppX were expressed in M. bovis BCG and BCG_2070c lnt knock-out mutant and lipid modifications were analyzed at molecular level by matrix-assisted laser desorption ionization time-of-flight/time-of-flight analysis. Lipoprotein N-acylation was observed in wildtype but not in BCG_2070c mutants. Lipoprotein N- acylation with palmitoyl and tuberculostearyl residues was observed. Lipoproteins are triacylated in slow-growing mycobacteria. BCG_2070c encodes a functional Lnt in M. bovis BCG. We identified mycobacteria-specific tuberculostearic acid as further substrate for N-acylation in slow-growing mycobacteria.

  10. Effect of a free-range raising system on growth performance, carcass yield, and meat quality of slow-growing chicken.

    Science.gov (United States)

    Wang, K H; Shi, S R; Dou, T C; Sun, H J

    2009-10-01

    Experiments were conducted to evaluate the effect of free-range raising systems on growth performance, carcass yield, and meat quality of slow-growing chickens. Slow-growing female chickens, Gushi chickens, were selected as the experimental birds. Two hundred 1-d-old female chicks were raised in a pen for 35 d. On d 36, ninety healthy birds, with similar BW (353.7+/-32.1g), were selected and randomly assigned to 2 treatments (indoor treatment and free-range treatment, P>0.05). Each treatment was represented by 3 groups containing 15 birds (45 birds per treatment). During the indoor treatment, the chickens were raised in floor pens in a conventional poultry research house (7 birds/m2). In the free-range treatment, the chickens were housed in a similar indoor house (7 birds/m2); in addition, they also had a free-range grass paddock (1 bird/m2). All birds were provided with the same starter and finisher diets and were raised for 112 d. Results showed that the BW and weight gain of the chickens in the free-range treatment were much lower than that of the chickens in the indoor floor treatments (Pfree-range raising system on eviscerated carcass, breast, thigh, and wing yield (P>0.05). However, the abdominal fat yield and tibia strength (P0.05) by the free-range raising system. The data indicated that the free-range raising system could significantly reduce growth performance, abdominal fat, and tibia strength, but with no effect on carcass traits and meat quality in slow-growing chickens.

  11. Polysomnographic findings in craniopharyngioma patients

    DEFF Research Database (Denmark)

    Pickering, Line; Klose, Marianne; Feldt-Rasmussen, Ulla

    2017-01-01

    of the same age (p = 0.09). No subjects had symptoms of hypnagogic hallucinations, sleep paralyses, or cataplexies. Four patients and one control had periodic leg movements (PLMs). One patient had fragmented sleep pattern, rapid eye movement (REM) sleep without atonia, and PLMs. One patient had short sleep...... periods during the daytime. Four patients had fragmented sleep pattern. With the MSLT, four patients and two controls had mean sleep latency of REM sleep in 2/5 and 3/5 sleep periods, respectively. All subjects showed normal hypocretin-1 levels......PURPOSE: The purpose of this study is to evaluate whether damage to the hypothalamus due to craniopharyngioma or consequent surgery may involve the sleep-wake regulatory system, resulting in sleep disturbances and sleepiness. METHODS: Seven craniopharyngioma patients and 10 healthy controls were...

  12. Craniopharyngioma with hyperprolactinaemia due to a prolactinoma.

    Science.gov (United States)

    Wheatley, T; Clark, J D; Stewart, S

    1986-01-01

    A case is presented in which a histologically proven prolactin secreting pituitary macroadenoma was associated with a large suprasellar craniopharyngioma. The pre-operative prolactin concentration was 8180 mU/l. Although hyperprolactinaemia up to 3000 mU/l in patients with a craniopharyngioma is usually due to stalk compression, greater values may indicate an associated prolactinoma. Images PMID:3794737

  13. P16.29 Malignant craniopharyngioma

    Science.gov (United States)

    Unal, E.; Kilic, K.; Ozdemir, N.; Gunver, F.; Isik, S.; Can, S.

    2017-01-01

    Abstract Introduction: Malignant transformation of craniopharyngioma has rarely been described. In this article, we report a case of 28th malignant craniopharyngioma ever mentioned in English literature. Materials and Methods: We performed a PUBMED, HUBMED, BAU Library Database and Ovid search on malignant craniopharyngiomas and identified 27 reported cases. CASE DESCRIPTION: 44 years old female patient was diagnosed with craniopharyngioma two years ago and underwent surgical resection of a typical craniopharyngioma, the histopathological result was adamantinomatous craniopharyngioma of Grade I. There was no malignancy. One year ago cavernous sinus invasion has been detected and gamma knife irradiation has been made. At admission she was blind in the right eye for the last six months and the vision was diminished in the left eye for a month. The MRI showed that nasal cavity was full of tumor, that the clivus was almost completely destructed and that orbita and maxillary sinus were also invaded. Firstly the ENT surgeons debulked the tumor via transmaxillary route and then the transcranial approach allowed only a subtotal removal due to a profuse bleeding. The histopatological examination showed malignant tumoral infiltration rich in cells with many mitoses. The patient died two years later. CONCLUSION: The relevant literature of malignant craniopharyngioma is reviewed and discussed. The surgeon must be aware that total removal of a malignant craniopharyngioma can be hazardous because of intractable bleedings occurring during surgery.

  14. Internal irradiation for cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Kobayashi, Tatsuya; Kageyama, Naoki

    1979-01-01

    Internal irradiation with P-32 chromic phosphate and Au-198 colloid was used to treat cystic craniopharyngioma. A newly developed dosimetric formula, by which the radiation dose can be calculated simultaneously at the cyst wall and at a point far from the radioactive source and the untoward effect of irradiation on surrounding brain tissue can be eliminated, especially in cases in which the wall is thin and can be penetrated by beta emission, was used. Radioactive phosphate or gold was injected into eight craniopharyngioma cysts throught the Ommaya reservoir and a tube inserted at the first craniotomy. All cysts were effectively treated for 3 to 33 months, to eliminate fluid retention or collapse. A collapsed cyst was removed at the second craniotomy and irradiation was histologically shown to be effective. Oculomotor palsy, a side effect of irradiation, occurred 10 days after the injection of 5 mc of P-32 chromic phosphate only in a case of small cysts (5.0 ml) in the supra- and intracellular regions. The thickness of the cyst wall was less than 0.5 mm and the oculomotor nerves were thought to adhere to the wall. Not only the amount of wall dose but also the thickness of the wall and localization of the cyst are important factors in internal irradiation. Sufficient and safer doses which kill tumor cells in the wall and have no side effects, are 9,000 to 30,000 rad. Internal irradiation can be used to treat large cysts of more than 10 ml which are supposedly difficult to remove radically and or multiple cysts. It is effective not only for cystic craniopharyngioma but also for intracrania cystic tumors other than craniopharyngioma, if dosimetry is accurate. (J.P.N.)

  15. Fractionated stereotactic radiotherapy for craniopharyngiomas

    International Nuclear Information System (INIS)

    Schulz-Ertner, Daniela; Frank, Claudia; Herfarth, Klaus K.; Rhein, Bernhard; Wannenmacher, Michael; Debus, Juergen

    2002-01-01

    Purpose: To investigate outcome and toxicity after fractionated stereotactic radiation therapy (FSRT) in patients with craniopharyngiomas. Methods and Materials: Twenty-six patients with craniopharyngiomas were treated with FSRT between May 1989 and February 2001. Median age was 33.5 years (range: 5-57 years). Nine patients received FSRT after surgery as primary treatment, and 17 patients were irradiated for recurrent tumor or progressive growth after initial surgery. Median target dose was 52.2 Gy (range: 50.0-57.6 Gy) with conventional fractionation. Follow-up included MRI and neurologic, ophthalmologic, and endocrinologic examinations. Results: The median follow-up was 43 months (range: 7-143 months). The actuarial local control rate and actuarial overall survival rates were 100% and 100%, respectively, at 5 years and 100% and 83%, respectively, at 10 years. Four patients showed complete response, 14 patients showed partial response, and 8 patients remained stable. In 5 patients, vision improved after radiation therapy. Acute toxicity was mild. One patient required cyst drainage 3 months after radiotherapy. Late toxicity after radiotherapy included impairment of hormone function in 3 out of 18 patients at risk. We did not observe any vision impairment, radionecrosis, or secondary malignancies. Conclusions: FSRT is effective and safe in the treatment of cystic craniopharyngiomas. Toxicity is extremely low using this conformal technique

  16. Polysomnographic findings in craniopharyngioma patients.

    Science.gov (United States)

    Pickering, Line; Klose, Marianne; Feldt-Rasmussen, Ulla; Jennum, Poul

    2017-12-01

    The purpose of this study is to evaluate whether damage to the hypothalamus due to craniopharyngioma or consequent surgery may involve the sleep-wake regulatory system, resulting in sleep disturbances and sleepiness. Seven craniopharyngioma patients and 10 healthy controls were evaluated with sleep questionnaires including the Epworth Sleepiness Scale, polysomnography, and a multiple sleep latency test (MSLT). Five patients and eight controls had lumbar puncture performed to determine hypocretin-1 levels. Patients tended to feel sleepier than control individuals of the same age (p = 0.09). No subjects had symptoms of hypnagogic hallucinations, sleep paralyses, or cataplexies. Four patients and one control had periodic leg movements (PLMs). One patient had fragmented sleep pattern, rapid eye movement (REM) sleep without atonia, and PLMs. One patient had short sleep periods during the daytime. Four patients had fragmented sleep pattern. With the MSLT, four patients and two controls had mean sleep latency of < 8 min. One patient and three controls had sudden onset of REM sleep in 2/5 and 3/5 sleep periods, respectively. All subjects showed normal hypocretin-1 levels. Four patients had electrophysiological findings indicative of central hypersomnia including one patient meeting the criteria of narcolepsy. The sleep-wake regulatory system may be involved in craniopharyngioma patients.

  17. Host gut-derived probiotic bacteria promote hypertrophic muscle progression and upregulate growth-related gene expression of slow-growing Malaysian Mahseer Tor tambroides

    Directory of Open Access Journals (Sweden)

    Md Asaduzzaman

    2018-02-01

    Full Text Available In modern aquaculture, dietary supplementation of probiotics is a novel approach for enhancing growth performance of slow-growing fish. However, the actual role of probiotics in regulating muscle growth at cellular and molecular levels in fish still needs to be clarified. In this study, we hypothesized that host gut derived probiotic bacteria would enhance cellular muscle growth, and upregulate growth-related gene expression in slow-growing Malaysian mahseer Tor tambroides. Therefore, three host-associated probiotics (Bacillus sp. AHG22, Alcaligenes sp. AFG22, and Shewanella sp. AFG21 were isolated from the gastro-intestinal tract of T. tambroides and screened based on their digestive enzyme activity. A fishmeal and casein based control diet (40% crude protein and 10% lipid was formulated, and three different probiotic supplemented diets were prepared by immersing the control diet in each isolated host-derived bacteria, suspended in sterile phosphate buffered saline (PBS, to achieve a final concentration of approximately 1.0 × 108 CFU g−1 feed. Triplicate groups of T. tambroides juveniles (initial weight 1.39 ± 0.06 g were stocked in twelve glass aquaria (100 L capacity with stocking density of 20 individuals per aquarium. The feed was applied twice daily at 3.0% of the fish body weight per day for 90 days. Growth performance (weight gain and specific growth rate of T. tambroides juveniles were significantly higher in Alcaligenes sp. AFG22 and Bacillus sp. AHG22 supplemented diet treatments. Muscle morphometric analysis revealed that dietary supplementation of host-associated probiotic bacteria did not influence the frequency distribution of hyperplastic (class 10 small diameter fibers (≤10 μm. However, hypertrophic (Class 50, Class 60 and Class 70 large diameter fibers (>50 μm were significantly higher in Alcaligenes sp. AFG22 and Bacillus sp. AHG22 supplemented treatments, indicating that increased growth rate of T

  18. XF-70 and XF-73, novel antibacterial agents active against slow-growing and non-dividing cultures of Staphylococcus aureus including biofilms.

    Science.gov (United States)

    Ooi, Nicola; Miller, Keith; Randall, Christopher; Rhys-Williams, William; Love, William; Chopra, Ian

    2010-01-01

    Slow-growing and non-dividing bacteria exhibit tolerance to many antibiotics. However, membrane-active agents may act against bacteria in all growth phases. We sought to examine whether the novel porphyrin antibacterial agents XF-70 and XF-73, which have rapid membrane-perturbing activity against Staphylococcus aureus, retained antistaphylococcal activity against growth-attenuated cells. The killing kinetics of XF-70, XF-73 and various comparator agents against exponential phase cultures of S. aureus SH1000 were compared with effects on cells held at 4 degrees C, non-growing cultures expressing the stringent response induced by mupirocin and bacteria in the stationary phase. Biofilms of S. aureus SH1000 were generated with the Calgary device to examine the activities of XF-70 and XF-73 under a further system exhibiting diminished bacterial growth. Cold culture, stringent response and stationary phase cultures remained susceptible to XF-70 and XF-73, which caused > or =5 log reductions in viability over 2 h. During this period the most active comparator agents (chlorhexidine and cetyltrimethylammonium bromide) only promoted a 3 log drop in viability. XF-70 and XF-73 were also highly active against biofilms, with both agents exhibiting low biofilm MICs (1 mg/L) and minimum biofilm eradication concentrations (2 mg/L). XF-70 and XF-73 remained highly active against various forms of slow-growing or non-dividing S. aureus. The results support the hypothesis that membrane-active agents may be particularly effective in eradicating slow- or non-growing bacteria and suggest that XF-70 and XF-73 could be utilized to treat staphylococcal infections where the organisms are only dividing slowly, such as biofilm-associated infections of prosthetic devices.

  19. Present-day radiodiagnosis of craniopharyngiomas

    International Nuclear Information System (INIS)

    Konovalov, A.N.; Ozerova, V.I.; Kovnienko, V.K.; Krasnova, T.S.; Shten'o, Yu.

    1983-01-01

    A total of 108 patients with craniopharyngiomas were examined with the help of computerized tomography, craniography, angiography; in some cases the liquor system of the brain was studied. The data obtained were intercorrelated permitting one to single out main X-ray signs of craniopharyngiomas of the endosupracellUlar, intraventricular and stalk types. At present CT is a method of patients' examiration pepmitting one to reveal with utmost precision the size and structure of tumors, their correlation with cerebral structures. CT makes it possible to follow-up patients with craniopharyngiomas over time in the early and late postoperative periods

  20. Depressive disorder due to craniopharyngioma.

    Science.gov (United States)

    Spence, S A; Taylor, D G; Hirsch, S R

    1995-01-01

    Secondary causes of depression are legion, and must always be considered in patients presenting with features atypical of primary idiopathic depressive disorder. The case described is that of a middle-aged woman presenting initially with a major depressive disorder who was subsequently found to have a craniopharyngioma, leading to a revised diagnosis of mood disorder due to the tumour. Some features of the presentation might have led to earlier diagnosis had their localizing significance been recognized. Diencephalic lesions should always be considered in patients presenting with the hypersomnic-hyperphagic variant of depressive disorder. Images Figure 1 PMID:8544149

  1. A rare presentation of craniopharyngioma: delayed puberty

    Science.gov (United States)

    İnci, Mehmet Fatih; Özkan, Fuat; Bozkurt, Selim; Demir, Caner Feyzi

    2012-01-01

    Craniopharyngiomas are the most frequently encountered suprasellar tumours in children. Owing to the slow growth rate of these tumours, they are often quite large before becoming symptomatic. They are more common among children and older adults (55–74 years). Depending upon the direction of growth and tumour size, craniopharyngiomas can affect the hypothalamus, pituitary stalk, optic nerves and chiasm and carotid arteries. Compression of these neural and vascular structures frequently precipitates endocrine disorders, visual loss and an increased intracranial pressure. Hypopituitarism leading to a delayed puberty is a rare presentation of craniopharyngioma. The diagnosis of craniopharyngioma is usually made with the classic radiological imaging features based on CT and MRI. PMID:23195827

  2. Craniopharyngioma: a clinicopathological study of 141 cases.

    Science.gov (United States)

    Tavangar, Seyed Mohammad; Larijani, Bagher; Mahta, Ali; Hosseini, Seyed Mehdi Abdolahzadeh; Mehrazine, Masoud; Bandarian, Fatemeh

    2004-01-01

    Craniopharyngioma is a tumor of the suprasellar region that histologically has two distinct variants with some differences in clinical behavior. The papillary type is almost always seen in adults and has a more indolent course compared with the adamantinomatous type, which is more common in childhood. In the present study, surgical specimens of craniopharyngiomas from 141 patients were reviewed. Their histomorphologic types were determined and the clinical features and prognosis of each group were assessed. The sizes of papillary type tumors were smaller and during the follow-up period there was no recurrence in the squamous papillary group. Aside from surgical resection (total vs subtotal), the recurrence rate for papillary type craniopharyngioma was lower than for adamantinomatous type. Histologic typing of craniopharyngioma especially in adults is useful for decision making with regard to treatment and follow-up.

  3. Internal irradiation for cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Kobayashi, T.; Kageyama, N.; Ohara, K.

    1981-01-01

    The authors report the results of internal irradiation with labeled chromic phosphate (32P) and gold-198 (198Au) colloid in eight cases of cystic craniopharyngiomas. They used a newly developed dosimetric formula, by which the radiation dose at the cyst wall and at any point far from the radioactive source can be calculated. Ten courses of irradiation in eight patients were carried out by injection of either 32P or 198Au colloid into the cyst through an Ommaya drainage system that had been placed at craniotomy. Follow-up studies ranging from 13 to 156 months revealed that all cysts were effectively treated, with elimination of fluid or collapse of the cyst. This was confirmed by Conray cystography and/or computerized tomography. Not only the dose delivered to the wall but also the thickness of the cyst wall and the location of the cyst are important factors in planning internal irradiation. A safe and adequate dose to the cyst wall could range between 9000 to 30,000 rads for craniopharyngioma. This treatment is suitable for large cysts that are thought to be difficult to remove radically, recurrent cysts resistant to previous treatment, or multiple cysts. Internal irradiation may also be applicable in other cystic intracranial tumors if dosimetry is calculated accurately

  4. Intracystic Therapies for Cystic Craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Ute Katharina Bartels

    2012-03-01

    Full Text Available AbstractIntroduction: Craniopharyngioma of childhood are commonly cystic in nature. An intracystic catheter insertion and subsequent instillation of substances inducing cyst shrinkage seems a beneficial strategy avoiding additional morbidity in a highly vulnerable brain location. Methods: A systematic review of the medical literature was performed to identify potentially relevant, all languages articles using Ovid MEDLINE and EMBASE from inception to July 2011 and Cochrane Central Register of Controlled Trials to 3rd quarter 2011. All references were examined for relevancy. Results: Of 142 unique references x referred to substances used for intracystic craniopharyngioma treatment. General aspects of intracystic catheter insertion as well as response rates, risks and outcomes of children treated with intracystic radio-isotopes, bleomycin and interferon are critically reviewed and an outline for potential future endeavours provided. Conclusions: Interferon seems currently the intracystic substance with the best benefit risk ratio. The authors advocate for consensus on prospective data collection and standardized intracystic treatment strategies to allow reliable comparisons and herewith optimize treatment and outcome.

  5. Energy expenditure in obesity associated with craniopharyngioma

    Science.gov (United States)

    Shah, Rachana; Tershakovec, Andy M.; Zemel, Babette S.; Sutton, Leslie N.; Grimberg, Adda; Moshang, Thomas

    2010-01-01

    Background and purpose Obesity is a common yet incompletely understood complication of childhood craniopharyngioma. We hypothesized that craniopharyngioma is associated with specific defects in energy balance compared to obese control children. Methods Eleven craniopharyngioma patients were recruited for a study on body composition and energy balance. Eight subjects were obese. The obese craniopharyngioma patients had a mean age (±SD) of 11.2±1.7 years. The average body mass index z score was 2.33 (±0.32). A previously studied group of obese children (BMI z score 2.46±0.46) served as controls. Resting energy expenditure (REE) was determined by indirect calorimetry and body composition by dual energy X-ray absorptiometry in all children. Results Obese craniopharyngioma patient subjects had increased mean (±standard error) fat-free mass compared to obese controls (57%±0.88 % vs 50.0%±0.87%, p=0.02). The obese craniopharyngioma patients had a 17% lower REE compared to values expected from the World Health Organization equation (1,541±112.6 vs 1,809±151.8 kcal; p=0.01). In contrast, the obese control children had measured REE within 1% of predicted (1,647±33.2 vs. 1,652±40.2; p=0.8). In a linear regression model, REE remained significantly lower than predicted after controlling for FFM. Conclusions Lower REE may be a factor contributing to obesity in children with craniopharyngioma. Further study is needed into the mechanisms for reduced energy expenditure in patients with craniopharyngioma. PMID:20107994

  6. Malignant astrocytoma following radiotherapy for craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Menon G

    2007-01-01

    Full Text Available Radiation induced gliomas are uncommon. Occurrence of glioma following radiotherapy for craniopharyngiomas is extremely uncommon and only eight case reports have been so far published. We present our experience with one similar case of temporal gliomas occurring twelve years following radiotherapy for a sub totally excised craniopharyngioma. Although the exact mechanism of gliomas formation is unclear, their occurrence following conventional radiotherapy is a distinct possibility and signifies a poor prognosis.

  7. Childhood Craniopharyngioma Treatment (PDQ®)—Patient Version

    Science.gov (United States)

    Childhood craniopharyngiomas are benign (not cancer) brain tumors found near the pituitary gland. Learn about the signs, tests to diagnose, and treatment (surgery, radiation therapy, chemotherapy, and immunotherapy) of pediatric craniopharyngioma in this expert-reviewed summary.

  8. [Social participation after childhood craniopharyngioma].

    Science.gov (United States)

    Olivari-Philiponnet, C; Roumenoff, F; Schneider, M; Chantran, C; Picot, M; Berlier, P; Mottolese, C; Bernard, J-C; Vuillerot, C

    2016-12-01

    Craniopharyngioma is a rare, benign central nervous system tumor, which may be a source of multiple complications, from endocrinology to vision, neurology and neurocognitive functions. This morbidity can lead to reduced participation in life activities, as described in the International Classification of Functioning, Disability, and Health. The primary objective of this study was to measure participation in life activities in a population of children and young adults affected by childhood craniopharyngioma, using the LIFE-H questionnaire (Assessment of Life Habits), validated as a social participation measurement tool in various pediatric disabilities. We also describe complications in our population and examined the potential links between tumor characteristics, complications, and participation in life activities. This was a descriptive study, including all patients having presented childhood craniopharyngioma (before 18 years of age), followed in the Lyon region between 2007 and 2013. The main criterion was the LIFE-H results, completed by the patient or the carer. Of 21 patients included in the study, 14 completed the questionnaire, a mean 6.7 years after the diagnosis (SD: 3.9 years). The mean total LIFE-H score was 8.4 (SD: 1.03) for a normal score estimated at 10 in the general population. The lowest scores affected the nutrition, community life, and recreation dimensions. No patient had a normal score on all dimensions; 57% of the patients had more than three dimensions affected. The variability of the results between patients was lower for some dimensions with high means (fitness, personal care, communication, housing, mobility, responsibilities, and education) than in others (nutrition, interpersonal relationships, community life, employment, and recreation) with rather low means. All patients had an endocrinological deficit, 19% hypothalamic syndrome, 52% an impaired fulfillment feeling, 76% visual impairment, 14% neurologic impairment, and 91

  9. Fast-growing Acer rubrum differs from slow-growing Quercus alba in leaf, xylem and hydraulic trait coordination responses to simulated acid rain.

    Science.gov (United States)

    Medeiros, Juliana S; Tomeo, Nicholas J; Hewins, Charlotte R; Rosenthal, David M

    2016-08-01

    We investigated the effects of historic soil chemistry changes associated with acid rain, i.e., reduced soil pH and a shift from nitrogen (N)- to phosphorus (P)-limitation, on the coordination of leaf water demand and xylem hydraulic supply traits in two co-occurring temperate tree species differing in growth rate. Using a full-factorial design (N × P × pH), we measured leaf nutrient content, water relations, leaf-level and canopy-level gas exchange, total biomass and allocation, as well as stem xylem anatomy and hydraulic function for greenhouse-grown saplings of fast-growing Acer rubrum (L.) and slow-growing Quercus alba (L.). We used principle component analysis to characterize trait coordination. We found that N-limitation, but not P-limitation, had a significant impact on plant water relations and hydraulic coordination of both species. Fast-growing A. rubrum made hydraulic adjustments in response to N-limitation, but trait coordination was variable within treatments and did not fully compensate for changing allocation across N-availability. For slow-growing Q. alba, N-limitation engendered more strict coordination of leaf and xylem traits, resulting in similar leaf water content and hydraulic function across all treatments. Finally, low pH reduced the propensity of both species to adjust leaf water relations and xylem anatomical traits in response to nutrient manipulations. Our data suggest that a shift from N- to P-limitation has had a negative impact on the water relations and hydraulic function of A. rubrum to a greater extent than for Q. alba We suggest that current expansion of A. rubrum populations could be tempered by acidic N-deposition, which may restrict it to more mesic microsites. The disruption of hydraulic acclimation and coordination at low pH is emphasized as an interesting area of future study. © The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  10. Effects of shelter type, early environmental enrichment and weather conditions on free-range behaviour of slow-growing broiler chickens.

    Science.gov (United States)

    Stadig, L M; Rodenburg, T B; Ampe, B; Reubens, B; Tuyttens, F A M

    2017-06-01

    Free-range use by broiler chickens is often limited, whereas better use of the free-range area could benefit animal welfare. Use of free-range areas could be stimulated by more appropriate shelter or environmental enrichment (by decreasing birds' fearfulness). This study aimed to assess the effects of shelter type, early environmental enrichment and weather conditions on free-range use. Three production rounds with 440 slow-growing broiler chickens (Sasso T451) were carried out. Birds were housed indoors in four groups (two with males, two with females) from days 0 to 25, during which two of the groups received environmental enrichment. At day 23 birds' fearfulness was assessed with a tonic immobility (TI) test (n=100). At day 25 all birds were moved (in mixed-sex groups) to mobile houses, and provided with free-range access from day 28 onwards. Each group could access a range consisting for 50% of grassland with 21 artificial shelters (ASs, wooden A-frames) and for 50% of short rotation coppice (SRC) with willow (dense vegetation). Free-range use was recorded by live observations at 0900, 1300 and 1700 h for 15 to 21 days between days 28 and 63. For each bird observed outside the shelter type (AS or SRC), distance from the house (0 to 2, 2 to 5, >5 m) and its behaviour (only rounds 2 and 3) were recorded. Weather conditions were recorded by four weather stations. On average, 27.1% of the birds were observed outside at any given moment of observation. Early environmental enrichment did not decrease fearfulness as measured by the TI test. It only had a minor effect on the percentage of birds outside (0.4% more birds outside). At all distances from the house, SRC was preferred over AS. In AS, areas closer to the house were preferred over farther ones, in SRC this was less pronounced. Free-range use increased with age and temperature and decreased with wind speed. In AS, rainfall and decreasing solar radiation were related to finding more birds outside, whereas the

  11. Craniopharyngioma: a roadmap for scientific translation.

    Science.gov (United States)

    Gupta, Saksham; Bi, Wenya Linda; Giantini Larsen, Alexandra; Al-Abdulmohsen, Sally; Abedalthagafi, Malak; Dunn, Ian F

    2018-06-01

    OBJECTIVE Craniopharyngiomas are among the most challenging of intracranial tumors to manage because of their pattern of growth, associated morbidities, and high recurrence rate. Complete resection on initial encounter can be curative, but it may be impeded by the risks posed by the involved neurovascular structures. Recurrent craniopharyngiomas, in turn, are frequently refractory to additional surgery and adjuvant radiation or chemotherapy. METHODS The authors conducted a review of primary literature. RESULTS Recent advances in the understanding of craniopharyngioma biology have illuminated potential oncogenic targets for pharmacotherapy. Specifically, distinct molecular profiles define two histological subtypes of craniopharyngioma: adamantinomatous and papillary. The discovery of overactive B-Raf signaling in the adult papillary subtype has led to reports of targeted inhibitors, with a growing acceptance for refractory cases. An expanding knowledge of the biological underpinnings of craniopharyngioma will continue to drive development of targeted therapies and immunotherapies that are personalized to the molecular signature of each individual tumor. CONCLUSIONS The rapid translation of genomic findings to medical therapies for recurrent craniopharyngiomas serves as a roadmap for other challenging neurooncological diseases.

  12. Metastatic craniopharyngioma: case report and literature review.

    Science.gov (United States)

    Frangou, Evan Mark; Tynan, Jennifer Ruth; Robinson, Christopher Adam; Ogieglo, Lissa Marie; Vitali, Aleksander Michal

    2009-09-01

    Distant spread of craniopharyngioma is a rare but important complication. Most cases are a result of spread along the surgical path. We describe a rare case of metastatic leptomeningeal craniopharyngioma as a result of dissemination along CSF pathways in a child. A review of previously described cases is provided. A 14-year-old male was diagnosed with metastatic craniopharyngioma on routine follow-up imaging after multiple surgeries and radiation for locally recurrent craniopharyngioma. The lesion was erosive through the right parietal bone, but had remained clinically silent. The lesion was distant from previous surgical paths. The patient underwent right parietal craniotomy and resection of the lesion. Duraplasty and cranioplasty were necessary for closure. Histopathology confirmed adamantinomatous craniopharyngioma. One-year follow-up demonstrated no recurrence. A review of reported cases suggests that leptomeningeal implantation may be an important step in metastases of craniopharyngioma, although the mechanism is poorly understood. Attention to tumor spillage at the time of surgery may be important in preventing distant recurrences.

  13. Sleep–wake and melatonin pattern in craniopharyngioma patients

    DEFF Research Database (Denmark)

    Pickering, Line; Jennum, Poul; Gammeltoft, Steen

    2014-01-01

    OBJECTIVE: To assess the influence of craniopharyngioma or consequent surgery on melatonin secretion, and the association with fatigue, sleepiness, sleep pattern and sleep quality. DESIGN: Cross-sectional study. METHODS: A total of 15 craniopharyngioma patients were individually matched to healthy......). Only patients with absent midnight peak had impaired sleep quality, increased daytime sleepiness and general and mental fatigue. CONCLUSION: Craniopharyngioma patients present with changes in circadian pattern and daytime symptoms, which may be due to the influence of the craniopharyngioma or its...

  14. Effects of ambient temperature and early open-field response on the behaviour, feed intake and growth of fast- and slow-growing broiler strains

    DEFF Research Database (Denmark)

    Nielsen, Birte Lindstrøm

    2012-01-01

    Increased activity improves broiler leg health, but also increases the heat production of the bird. This experiment investigated the effects of early open-field activity and ambient temperature on the growth and feed intake of two strains of broiler chickens. On the basis of the level of activity...... in an open-field test on day 3 after hatching, fast-growing Ross 208 and slow-growing i657 chickens were allocated on day 13 to one of the 48 groups. Each group included either six active or six passive birds from each strain and the groups were housed in floor-pens littered with wood chips and fitted...... with two heat lamps. Each group was fed ad libitum and subjected to one of the three temperature treatments: two (HH; 268C), one (HC; 168C to 268C) or no (CC; 168C) heat lamps turned on. Production and behavioural data were collected every 2 weeks until day 57. For both strains, early open-field activity...

  15. Motivation and ability to walk for a food reward in fast- and slow-growing broilers to 12 weeks of age.

    Science.gov (United States)

    Bokkers, Eddie A M; Koene, Paul

    2004-09-30

    Poor physical abilities of broilers may prevent them from performing behaviours for which they are motivated. The aim of this study was to measure the influence of physical ability and motivation on the performance of broilers in short physical tasks. We tested birds from a fast- and a slow-growing broiler strain in a runway to 12 weeks of age. To manipulate motivation, half of the birds of each strain was feed deprived for 3h and the other half for 24h before testing. Each bird was tested in a control and a slalom runway test once a week. With a similar motivation, slow growers had a shorter latency to start walking and walked faster through the runway than fast growers in both tests. In fast growers walking speed decreased faster with age than in slow growers. Slow growers vocalised more in both tests. In the slalom test, 24h deprived birds vocalised more than 3h deprived birds. Although the fast and slow growers have a different genetic background, the results indicated that motivation is the dominant determinative factor for walking in birds with a low body weight, while physical ability is the dominant determinative factor for walking in birds with a high body weight.

  16. Assessing the Significance of Above- and Belowground Carbon Allocation of Fast- and Slow-Growing Families of Loblolly Pine - Final Report

    Energy Technology Data Exchange (ETDEWEB)

    Topa, M. A.; Weinstein, D. A.; Retzlaff, W. A.

    2001-03-01

    During this project we experimentally evaluated the below-ground biomass and carbon allocation and partitioning of four different fast- and slow-growing families of loblolly pine located in Scotland County, NC, in an effort to increase the long-term performance of the crop. The trees were subjected to optimal nutrition and control since planting in 1993. Destructive harvests in 1998 and 2000 were used for whole?plant biomass estimates and to identify possible family differences in carbon acquisition (photosynthesis) and water use efficiency. At regular intervals throughout each year we sampled tissues for carbohydrate analyses to assess differences in whole-tree carbon storage. Mini rhizotron observation tubes were installed to monitor root system production and turnover. Stable isotope analysis was used to examine possible functional differences in water and nutrient acquisition of root systems between the various families. A genetic dissection of root ontogenic and architectural traits, including biomass partitioning, was conducted using molecular markers to better understand the functional implications of these traits on resource acquisition and whole-plant carbon allocation.

  17. Impact of overall treatment time on local control of slow growing human GL squamous cell carcinoma in nude mice treated by fractionated irradiation

    International Nuclear Information System (INIS)

    Baumann, M.; Petersen, C.; Schulz, P.; Baisch, H.

    1999-01-01

    Background and purpose: The impact of overall treatment time of fractionated irradiation on local control of slow growing human GL squamous cell carcinoma (SCC) was determined. Materials and methods: Moderately well differentiated and keratinizing human GL SCC with a volume doubling time of 8 days were transplanted subcutaneously into the right hindleg of NMRI (nu/nu) mice and irradiated with 30 fractions under ambient conditions over 2, 3, 4.5, 6 and 10 weeks. Endpoint of the experiments was local tumor control at day 180 after end of treatment.Results: The tumor control dose 50% (TCD 50) increased from 40 to 57 Gy when the treatment time was extended from 2 to 10 weeks. The data can be well described by a linear increase in TCD 50 with time. The recovered dose per day (D r ) was 0.28 Gy (95% confidence interval 0.06; 0.48). The fit to the data was not significantly improved by assuming a biphasic (dog-leg) time course with constant TCD 50 values in the initial part of treatment followed by a more rapid increase of TCD 50 thereafter.Conclusions: D r in GL SCC was significantly less than the value of 1.0 Gy (0.7; 1.3 ) previously reported for poorly differentiated, non-keratinizing and fast growing human FaDu SCC (Baumann M, Liertz C, Baisch H, Wiegel T, Lorenzen J, Arps H. Impact of overall treatment time of fractionated irradiation on local control of human FaDu squamous cell carcinoma in nude mice. Radiother. Oncol. 1994;32:137-143), indicating important heterogeneity of the time factor between different tumors of the same histological type. (Copyright (c) 1999 Elsevier Science B.V., Amsterdam. All rights reserved.)

  18. MR differentiation of craniopharyngioma from pituitary macroadenoma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Soo; Ahn, Kook Jin; Kim, Jee Young; Lee, Jeong Whee; Jeh, Soo Kyung; Park, Hae Kwan; Lee, Kyung Jin; Hahn, Seong Tae [Catholic University College of Medicine, Seoul (Korea, Republic of)

    2006-07-15

    We wanted to determine the differential points between craniopharyngioma and pituitary macroadenoma on MRI. The MRI findings in twenty seven patients (age range: 14-67 years, mean age: 46 years, 17 males and 10 females) with pathologically proven craniopharyngioma and twenty four patients (age range: 23-64 years, mean age: 54 years, 8 males and 16 females) with pathologically proven pituitary macroadenoma were analyzed retrospectively by two radiologists. We analyzed the location, the contour of the mass, the presence of high signal intensity on the T1 weighted images, the thickness of the enhancing wall, separation between the mass and the pituitary gland, and the presence of attachment or compression to the midbrain. On MRI, craniopharyngiomas showed a suprasellar location, high signal intensity on the T1 weighted images and a larger suprasellar portion. After contrast enhancement, the separation of the mass from the pituitary gland is more distinct than that of the pituitary macroadenomas. The craniopharyngiomas showed the presence of attachment or compression to the midbrain. The pituitary macroadenomas had a larger intra-or infrasellar portion than that of the craniopharyngiomas, and they also showed a thicker enhancing wall after contrast enhancement. The location, contour of the mass, presence of high signal intensity on T1 weighted images, thickness of the enhancing wall, separation of the mass from the pituitary gland and the presence of attachment or compression to midbrain are useful differential points between craniophayngioma and pituitary macroadenoma on MRI.

  19. MR differentiation of craniopharyngioma from pituitary macroadenoma

    International Nuclear Information System (INIS)

    Kim, Hyun Soo; Ahn, Kook Jin; Kim, Jee Young; Lee, Jeong Whee; Jeh, Soo Kyung; Park, Hae Kwan; Lee, Kyung Jin; Hahn, Seong Tae

    2006-01-01

    We wanted to determine the differential points between craniopharyngioma and pituitary macroadenoma on MRI. The MRI findings in twenty seven patients (age range: 14-67 years, mean age: 46 years, 17 males and 10 females) with pathologically proven craniopharyngioma and twenty four patients (age range: 23-64 years, mean age: 54 years, 8 males and 16 females) with pathologically proven pituitary macroadenoma were analyzed retrospectively by two radiologists. We analyzed the location, the contour of the mass, the presence of high signal intensity on the T1 weighted images, the thickness of the enhancing wall, separation between the mass and the pituitary gland, and the presence of attachment or compression to the midbrain. On MRI, craniopharyngiomas showed a suprasellar location, high signal intensity on the T1 weighted images and a larger suprasellar portion. After contrast enhancement, the separation of the mass from the pituitary gland is more distinct than that of the pituitary macroadenomas. The craniopharyngiomas showed the presence of attachment or compression to the midbrain. The pituitary macroadenomas had a larger intra-or infrasellar portion than that of the craniopharyngiomas, and they also showed a thicker enhancing wall after contrast enhancement. The location, contour of the mass, presence of high signal intensity on T1 weighted images, thickness of the enhancing wall, separation of the mass from the pituitary gland and the presence of attachment or compression to midbrain are useful differential points between craniophayngioma and pituitary macroadenoma on MRI

  20. Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults

    DEFF Research Database (Denmark)

    Nielsen, Eigil Husted; Feldt-Rasmussen, Ulla; Poulsgaard, Lars

    2011-01-01

    We studied the incidence of craniopharyngioma in Denmark during the period 1985-2004 and estimated worldwide incidence rates (IR) of craniopharyngioma based on a literature review. Craniopharyngioma patients diagnosed during the period 1985-2004 were identified from the Danish National Patient...... PubMed and, if appropriate, were included in a weighted analysis estimating overall and children's IRs of craniopharyngioma. IRs are given as new cases per million per year. We identified 189 patients with new verified (162) or probable craniopharyngioma. The overall WHO World-standardised incidence...

  1. CT and MR imaging of craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Tsuda, M. [Tohoku Univ. School of Medicine, Sendai (Japan). Dept. of Radiology; Takahashi, S. [Tohoku Univ. School of Medicine, Sendai (Japan). Dept. of Radiology; Higano, S. [Tohoku Univ. School of Medicine, Sendai (Japan). Dept. of Radiology; Kurihara, N. [Tohoku Univ. School of Medicine, Sendai (Japan). Dept. of Radiology; Ikeda, H. [Tohoku Univ. School of Medicine, Sendai (Japan). Dept. of Neurosurgery; Sakamoto, K. [Tohoku Univ. School of Medicine, Sendai (Japan). Dept. of Radiology

    1997-05-01

    We reviewed imaging findings of CT and MR imaging in 20 cases of surgically confirmed craniopharyngioma in an attempt to determine their relation to patterns of tumor extent. The relationship between these patterns and the frequency of preoperative CT diagnosis and MR imaging diagnosis according to the surgical diagnosis were determined. The CT technique was superior to MR imaging in the detection of calcification. The MR imaging technique was superior to CT for determining tumor extent and provided valuable information about the relationships of the tumor to surrounding structures. Thus, CT and MR imaging have complementary roles in the diagnosis of craniopharyngiomas. In cases of possible craniopharyngioma, noncontrast sagittal T1-weighted images may enable the identification of the normal pituitary, possibly leading to the correct diagnosis. (orig.)

  2. CT and MR imaging of craniopharyngioma

    International Nuclear Information System (INIS)

    Tsuda, M.; Takahashi, S.; Higano, S.; Kurihara, N.; Ikeda, H.; Sakamoto, K.

    1997-01-01

    We reviewed imaging findings of CT and MR imaging in 20 cases of surgically confirmed craniopharyngioma in an attempt to determine their relation to patterns of tumor extent. The relationship between these patterns and the frequency of preoperative CT diagnosis and MR imaging diagnosis according to the surgical diagnosis were determined. The CT technique was superior to MR imaging in the detection of calcification. The MR imaging technique was superior to CT for determining tumor extent and provided valuable information about the relationships of the tumor to surrounding structures. Thus, CT and MR imaging have complementary roles in the diagnosis of craniopharyngiomas. In cases of possible craniopharyngioma, noncontrast sagittal T1-weighted images may enable the identification of the normal pituitary, possibly leading to the correct diagnosis. (orig.)

  3. Abscess inside craniopharyngioma: diagnostic and management implications.

    Science.gov (United States)

    Bhaisora, Kamlesh Singh; Prasad, Surya Nandan; Das, Kuntal Kanti; Lal, Hira

    2018-02-03

    Abscess inside the pituitary fossa is very rare. Such abscess can be primary, occurring in an otherwise healthy pituitary gland, or secondary, developing inside a diseased gland (ie, harbouring craniopharyngioma, Rathke's cleft cyst, etc). Secondary pituitary abscess inside a craniopharyngioma remains an extremely rare occurrence. Our literature search revealed only six such cases reported so far. In this report, we present the seventh case of craniopharyngioma with abscess in a 38-year-old woman. We describe the uniqueness of the clinical presentation of our case, the radiological pointers to the possible diagnosis and the management issues in our patient. A review of literature is also included to provide a comprehensive picture of this rare condition to the readers. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  4. Intrinsic third ventricular craniopharyngioma: A case report

    Directory of Open Access Journals (Sweden)

    Nazila Tayari

    2011-01-01

    Full Text Available Craniopharyngioma accounts for 2.5-4 percent of all intracranial tumors. The tumor is more observed in the chiasmatic region in adults and the intraventricular subtype is rare. We report an intraventricular craniopharyngioma in a 22-year-old woman presented with chronic headache. Magnetic Resonance Imaging showed hyperintense large mass on T 1 -weighted images and hypointense mass on T 2 -weighted images in third ventricle with pressure effect on both lateral ventricles and foramen of Monro. The diagnosis of craniopharyngioma was confirmed through histopathological examination of the resected tumor after surgery. After a follow-up period of nine months, neither tumor recurrence nor regrowth occurred. The early diagnosis of this relatively frequent tumor would help to prevent related sequelae.

  5. Effects of free-range access on production parameters and meat quality, composition and taste in slow-growing broiler chickens.

    Science.gov (United States)

    Stadig, Lisanne M; Rodenburg, T Bas; Reubens, Bert; Aerts, Johan; Duquenne, Barbara; Tuyttens, Frank A M

    2016-12-01

    Demand for meat from free-range broiler chickens is increasing in several countries. Consumers are motivated by better animal welfare and other product attributes such as quality and taste. However, scientific literature is not unanimous about whether free-range access influences quality, composition, and taste of the meat. Because chickens normally do not use free-range areas optimally, it is possible that provision of more suitable shelter will lead to more pronounced differences between chickens raised indoors and outdoors. In this study, an experiment with 2 production rounds of 600 slow-growing broilers each was performed. In each round, 200 chickens were raised indoors (IN), 200 had free-range access to grassland with artificial shelter (AS), and 200 had free-range access to short-rotation coppice with willow (SRC). Free-range use, feed intake, and growth were monitored, and after slaughter (d72) meat quality, composition, and taste were assessed. Free-range use was higher in SRC than in AS chickens (42.8 vs. 35.1%, P free-range access was darker (P = 0.021) and yellower (P = 0.001) than that of IN chickens. Ultimate pH was lower (5.73 vs. 5.79; P = 0.006) and drip loss higher (1.29 vs. 1.09%; P = 0.05) in IN versus AS chickens. The percentage of polyunsaturated fatty acids was higher in AS than in IN meat (35.84 vs. 34.59%; P = 0.021). The taste panel judged breast meat of SRC chickens to be more tender (P = 0.003) and less fibrous (P = 0.013) compared to that of AS and IN chickens, and juicier compared to the IN chickens (P = 0.017). Overall, free-range access negatively affected slaughter weight, but positively affected meat quality, taste, and composition. Only a few differences between AS and SRC were found, possibly due to limited differences in free-range use. © 2016 Poultry Science Association Inc.

  6. Malignant Transformation of Radiotherapy-Naïve Craniopharyngioma.

    Science.gov (United States)

    Chunhui, Liu; Chuzhong, Li; Zhenye, Li; Yilin, Sun; Yazhuo, Zhang

    2016-04-01

    Craniopharyngioma is a rare benign intracranial neoplasm that is successfully managed with surgery or adjuvant radiotherapy. The malignant transformation of craniopharyngioma has seldom been reported. A 30-year-old woman presented with a 5-month history of amenorrhea and was admitted to the hospital. She underwent surgical resection for three times and died at last. MRI revealed a new solid component of craniopharyngioma. Pathologic examination revealed malignant changes in the craniopharyngioma. In addition, We analyzed the expression of Ki-67, p53, VEGF, and MMP-9 in this malignant case after the third operation and in samples from 9 benign craniopharyngiomas. Immunohistochemical analysis showed that the Ki-67 index was higher in malignant craniopharyngiomas (50%) compared with benign craniopharyngiomas (3.0% ± 1.5%; range, 1.0%-6.0%). The p53, MMP-9, and VEGF protein levels were higher in the malignant craniopharyngioma compared with the benign craniopharyngiomas. Patients with a high Ki-67 index and high p53, MMP-9, and VEGF protein levels and a new solid component of craniopharyngioma on MRI may benefit from aggressive treatment and close surveillance. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Endoscopic Transsphenoidal Resection of Craniopharyngioma.

    Science.gov (United States)

    Liew, Kong Yew; Narayanan, Prepageran; Waran, Vicknes

    2018-02-01

    Objectives  To demonstrate, step-by-step, the technique and efficacy of endoscopic transsphenoidal approach in resection of a suprasellar craniopharyngioma. Design  The video shows a step-by-step approach to the resection, covering the exposure, access, resection, and confirmation of resection and reconstruction. Setting  The surgery was performed in the University of Malaya Medical Centre, a tertiary referral center in the capital of Malaysia. Participants  Surgery was performed jointly by Professor Prepageran from the department of otorhinolaryngology and Professor Vicknes Waran from the division of neurosurgery. Both surgeons are from the University of Malaya. Video compilation, editing, and voice narration was done by Dr. Kong Yew Liew. Main Outcome Measures  Completeness of resection and avoidance of intra- and postoperative complications. Results  Based on intraoperative views and MRI findings, the tumor was completely resected with the patient suffering only transient diabetes insipidus. Conclusion  Central suprasellar tumors can be removed completely via an endoscopic transsphenoidal approach with minimal morbidity to the patient. The link to the video can be found at: https://youtu.be/ZNIHfk12cYg .

  8. Postoperative Radiation Therapy of Craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Kyung Hwan; Kim, Il Han; Park, Charn Il; Cho, Byung Kyu [Seoul National University College of Medicine, Seoul (Korea, Republic of); Yun, Hyong Geln [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    1993-06-15

    Between December 1979 and September 1989, 23 patients with craniopharyngioma who underwent surgery and postoperative radiation therapy were retrospectively evaluated to assess the efficacy of this management at the Department of Therapeutic Radiology, Seoul National University Hospital. Total removal of tumor was attempted in all patients. Of these, surgeons tried total removal in eight patients, but revealed residual mass by postoperative CT, and partial removal was done in 15 patients. The morphology of tumor on the operative finding was grouped into three types : cystic 13 (57%), solid 4 (17%), and mixed 6 (26%). Cystic type was predominant in {<=}20 years old group. Actuarial overall survival rates at 5 and 10 years were 95% and 81% respectively and actuarial tumor control rates were 74% and 50%. Surgical extent was not related to the survival rates(p=0.41). Pediatric and adolescent Patients(age of {<=}20 year) had a trend of better survival than that of adult patients(p=0.10). The results indicated that limited surgical excision followed by radiation therapy is recommended when total excision is not possible.

  9. Gamma knife therapy for craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki; Yoshida, Kazuo; Yoshimoto, Masayuki; Maezawa, Satoshi; Hasegawa, Toshinori [Komaki Hospital, Aichi (Japan)

    1997-01-01

    Gamma knife therapy in stereotactic radiosurgery was evaluated as a tool to solve problems raised in therapy for craniopharyngioma. Subjects were 9 childhood patients (<16 y, mean age 9.75 y) and 16 adult patients (mean age 43.9 y), 23 cases of whom had been treated with surgery before gamma knife. Planning of irradiation to the solid part of the tumor was based on their T1-weighted MRI images of 3 mm-thick slice. The mean size of the tumors was 20.7 (9.6-31.2) mm in diameter. The mean central dose of 23.3 (20-30) Gy was irradiated with the mean marginal dose of 11.8 (18-11.3) Gy through the mean shot of 4.8. Results were followed in every 3-6 months by MRI, neurological and endocrinological examinations for 7-52 (mean 23.3) months. Reduction of tumor size including its disappearance (7 cases) were observed in 22 cases (88%) with adverse effects of hypopituitarism (3 cases) and hemianopsia (1). Gamma knife therapy was thus safe and effective. (K.H.)

  10. Postoperative Radiation Therapy of Craniopharyngioma

    International Nuclear Information System (INIS)

    Shin, Kyung Hwan; Kim, Il Han; Park, Charn Il; Cho, Byung Kyu; Yun, Hyong Geln

    1993-01-01

    Between December 1979 and September 1989, 23 patients with craniopharyngioma who underwent surgery and postoperative radiation therapy were retrospectively evaluated to assess the efficacy of this management at the Department of Therapeutic Radiology, Seoul National University Hospital. Total removal of tumor was attempted in all patients. Of these, surgeons tried total removal in eight patients, but revealed residual mass by postoperative CT, and partial removal was done in 15 patients. The morphology of tumor on the operative finding was grouped into three types : cystic 13 (57%), solid 4 (17%), and mixed 6 (26%). Cystic type was predominant in ≤20 years old group. Actuarial overall survival rates at 5 and 10 years were 95% and 81% respectively and actuarial tumor control rates were 74% and 50%. Surgical extent was not related to the survival rates(p=0.41). Pediatric and adolescent Patients(age of ≤20 year) had a trend of better survival than that of adult patients(p=0.10). The results indicated that limited surgical excision followed by radiation therapy is recommended when total excision is not possible

  11. Craniopharyngioma in Children: Long-term Outcomes

    Science.gov (United States)

    STEINBOK, Paul

    2015-01-01

    The survival rate for childhood craniopharyngioma has been improving, with more long-term survivors. Unfortunately it is rare for the patient to be normal, either from the disease itself or from the effects of treatment. Long-term survivors of childhood craniopharyngioma suffer a number of impairments, which include visual loss, endocrinopathy, hypothalamic dysfunction, cerebrovascular problems, neurologic and neurocognitive dysfunction. Pituitary insufficiency is present in almost 100%. Visual and hypothalamic dysfunction is common. There is a high risk of metabolic syndrome and increased risk of cerebrovascular disease, including stroke and Moyamoya syndrome. Cognitive, psychosocial, and emotional problems are prevalent. Finally, there is a higher risk of premature death among survivors of craniopharyngioma, and often this is not from tumor recurrence. It is important to consider craniopharyngioma as a chronic disease. There is no perfect treatment. The treatment has to be tailored to the individual patient to minimize dysfunction caused by tumor and treatments. So “cure” of the tumor does not mean a normal patient. The management of the patient and family needs multidisciplinary evaluation and should involve ophthalmology, endocrinology, neurosurgery, oncology, and psychology. Furthermore, it is also important to address emotional issues and social integration. PMID:26345668

  12. Huge cystic craniopharyngioma with unusual extensions

    Energy Technology Data Exchange (ETDEWEB)

    Kitano, I.; Yoneda, K.; Yamakawa, Y.; Fukui, M.; Kinoshita, K.

    1981-09-01

    The findings on computed tomography (CT) of a huge cystic craniopharyngioma in a 3-year-old girl are described. The cyst occupied both anterior cranial fossae and a part of it extended to the region of the third ventricle which was displaced posteriorly. The tumor showed no contrast enhancement after the intravenous administration of contrast medium.

  13. Malignant astrocytoma following radiotherapy for craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Maat-Schieman, M.L.C.; Bots, G.T.A.M.; Thomeer, T.W.M.; Vielvoye, G.J. (Rijksuniversiteit Leiden (Netherlands). Hospital)

    1985-05-01

    The case report describes a boy with a malignant astrocytoma in the mid-line of the cerebellum 14 years after X-ray therapy for craniopharyngioma. In Leiden University Hospital this is the first case of a suspected radiation-induced brain tumour in 66 patients treated for cranial lesions by radiotherapy between 1969 and 1979 who have survived more than 5 years.

  14. Bleomycin toxicity post injection into craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Ivan Norval

    2012-02-01

    Full Text Available Craniopharyngioma is a common suprasellar mass that is frequently encountered in radiology. These tumours are difficult to treat, with a combination of resection and radiation therapy having been the gold standard. Our case highlights a complication of a newer approach to treatment in which bleomycin is administered into the tumour via a catheter, usually an Ommaya reservoir that is surgically placed.

  15. Glioblastoma after radiotherapy for craniopharyngioma: case report

    International Nuclear Information System (INIS)

    Ushio, Y.; Arita, N.; Yoshimine, T.; Nagatani, M.; Mogami, H.

    1987-01-01

    A 6-year-old girl developed a glioblastoma in the basal ganglia and brain stem 5 years after surgical excision and local irradiation (5460 cGy) for craniopharyngioma. Clinical and histological details are presented, and the literature on radiation-induced gliomas is reviewed

  16. Protean appearance of craniopharyngioma on computed tomography

    International Nuclear Information System (INIS)

    Danziger, A.; Price, H.I.

    1979-01-01

    Craniopharyngiomas present a diverse appearance on computed tomography. Histological diagnosis is not always possible, but computed tomography is of great assistance in the delineation of the tumour as well as of the degree of associated hydrocephalus. Computed tomography also enables rapid non-invasive follow-up after surgery or radiotherapy, or both

  17. Bleomycin toxicity post injection into craniopharyngioma | Norval ...

    African Journals Online (AJOL)

    Craniopharyngioma is a common suprasellar mass that is frequently encountered in radiology. These tumours are difficult to treat, with a combination of resection and radiation therapy having been the gold standard. Our case highlights a complication of a newer approach to treatment in which bleomycin is administered ...

  18. CRANIOPHARYNGIOMA PRESENTING AS ‘MANIA’—CASE REPORT

    Science.gov (United States)

    Prusty, Gouri K.; Subramanya; Hemalatha, V.; Narayanan, H.S.

    1982-01-01

    SUMMARY We are reporting a case of craniopharyngioma presenting with features of mania. To our knowledge, this is the first reported case of craniopharyngioma with presenting features of mania- The patient is a six years old child with history of maniac behaviour of six months duration. There is no significant family history. During the course in the hospital he was found to be having craniopharyngioma. The patient recovered completely following the surgical intervention without any aid of antipsychotics. PMID:21965929

  19. Chemical meningitis from a leaking craniopharyngioma: a case report.

    Science.gov (United States)

    Hakizimana, David; Poulsgaard, Lars; Fugleholm, Kåre

    2018-06-01

    Recurrent chemical meningitis from cyclic leakage of cyst content from a craniopharyngioma is a rare phenomenon. Here, we report a case of leaking cystic craniopharyngioma presenting with recurrent episodes of sterile meningitis, depression, and paranoia. The diagnosis after an initial craniotomy and exploration was hypophysitis. Signs and symptoms were not alleviated by puncture and biopsy of the tumour but they disappeared after complete resection with a final histological diagnosis of craniopharyngioma.

  20. A typical magnetic resonance imaging findings of craniopharyngioma

    International Nuclear Information System (INIS)

    Wang, Y.-X.J.; Jiang, H.; He, G.-X.

    2001-01-01

    Three cases of craniopharyngiomas with atypical MRI findings are reported. The first patient had a nasopharyngeal craniopharyngioma. Its unusual location made diagnosis difficult. The second patient had a massive craniopharyngioma with extensive cystic expansion, involving the anterior, middle and posterior cranial fossae, and extending into the foramen magnum. The tumour of the third patient involved the suprasellar region with a large extension into the third ventricle, and demonstrated a predominantly high signal intensity on all T1-weighted, proton-weighted and T2-weighted images. These patients further stressed the complexity of MRI findings in craniopharyngiomas. Copyright (2001) Blackwell Science Pty Ltd

  1. Ectopic craniopharyngioma of the fourth ventricle in a patient with Gardner syndrome.

    Science.gov (United States)

    Pena, Andres H; Chaudhry, Ammar; Seidman, Roberta J; Peyster, Robert; Bangiyev, Lev

    2016-01-01

    Ectopic craniopharyngioma is uncommon and a craniopharyngioma confined purely within the fourth ventricle is extremely rare. We report a craniopharyngioma of the fourth ventricle in a 20-year-old man with Gardner syndrome. Imaging characteristics of craniopharyngiomas and fourth ventricle lesions are discussed with a review of the literature regarding the pathogenesis of craniopharyngiomas and the possible association with Gardner syndrome. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. Fever of unknown origin as a presenting manifestation of craniopharyngioma in a child

    OpenAIRE

    NANDI, Madhumita; MONDAL, Rakesh

    2010-01-01

    An unusual case of cranio-pharyngioma which presented with prolonged fever described here. Investigation revealed that the child was suffering from leaking craniopharyngioma with hypo-pituitarism Fever was due to chemical meningitis following cranio-pharyngioma as evident from the CSF findings. Craniopharyngioma can cause prolonged or recurrent fever due to various reasons [1]. Prolonged fever as the sole manifestation of cranio-pharyngioma has been rarely reported in literature and this is p...

  3. Craniopharyngiomas: The spectrum of MR appearances

    International Nuclear Information System (INIS)

    Young, S.C.; Zimmerman, R.A.; Hochman, M.; Bilaniuk, L.T.; Hackney, D.B.; Sutton, L.N.; Grossman, R.I.; Goldberg, H.I.

    1987-01-01

    A retrospective study of craniopharyngiomas examined by a 1.5-T MR imaging unit was conducted. A total of 23 patients were proved or presumed craniopharyngiomas are included. Eleven of these were imaged by MR preoperatively and the findings were subsequently proved by pathology. Three of five patients with residual or recurrent craniopharyngiobima imaged postoperatively were subsequently proved to have tumor. Seven cases that were surgically unproved are by clinical and imaging criteria consistent with proved cases. The findings on short TR and TE spin-echo images (TR = 600 msec, TE = 20 msec) and long TR (2,500 msec), short TE (30 msec) and long TE (80 msec) spin-echo images are presented. Calcification shown by CT in nine cases could be detected with MR in three cases but only retrospect. Cyst fluid was sent in several cases for chemical analysis

  4. Management of craniopharyngiomas: Role of conservative strategies

    Directory of Open Access Journals (Sweden)

    Shibu Pillai

    2013-01-01

    Full Text Available Craniopharyngiomas are uncommon tumors and their management remains controversial. Despite gross total resection, recurrences can occur in 15-43% during long-term follow-up of more than 10 years. Furthermore, radical surgery can be associated with complications associated with hypothalamic damage, which can lead to a poor quality-of-life. Limited surgery followed by adjuvant radiotherapy (RT can provide excellent long-term tumor control with minimum complications and good functional outcome. RT can however, produce insidious onset of hypopituitarism and vasculopathy. Secondary malignancies are a rare complication of RT. Modern radiation techniques and intracystic radiation can reduce these complications. Intracystic radiation is highly effective for cystic craniopharyngiomas. Intracystic bleomycin (ICB and interferon alpha (IFN are rarely curative, but can produce long lasting control of tumor cysts and this is very useful in very young children who may not tolerate radical surgery or RT. IFN has fewer toxicity issues compared to ICB.

  5. Childhood Craniopharyngioma Treatment (PDQ®)—Health Professional Version

    Science.gov (United States)

    Craniopharyngiomas are believed to be congenital in origin, arising from ectodermal remnants, Rathke cleft, or other embryonal epithelium. The 10-year survival rate exceeds 90%. Get detailed information about the presentation, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood craniopharyngioma in this summary for clinicians.

  6. Craniopharyngioma in the temporal lobe: a case report

    International Nuclear Information System (INIS)

    Sohn, Chul-Ho; Baik, Seung-Kug; Kim, Sang-Pyo; Kim, Il-Man; Sevick, Robert J.

    2004-01-01

    Herein, we report on an unusual case of craniopharyngioma arising in the temporal lobe with no prior history of surgery and with no connection to the craniopharyngeal duct. MR images showed cystic tumor with a small solid portion. To the best of our knowledge, this is the first case of a craniopharyngioma occurring in the temporal lobe

  7. A Rare Case of Craniopharyngioma in the Temporal Lobe

    Directory of Open Access Journals (Sweden)

    Sasan Razmjoo

    2017-01-01

    Full Text Available Herein, we report on a rare case of craniopharyngioma arising in the left temporal lobe with no prior history of head trauma or surgery. There was a solid-cystic mass in the left temporal lobe on MR images. To the best of our knowledge, this is the second case of a craniopharyngioma occurring in the temporal lobe.

  8. Craniopharyngioma in the temporal lobe: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Sohn, Chul-Ho; Baik, Seung-Kug; Kim, Sang-Pyo; Kim, Il-Man; Sevick, Robert J. [University of Calgary, Calgary (Canada)

    2004-03-15

    Herein, we report on an unusual case of craniopharyngioma arising in the temporal lobe with no prior history of surgery and with no connection to the craniopharyngeal duct. MR images showed cystic tumor with a small solid portion. To the best of our knowledge, this is the first case of a craniopharyngioma occurring in the temporal lobe.

  9. Quality of life in treated adult craniopharyngioma patients

    NARCIS (Netherlands)

    Dekkers, O. M.; Biermasz, N. R.; Smit, J. W. A.; Groot, L. E.; Roelfsema, F.; Romijn, J. A.; Pereira, A. M.

    2006-01-01

    Quality of life (QoL) has become increasingly important in the evaluation of treatment of pituitary and hormonal diseases. A reduced QoL has been reported in childhood-onset craniopharyngioma; however, reports of QoL in adult craniopharyngioma patients are scarce. In the present study, we assessed

  10. Craniopharyngioma in the Temporal Lobe: A Case Report

    Science.gov (United States)

    Baik, Seung Kug; Kim, Sang-Pyo; Kim, Il-Man; Sevick, Robert J.

    2004-01-01

    Herein, we report on an unusual case of craniopharyngioma arising in the temporal lobe with no prior history of surgery and with no connection to the craniopharyngeal duct. MR images showed a cystic tumor with a small solid portion. To the best of our knowledge, this is the first case of a craniopharyngioma occurring in the temporal lobe. PMID:15064562

  11. Huge cystic craniopharyngioma. Changes of cyst density on computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Takamura, Seishi; Fukumura, Akinobu; Ito, Yoshihiro; Itoyama, Yoichi; Matsukado, Yasuhiko

    1986-06-01

    The findings of computed tomography (CT) of a huge cystic craniopharyngioma in a 57-year-old woman are described. Cyst density varied from low to high levels in a short duration. Follow-up CT scans were regarded as important to diagnose craniopharyngioma. The mechanism of increment of cyst density was discussed.

  12. Pre- and postoperative MR imaging of craniopharyngiomas

    Energy Technology Data Exchange (ETDEWEB)

    Hald, J.K. [Rijkshospitalet, Oslo (Norway). Dept. of Radiology; Eldevik, O.P. [Rijkshospitalet, Oslo (Norway). Dept. of Neurosurgery; Quint, D.J. [Rijkshospitalet, Oslo (Norway). Dept. of Neurosurgery; Chandler, W.F. [Univ. of Michigan Hospital, Ann Arbor, MI (United States). Dept. of Radiology; Kollevold, T. [Univ. of Michigan Hospital, Ann Arbor, MI (United States). Dept. of Neurosurgery

    1996-09-01

    Purpose: To compare the pre- and postoperative MR appearance of craniopharyngiomas with respect to lesion size, tumour morphology and identification of surrounding normal structures. Material and Methods: MR images obtained prior to and following craniopharyngioma resection were evaluated retrospectively in 10 patients. Tumour signal charcteristics, size and extension with particular reference to the optic chiasm, the pituitary gland, the pituitary stalk and the third ventricle were evaluated. Results: Following surgery, tumour volume was reduced in all patients. In 6 patients there was further tumour volume reduction between the first and second postoperative images. Two of these patients received radiation therapy between the 2 postoperative studies, while 4 had no adjuvant treatment to the surgical intervention. There was improved visualization of the optic chiasm, in 3, the pituitary stalk in one, and the third ventricle in 9 of the 10 patients. The pituitary gland was identified preoperatively only in one patient, postoperatively only in another, pre- and postoperatively in 5, and neither pre- nor postoperatively in 3 patients. In 3 patients MR imaging 0-7 days postoperatively identified tumour remnants not seen at the end of the surgical procedure. The signal intensities of solid and cystic tumour components were stable from pre- to the first postoperative MR images. Optic tract increased signal prior to surgery was gone 28 days postoperatively in one patient, but persisted on the left side for 197 days after surgery in another. Conclusion: Postoperative MR imaging of craniopharyngiomas demonstrated tumour volume reduction and tumour remnants not seen at surgery. Early postoperative MR imaging of craniopharyngiomas may overestimate the size of residual tumour. Improved visualization of peritumoral structures may be achieved. (orig.).

  13. Diagnostic imaging of craniopharyngioma; Diagnostyka obrazowa czaszkogardlakow

    Energy Technology Data Exchange (ETDEWEB)

    Gradzki, J.; Nowak, S.; Paprzycki, W. [Akademia Medyczna, Poznan (Poland)

    1993-12-31

    40 patients have been examined with operational and histological confirmation of craniopharyngioma. CT image and X-ray plane of skull were performed in case all of these patients. TMR was conformed to examine 4 patients. X-ray planes was compared to CT. CT permits tumor cyst detection. The efficacy of mentioned above diagnostic techniques was compared with surgical findings. (author). 7 refs, 5 figs, 2 tabs.

  14. Malignant astrocytoma following radiotherapy for craniopharyngioma

    International Nuclear Information System (INIS)

    Maat-Schieman, M.L.C.; Bots, G.T.A.M.; Thomeer, T.W.M.; Vielvoye, G.J.

    1985-01-01

    The case report describes a boy with a malignant astrocytoma in the mid-line of the cerebellum 14 years after X-ray therapy for craniopharyngioma. In Leiden University Hospital this is the first case of a suspected radiation-induced brain tumour in 66 patients treated for cranial lesions by radiotherapy between 1969 and 1979 who have survived more than 5 years. (author)

  15. Expanded Endoscopic Endonasal Resection of Retrochiasmatic Craniopharyngioma.

    Science.gov (United States)

    Davanzo, Justin R; Goyal, Neerav; Zacharia, Brad E

    2018-02-01

    This video abstract demonstrates the use of the expanded endoscopic endonasal approach for the resection of a retrochiasmatic craniopharyngioma. These tumors are notoriously difficult to treat, and many approaches have been tried to facilitate safe and effective resection. The endoscopic endonasal approach has been increasingly utilized for selected sellar/suprasellar pathology. We present the case of a 39-year-old man who was found to have a cystic, partially calcified suprasellar mass consistent with a craniopharyngioma. To facilitate robust skull base repair, a vascularized nasoseptal flap was harvested. A wide sphenoidotomy was performed and the sella and tuberculum were exposed. After the dural opening and arachnoid dissection, the stalk was identified, merging seamlessly with the tumor capsule. The lesion was then internally debulked with the use of an ultrasonic aspirator. The capsule was then dissected off of the optic chiasm, thalamus, and hypothalamus. The cavity was inspected with an angled endoscope to ensure complete resection. A multilayered reconstruction was performed using autologous fascia lata, the previously harvested nasoseptal flap, and dural sealant. Postoperatively, the patient did have expected panhypopituitarism but remained neurologically intact and had improvement in his vision. In conclusion, this video demonstrates how an expanded endonasal approach can be used to safely resect a craniopharyngioma, even when in close proximity to delicate structures such as the optic chiasm. The link to the video can be found at: https://youtu.be/tahjHmrXhc4 .

  16. Endoscopic Endonasal Approach for a Suprasellar Craniopharyngioma.

    Science.gov (United States)

    Zenonos, Georgios A; Snyderman, Carl H; Gardner, Paul A

    2018-04-01

    Objectives  The current video presents the nuances of an endoscopic endonasal approach to a suprasellar craniopharyngioma. Design  The video analyzes the presentation, preoperative workup and imaging, surgical steps and technical nuances of the surgery, the clinical outcome, and follow-up imaging. Setting  The patient was treated by a skull base team consisting of a neurosurgeon and an ENT surgeon, at a teaching academic institution. Participants  The case refers to a 67-year-old man who presented with vision loss and headaches, and was found to have a suprasellar mass, with imaging characteristics consistent with a craniopharyngioma. Main Outcome Measures  The main outcome measures consistent of the reversal of the patient symptoms (vision loss and headaches), the recurrence-free survival based on imaging, as well as the absence of any complications. Results  The patient's vision improved after the surgery; at his last follow-up there was no evidence of recurrence on imaging. Conclusions  The endoscopic endonasal approach is safe and effective in treating suprasellar craniopharyngiomas. The link to the video can be found at: https://youtu.be/p1VXbwnAWCo .

  17. Phosphorus-32 therapy for cystic craniopharyngiomas

    International Nuclear Information System (INIS)

    Barriger, Robert Bryan; Chang, Andrew; Lo, Simon S.; Timmerman, Robert D.; DesRosiers, Colleen; Boaz, Joel C.; Fakiris, Achilles J.

    2011-01-01

    Background and purpose: To examine control rates for predominantly cystic craniopharyngiomas treated with intracavitary phosphorus-32 (P-32). Material and methods: 22 patients with predominantly cystic craniopharyngiomas were treated at Indiana University between October 1997 and December 2006. Nineteen patients with follow-up of at least 6 months were evaluated. The median patient age was 11 years, median cyst volume was 9 ml, a median dose of 300 Gy was prescribed to the cyst wall, and median follow-up was 62 months. Results: Overall cyst control rate after the initial P-32 treatment was 67%. Complete tumor control after P-32 was 42%. Kaplan-Meier 1-, 3-, and 5-year initial freedom-from-progression rates were 68%, 49%, and 31%, respectively. Following salvage therapy, the Kaplan-Meier 1-, 3-, and 5-year ultimate freedom-from-progression rates were 95%, 95%, and 86%, respectively. All patients were alive at the last follow-up. Visual function was stable or improved in 81% when compared prior to P-32 therapy. Pituitary function remained stable in 74% of patients following P-32 therapy. Conclusions: Intracystic P-32 can be an effective and tolerable treatment for controlling cystic components of craniopharyngiomas as a primary treatment or after prior therapies, but frequently allows for progression of solid tumor components. Disease progression in the form of solid tumor progression, re-accumulation of cystic fluid, or development of new cysts may require further radiotherapy or surgical intervention for optimal long-term disease control.

  18. Recovery from diabetes insipidus and preservation of thyroid function after craniopharyngioma removal and pituitary stalk sectioning.

    Science.gov (United States)

    Ogawa, Yoshikazu; Niizuma, Kuniyasu; Tominaga, Teiji

    2017-11-01

    Craniopharyngioma is a slow-growing tumor, but long-term tumor control with maintenance of quality of life is sometimes very difficult to achieve, and hypothalamic disturbance should be strictly avoided in the treatment. However, management of the pituitary gland and/or pituitary stalk varies among surgeons and institutions. This retrospective review identified 44 patients, 24 males and 20 females with craniopharyngiomas who were initially treated by surgery through the extended transsphenoidal approach with pituitary stalk sectioning at a single institute. If the tumor bed involved the posterior lobe of the pituitary gland, pituitary stalk, anterior and/or mid portion of the third ventricle floor, these structures were removed en-bloc together with the tumor. The closest attention was paid to preserve fine arteries running along the surface of optic chiasm and the lateral walls of the third ventricle. Surgical outcome and changes in postoperative endocrinological status were investigated. Gross total removal was achieved in 40 of 44 patients (91%), and all patients could discharge without autonomic and/or thermal disturbances. Tumor remnants were identified with tight adhesion to the perforating arteries in 2 cases, tight adhesion to mammillary bodies in 1, and optic chiasm in 1. Administration of anti-diuretic hormone could be discontinued in 23 of 44 patients (52.3%) with improved diabetes insipidus (DI), although no patient could discontinue glucocorticoid administration. Preservation of thyroid function was achieved in another 23 of 44 patients (52.3%), and recovery from DI was correlated with preservation of thyroid function (p=0.016). Pituitary dysfunction is partially reversible even with pituitary stalk sectioning. Regrowth of tumor in the anterior and/or mid portion of the third ventricle floor including pituitary stalk can possibly be prevented by aggressive tumor removal, and co-achievement of long-term tumor control with maintenance of quality of life

  19. Early Ectopic Recurrence of Craniopharyngioma in the Cerebellopontine Angle.

    Science.gov (United States)

    Mahdi, Mohamad-Motaz Al; Krauss, Joachim K; Nakamura, Makoto; Brandis, Almuth; Hong, Bujung

    2018-01-01

    Ectopic recurrence of craniopharyngioma in the cerebellopontine angle after surgical resection of a suprasellar craniopharyngioma is rare. Thus, only 5 cases were reported with a delay ranging between 4 and 26 years after removal of the primary tumor. We report a unique case of ectopic recurrence of craniopharyngioma in the cerebellopontine angle, which occurred at only 4 months after surgical resection of the primary tumor. A 24-year-old man underwent resection of a suprasellar craniopharyngioma via a right pterional approach four months earlier. During follow-up, cerebral magnetic resonance imaging (MRI) showed a round homogeneous contrast-enhancing tumor in the right cerebellopontine angle with neither relation to the internal auditory canal nor to the dura mater. After microsurgical resection, histopathological findings revealed ectopic recurrence of craniopharyngioma with similar tumors like the primary tumor. Although infrequent, craniopharyngioma may disseminate via the cerebrospinal fluid during surgical resection and grow in an ectopic place. Early follow-up and MRI scan following resection of a craniopharyngioma is recommended.

  20. Craniopharyngioma: treatment by conservative surgery and radiation therapy.

    Directory of Open Access Journals (Sweden)

    Nagpal R

    1992-10-01

    Full Text Available Benign neoplasms are curable only when excised. This applies even to craniopharyngiomas. The proximity of craniopharyngiomas to the hypothalamus and neurovascular structures makes total excision difficult to achieve. Over the last 3-4 decades, it has become increasingly obvious that craniopharyngiomas respond to radiation therapy. Early, unhappy results with major excisions have prompted us to adopt a policy of conservative surgery and radiation therapy to the residual tumour. Preliminary results suggest a good outcome in 35 of the 63 patients so treated from 1981. Details of the study are presented.

  1. Cystic craniopharyngioma: intratumoral chemotherapy with alpha interferon

    Directory of Open Access Journals (Sweden)

    Patrícia Alessandra Dastoli

    2011-02-01

    Full Text Available OBJECTIVE: To assess whether the cystic craniopharyngiomas can be controlled with the use of intratumoral applications of interferon alpha. METHOD: Nineteen patients with the diagnosis of cystic craniopharyngioma were treated with intratumoral chemotherapy with interferon alpha from January 2002 to April 2006. All patients underwent placement of an intracystic catheter connected to an Ommaya reservoir. Through this reservoir were made applications during chemotherapy cycles. Each cycle corresponded to application of 3,000,000 units of interferon alpha three times per week on alternate days totalizing 36,000,000 units. Response to treatment was evaluated by calculating the tumor volume on MRI control after one, three and six months after the end of each cycle. Patients who developed worsening of symptoms or who had insignificant reduction in tumor volume during follow-up underwent repeat cycle chemotherapy. RESULTS: Four patients received four cycles of chemotherapy, three patients received three cycles, six patients received two cycles and six patients received one. The lower percentage of reduction in tumor volume was 60% and the bigger reduction was 98.37%. Eleven patients had a reduction greater than 90%. Five patients had a tumor reduction between 75 and 90% and in three patients the tumors were reduced by less than 75%. No deaths occurred during treatment and side effects of interferon alpha were well tolerated. No treatment was discontinued. Follow-up after the last application ranged from one year and five months to three years and nine months. CONCLUSION: The intratumoral chemotherapy with interferon alpha decreases the volume of cystic craniopharyngiomas and so far can be considered a new therapeutic alternative.

  2. Extended Endoscopic Endonasal Approach for Craniopharyngioma Removal.

    Science.gov (United States)

    Messerer, Mahmoud; Maduri, Rodolfo; Daniel, Roy Thomas

    2018-02-01

    Objective  Endoscopic transsphenoidal extended endoscopic approach (EEA) represents a valid alternative to microsurgery for craniopharyngiomas removal, especially for retrochiasmatic lesions without large parasellar extension. The present video illustrates the salient surgical steps of the EEA for craniopahryngioma removal. Patient  A 52-year-old man presented with a bitemporal hemianopia and a bilateral decreased visual acuity. MRI showed a Kassam type III cystic craniopharyngioma with a solid component ( Fig. 1 , panels A and B). Surgical Procedure  The head is rotated 10 degrees toward the surgeons. The nasal step is started through the left nostril with a middle turbinectomy. A nasoseptal flap is harvested and positioned in the left choana. The binostril approach allows a large sphenoidotomy to expose the key anatomic landmarks. The craniotomy boundaries are the planum sphenoidale superiorly, the median opticocarotid recesses, the internal carotid artery laterally and the clival recess inferiorly. After dural opening and superior intercavernous sinus coagulation, the tumor is entirely removed ( Fig. 2 , panels A and B). Skull base reconstruction is ensured by fascia lata grafting and nasoseptal flap positioning. Results  Postoperative MRI showed the complete tumor resection ( Fig. 1 , panels C and D). At 3 months postoperatively, the bitemporal hemianopia regressed and the visual acuity improved. A novel left homonymous hemianopia developed secondary to optic tract manipulation. Conclusions  The extended EEA is a valid surgical approach for craniopharyngioma resection. A comprehensive knowledge of the sellar and parasellar anatomy is mandatory for safe tumor removal with decreased morbidity and satisfactory oncologic results. The link to the video can be found at: https://youtu.be/NrCPPnVK2qA .

  3. The role of radiation treatment in craniopharyngioma

    International Nuclear Information System (INIS)

    Chin, H.W.; Maruyama, Y.; Young, B.

    1983-01-01

    The long natural course of craniopharyngioma and short-term follow-up period in many reports make comparison of various treatment results difficult. Some patients may enjoy virtually symptom-free lives despite known recurrence. Some patients with recurrence may have a good response to retreatment. Such unpredictable behavior and treatment responses have led to considerable disparity in clinical reports concerning the best treatment method. Treatments using surgery alone and/or low dose postoperative radiation treatment could prolong survival time, but may not prevent recurrence leading to ultimate failure. High dose postoperative radiotherapy following radical surgery should be an ideal approach in dealing with this tumor. (orig.) [de

  4. Role of radiation treatment in craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Chin, H.W.; Maruyama, Y.; Young, B.

    1983-12-01

    The long natural course of craniopharyngioma and short-term follow-up period in many reports make comparison of various treatment results difficult. Some patients may enjoy virtually symptom-free lives despite known recurrence. Some patients with recurrence may have a good response to retreatment. Such unpredictable behavior and treatment responses have led to considerable disparity in clinical reports concerning the best treatment method. Treatments using surgery alone and/or low dose postoperative radiation treatment could prolong survival time, but may not prevent recurrence leading to ultimate failure. High dose postoperative radiotherapy following radical surgery should be an ideal approach in dealing with this tumor.

  5. Solid craniopharyngiomas treated by stereotactic radiosurgery

    International Nuclear Information System (INIS)

    Backlund, E.-O.

    1979-01-01

    The radiological changes of solid craniopharyngiomas treated by stereotactic radiosurgery have been followed. Nine cases are considered, the patients having received gamma radiation treatment with a dose distribution permitting no part of the tumour to receive doses less than 2-3 Gy. Target doses were 20 to 50 Gy. Tumour shrinkage was registered and no complications which could be attributed with certainty to the irradiation were encountered. The results did not allow an optimal single dose to be determined with accuracy but vaguely indicated that lower doses than those used are sufficient for desired effect on the tumour without jeopardizing its surroundings. (Auth./C.F.)

  6. Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults

    DEFF Research Database (Denmark)

    Nielsen, E H; Feldt-Rasmussen, U; Poulsgaard, L

    2011-01-01

    Registry, the Danish Cancer Registry and regional registries. Medical records were reviewed. Danish population data were obtained from Statistics Denmark. European and World population data were obtained from EU and WHO homepages. Prior studies providing data on craniopharyngioma IRs were identified via...... PubMed and, if appropriate, were included in a weighted analysis estimating overall and children's IRs of craniopharyngioma. IRs are given as new cases per million per year. We identified 189 patients with new verified (162) or probable craniopharyngioma. The overall WHO World-standardised incidence...

  7. Oxytocin in survivors of childhood-onset craniopharyngioma

    NARCIS (Netherlands)

    Daubenbuechel, Anna M. M.; Hoffmann, Anika; Eveslage, Maria; Oezyurt, Jale; Lohle, Kristin; Reichel, Julia; Thiel, Christiane M.; Martens, Henri; Geenen, Vincent; Mueller, Hermann L.

    2016-01-01

    Quality of survival of childhood-onset craniopharyngioma patients is frequently impaired by hypothalamic involvement or surgical lesions sequelae such as obesity and neuropsychological deficits. Oxytocin, a peptide hormone produced in the hypothalamus and secreted by posterior pituitary gland, plays

  8. Infrasellar craniopharyngioma of the posterior nasal septum: a rare entity.

    Science.gov (United States)

    Chiun, Kian Chai; Tang, Ing Ping; Vikneswaran, Tharumalingam; Nurshaline Pauline, H Kipli

    2012-02-01

    To report an unusual location of infrasellar craniopharyngioma in a peadiatric patient. A six-year-old boy presented with persistent bilateral nasal obstruction for one year. Clinical examination revealed a posterior choanal mass arising from septum and the finding was confirmed by paranasal sinuses computed tomography scan. He then underwent wide local excision. Histopathological examination confirmed the diagnosis of craniopharyngioma (adamantinomatous type). There were no signs and symptoms of recurrence after a year of followup. Infracranial craniopharyngioma without sellar involvement is extremely rare. Persistent nasal obstruction without endocrine dysfunction is the common presentation. Radiological imaging is important to diagnose and assess the extent. The mainstay of treatment for infrasellar craniopharyngioma is surgery. Regular follow up is mandatory.

  9. Oxytocin in survivors of childhood-onset craniopharyngioma

    NARCIS (Netherlands)

    Daubenbuechel, Anna M. M.; Hoffmann, Anika; Eveslage, Maria; Oezyurt, Jale; Lohle, Kristin; Reichel, Julia; Thiel, Christiane M.; Martens, Henri; Geenen, Vincent; Mueller, Hermann L.

    Quality of survival of childhood-onset craniopharyngioma patients is frequently impaired by hypothalamic involvement or surgical lesions sequelae such as obesity and neuropsychological deficits. Oxytocin, a peptide hormone produced in the hypothalamus and secreted by posterior pituitary gland, plays

  10. Metastatic papillary craniopharyngioma: case study and study of tumor angiogenesis.

    Science.gov (United States)

    Elmaci, Lhan; Kurtkaya-Yapicier, Ozlem; Ekinci, Gazanfer; Sav, Aydin; Pamir, M. Necmettin; Vidal, Sergio; Kovacs, Kalman; Scheithauer, Bernd W.

    2002-01-01

    We report a case of suprasellar papillary craniopharyngioma metastatic to the temporoparietal region 2 years after its initial resection. The literature documents examples of craniopharyngioma recurrences along the surgical tract, as well as remote ipsi- and contralateral metastases via cerebrospinal fluid seeding. Ours is the second report of a craniopharyngioma of papillary type to exhibit metastatic behavior. The tumor spread opposite the side of craniotomy. Although a rare occurrence, it confirms the limited capacity of histologically benign craniopharyngiomas to undergo meningeal seeding, likely the result of surgical manipulation. Immunohistochemical demonstration of increased microvascular density and vascular endothelial growth factor expression, as well as a high vascular endothelial growth receptor (VEGFR2) signal by in situ hybridization, suggests that tumor vascularity facilitated angiogenesis and may have been involved in the establishment and growth of the metastatic deposit. PMID:11916504

  11. Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

    Science.gov (United States)

    Daubenbüchel, Anna M. M.; Müller, Hermann L.

    2015-01-01

    Childhood-onset craniopharyngiomas are partly cystic embryonic malformations of the sellar/parasellar region. The therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining optical and hypothalamic neuroendocrine functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), a limited hypothalamus-sparing surgical strategy followed by local irradiation is recommended. Involvement and/or surgical lesions of posterior hypothalamic areas cause major neuroendocrine sequelae. The overall survival rates are high (92%) but neuroendocrine disorders such as obesity and metabolic syndrome due to involvement and/or treatment-related hypothalamic lesions have major negative impact on survival and quality of life. Recurrences and progressions are frequent post-surgical events. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007). Childhood-onset craniopharyngioma should be recognized as a chronic disease requiring treatment and constant monitoring of the clinical and quality of life consequences, frequently impaired due to neuroendocrine disorders, by experienced multidisciplinary teams in order to provide optimal care of surviving patients. PMID:26239246

  12. Stereotaxic intracavitary irradiation for cystic craniopharyngiomas

    International Nuclear Information System (INIS)

    Pollack, I.F.; Lunsford, L.D.; Slamovits, T.L.; Gumerman, L.W.; Levine, G.; Robinson, A.G.

    1988-01-01

    Stereotaxic intracavitary irradiation with instillation of phosphorus-32 ( 32 P) colloidal chromic phosphate was performed in nine patients with cystic craniopharyngiomas. Serial neurological, ophthalmological, neuroendocrinological, and radiological examinations were performed before and after treatment. Dosimetry was determined based on a computerized tomography (CT) estimation of tumor volume, and was calculated to provide a tumoricidal dose (200 to 300 Gy) to the cyst wall. The follow-up period ranged from 14 to 45 months (mean 27 months). After treatment, all nine patients showed improvement of symptoms and radiological evidence of cyst regression. Because of an expanding solid component producing recurrent symptoms, one patient required a craniotomy 14 months after isotope instillation. Three of five patients with impaired visual acuity before surgery had significant improvement in acuity after treatment. Preoperative visual field defects in eight patients improved in four after 32 P therapy. Of seven patients with preoperative endocrine abnormalities, one individual showed almost complete normalization and another had improvement in endocrine function. Patients who exhibited residual neuroendocrine function before isotope instillation developed no significant deterioration in endocrine status during the follow-up period. The findings suggest that stereotaxic intracavitary irradiation is a safe and effective treatment which should be considered as the initial surgery for cystic craniopharyngiomas

  13. Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

    Directory of Open Access Journals (Sweden)

    Anna M. M. Daubenbüchel

    2015-03-01

    Full Text Available Childhood-onset craniopharyngiomas are partly cystic embryonic malformations of the sellar/parasellar region. The therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining optical and hypothalamic neuroendocrine functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement, a limited hypothalamus-sparing surgical strategy followed by local irradiation is recommended. Involvement and/or surgical lesions of posterior hypothalamic areas cause major neuroendocrine sequelae. The overall survival rates are high (92% but neuroendocrine disorders such as obesity and metabolic syndrome due to involvement and/or treatment-related hypothalamic lesions have major negative impact on survival and quality of life. Recurrences and progressions are frequent post-surgical events. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007. Childhood-onset craniopharyngioma should be recognized as a chronic disease requiring treatment and constant monitoring of the clinical and quality of life consequences, frequently impaired due to neuroendocrine disorders, by experienced multidisciplinary teams in order to provide optimal care of surviving patients.

  14. Role of gamma knife radiosurgery in craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Ashis Patnaik

    2013-01-01

    Full Text Available Craniopharyngiomas continue to be a challenge to manage, due to difficulty in complete excision and frequent recurrences. The management protocol remains controversial. They have a tendency to invade the normal brain tissues around them and due to their position in suprasellar region in close relationship with vital structures like optic apparatus, pituitary-hypothalamic axis, complete removal is often not feasible without causing serious morbidity and mortality. In this scenario, sub-total excision seems to be a better alternative, which is plagued by early and frequent recurrences. Radiotherapy has been used for increasing the progression free survival and to improve the overall quality of life. Recently Gamma knife radiosurgery has evolved as a promising technique of radiating the residual or recurrent tumor in a single session with great accuracy and precision. This helps in maximizing the radiation dose to the tumor with steep dose fall off to the surrounding tissue, and hence there is better control of the tumor and minimal radiation exposure to surrounding normal, vital brain tissues. We discuss the current strategies of Gamma knife treatment for craniopharyngioma and review the literature.

  15. Oxytocin in survivors of childhood-onset craniopharyngioma.

    Science.gov (United States)

    Daubenbüchel, Anna M M; Hoffmann, Anika; Eveslage, Maria; Özyurt, Jale; Lohle, Kristin; Reichel, Julia; Thiel, Christiane M; Martens, Henri; Geenen, Vincent; Müller, Hermann L

    2016-11-01

    Quality of survival of childhood-onset craniopharyngioma patients is frequently impaired by hypothalamic involvement or surgical lesions sequelae such as obesity and neuropsychological deficits. Oxytocin, a peptide hormone produced in the hypothalamus and secreted by posterior pituitary gland, plays a major role in regulation of behavior and body composition. In a cross-sectional study, oxytocin saliva concentrations were analyzed in 34 long-term craniopharyngioma survivors with and without hypothalamic involvement or treatment-related damage, recruited in the German Childhood Craniopharyngioma Registry, and in 73 healthy controls, attending the Craniopharyngioma Support Group Meeting 2014. Oxytocin was measured in saliva of craniopharyngioma patients and controls before and after standardized breakfast and associations with gender, body mass index, hypothalamic involvement, diabetes insipidus, and irradiation were analyzed. Patients with preoperative hypothalamic involvement showed similar oxytocin levels compared to patients without hypothalamic involvement and controls. However, patients with surgical hypothalamic lesions grade 1 (anterior hypothalamic area) presented with lower levels (p = 0.017) of oxytocin under fasting condition compared to patients with surgical lesion of posterior hypothalamic areas (grade 2) and patients without hypothalamic lesions (grade 0). Craniopharyngioma patients' changes in oxytocin levels before and after breakfast correlated (p = 0.02) with their body mass index. Craniopharyngioma patients continue to secrete oxytocin, especially when anterior hypothalamic areas are not involved or damaged, but oxytocin shows less variation due to nutrition. Oxytocin supplementation should be explored as a therapeutic option in craniopharyngioma patients with hypothalamic obesity and/or behavioral pathologies due to lesions of specific anterior hypothalamic areas. Clinical trial number: KRANIOPHARYNGEOM 2000/2007(NCT00258453; NCT01272622).

  16. Ectopic recurrence of craniopharyngioma: Reporting three new cases.

    Science.gov (United States)

    Yang, Yang; Shrestha, David; Shi, Xiang-En; Zhou, Zhongqing; Qi, Xueling; Qian, Hai

    2015-04-01

    Ectopic recurrence of craniopharyngioma is extremely rare following transcranial procedures of primary tumour. Here we describe 3 new cases of ectopic recurrence along the surgical route after transcranial gross total resection of primary tumour. All 3 cases are male adults--2 of them had papillary-type tumour with the other being adamantinomatous. All ectopic tumours were safely resected via repeated craniotomy. Long-term surveillance of patients with resected craniopharyngioma is essential.

  17. Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

    Science.gov (United States)

    Lee, Young-Hen; Lim, Dong-Jun; Park, Jung-Yul; Chung, Yong-Gu; Kim, Young-Sik

    2009-01-01

    We report a rare case of petroclival craniopharyngioma with no connection to the sellar or suprasellar region. MRI and CT images revealed a homogenously enhancing retroclival solid mass with aggressive skull base destruction, mimicking chordoma or aggressive sarcoma. However, there was no calcification or cystic change found in the mass. Here, we report the clinical features and radiographic investigation of this uncommon craniopharyngioma arising primarily in the petroclival region. PMID:19881982

  18. Long-term outcome of craniopharyngioma in children. A review

    International Nuclear Information System (INIS)

    Tamiya, Takashi; Okada, Masaki; Miyake, Keisuke; Kawai, Nobuyuki

    2011-01-01

    The treatment for pediatric craniopharyngioma remains challenging and controversial. Although this tumor is histologically benign, the treatments include radical surgery, conservative surgery, radiotherapy, intracystic chemotherapy and multimodality approaches. In addition, the long-term functional outcomes including visual function, endocrine function, cognitive function, hypothalamic function, and quality of life are complex and major problems among survivors. In this paper, we reviewed the recent treatments for pediatric craniopharyngioma and the long-term outcomes after treatment in literatures. (author)

  19. Preliminary characterization of slow growing rhizobial strains ...

    African Journals Online (AJOL)

    COMPAQ

    2016-05-18

    May 18, 2016 ... number of nodules was equal to 5.6 nodules per plant for the strain RMB1 from Bousfer site. The intrinsic .... The salt tolerance of rhizobia was tested on YMA plates containing ..... through deep explorations searching for an.

  20. Preliminary characterization of slow growing rhizobial strains ...

    African Journals Online (AJOL)

    COMPAQ

    2016-05-18

    May 18, 2016 ... strains had a very slow growth rate in yeast malt (YM) agar medium, forming colonies less than 1 mm in ... dominant genus of symbiotic nitrogen-fixing bacteria ... Single colonies were picked up and checked for purity by.

  1. Sir Victor Horsley: pioneer craniopharyngioma surgeon.

    Science.gov (United States)

    Pascual, José M; Prieto, Ruth; Mazzarello, Paolo

    2015-07-01

    Sir Victor Horsley (1857-1916) is considered to be the pioneer of pituitary surgery. He is known to have performed the first surgical operation on the pituitary gland in 1889, and in 1906 he stated that he had operated on 10 patients with pituitary tumors. He did not publish the details of these procedures nor did he provide evidence of the pathology of the pituitary lesions operated on. Four of the patients underwent surgery at the National Hospital for Neurology and Neurosurgery (Queen Square, London), and the records of those cases were recently retrieved and analyzed by members of the hospital staff. The remaining cases corresponded to private operations whose records were presumably kept in Horsley's personal notebooks, most of which have been lost. In this paper, the authors have investigated the only scientific monograph providing a complete account of the pituitary surgeries that Horsley performed in his private practice, La Patologia Chirurgica dell'Ipofisi (Surgical Pathology of the Hypophysis), written in 1911 by Giovanni Verga, Italian assistant professor of anatomy at the University of Pavia. They have traced the life and work of this little-known physician who contributed to the preservation of Horsley's legacy in pituitary surgery. Within Verga's pituitary treatise, a full transcription of Horsley's notes is provided for 10 pituitary cases, including the patients' clinical symptoms, surgical techniques employed, intraoperative findings, and the outcome of surgery. The descriptions of the topographical and macroscopic features of two of the lesions correspond unmistakably to the features of craniopharyngiomas, one of the squamous-papillary type and one of the adamantinomatous type. The former lesion was found on necropsy after the patient's sudden death following a temporal osteoplastic craniectomy. Surgical removal of the lesion in the latter case, with the assumed nature of an adamantinomatous craniopharyngioma, was successful. According to the

  2. Trans-sphenoidal excision of craniopharyngiomas.

    Directory of Open Access Journals (Sweden)

    Nagpal R

    1991-04-01

    Full Text Available Craniopharyngiomas have been by and large excised by the transcranial route. Since 1982, 11 patients have been operated by the traus-sphensidal route in the department. The clinical features with which they were presented, diagnostic investigations, details of surgical procedures and follow-up analysis is being presented here. A retrospective study of radiological investigation was done to determine the features that help decide the choice of surgical approach to these lesions. Only lesions that were primarily intrasellar, cystic and those that expanded the sella could be treated by the trans-sphenoidal route. Associated suprasellar extensions could also be removed. Predominantly calcified or firm, fleshy tumours lent themselves poorly to removal by the trans-sphenoidal route.

  3. Anaesthetic management of children with craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Srilata Moningi

    2017-01-01

    Full Text Available The perioperative management of craniopharyngioma in children is quite challenging not only to the neurosurgeons and anaesthesiologists but also to the oncologists, endocrinologists and intensivists. The various concerns include size of the tumour and its effects on intracranial pressure, vicinity of the tumour to nearby vessels and neural structures, endocrine disturbances, compressive symptoms, paediatric age, obesity-related problems, high recurrence rate of the tumour, undesired effects of radiotherapy, high morbidity and mortality. As anaesthesiologists, we deal with most of the challenges. The success of the outcome depends on the proper identification of the patient, pre-operative optimisation, surgical decision, awareness of the complications, timely intervention and optimal post-operative care.

  4. Transcallosal, Transchoroidal Resection of a Recurrent Craniopharyngioma.

    Science.gov (United States)

    Jean, Walter C

    2018-04-01

    Objective  To demonstrate the transchoroidal approach for the resection of a recurrent craniopharyngioma. Design  Video case report. Setting  Microsurgical resection. Participant  The patient was a 27-year-old woman with a history of a craniopharyngioma, resected twice during the year prior to presentation to our unit. Both operations were done via the left anterolateral corridor, and afterward, she was blind in the left eye and was treated with Desmopressin (DDAVP) for diabetes insipidus (DI). Serial magnetic resonance imaging (MRI) showed progression of the tumor residual, and she was referred for further surgical intervention. Main Outcome Measures  Pre- and postoperative MRIs measured the degree of resection. Results  For this, her third surgery, a transcallosal, transchoroidal approach, was chosen to offer the widest possible exposure. Given her history, an aggressive total resection was the best strategy. The patient was placed supine with the head neutral. A right frontal craniotomy allowed access to the interhemispheric fissure. By opening the corpus callosum, the left lateral ventricle was entered. The transchoroidal approach started with dissection of the tenia fornicis to open the choroidal fissure. After this, sufficient exposure to the posterior parts of the tumor was gained. Resection proceeded to the bottom of the tumor, exposing the basilar apex and interpeduncular cistern, and continued back anteriorly. In the end, a microscopic total resection was achieved. With a long hospital stay to treat her brittle DI, the patient slowly returned to neurological baseline. Conclusion  The transchoroidal approach is an effective way to remove large tumors in the third ventricle. The link to the video can be found at: https://youtu.be/2-Aqjaay8dg .

  5. Extended Endoscopic Endonasal Approach for Suprasellar Craniopharyngioma.

    Science.gov (United States)

    Locatelli, D; Pozzi, F; Agresta, G; Padovan, S; Karligkiotis, A; Castelnuovo, P

    2018-02-01

    Objectives  We illustrate a suprasellar craniopharyngiomas treated with an extended endoscopic endonasal approach (EEEA). Design  Case report of a 43-year-old male affected by cerebral lesion located in suprasellar region involving the third ventricle and compressing the neurovascular structures, causing an anterosuperior dislocation of the chiasma. There is a complete disruption of the pituitary stalk that can explain the clinical finding of partial anterior hypopituitarism and hyperprolactinemia. The lesion is characterized by a solid and cystic component. Considering the absence of lateral extension and the suprasellar location of the lesion, an EEEA is preferred. Setting  University Hospital "Ospedale di Circolo," Department of Neurosurgery, Varese, Italy. Participants  Neurosurgical and ENT Skull Base Team. Main Outcome Measures  A bilateral parasagittal approach is performed using a four-hand technique. The first step of the surgery is the preparation of the Hadad's flap. The approach is extended to the planum sphenoidalis to expose the suprasellar region. The lesion is completely removed employing also an ultrasound aspirator. Skull base reconstruction is performed with three-layer technique: graft of fat tissue, fascia lata, and nasoseptal flap. Results  No postoperative complications occurred. In the post-op, the patient presents a panhypopituitarism and an improvement in neurological status. The visual deficit remains stable. Post-op magnetic resonance imaging at 1 year documents the complete absence of pathological contrast enhancement. Conclusions  EEEA is a feasible approach in treating craniopharyngioma with suprasellar extension. The advantages include optimal visualization, good resection rate, and absence of brain retraction. The link to the video can be found at: https://youtu.be/IYm-8P1jbBo .

  6. Endoscopic Endonasal Approach to a Suprasellar Craniopharyngioma.

    Science.gov (United States)

    Todeschini, Alexandre B; Montaser, Alaa S; Shahein, Mostafa; Revuelta, Juan Manuel; Otto, Bradley A; Carrau, Ricardo L; Prevedello, Daniel M

    2018-04-01

    We present the case of a 57-year-old male who presented with progressive right side vision loss whose workup revealed a large suprasellar lesion with invasion of the third ventricle. The pituitary stalk was not visible. Hormonal panel showed no hormonal deficits. The initial diagnosis was of a type II transinfundibular craniopharyngioma (as classified by Kassam et al). An endoscopic endonasal transplanum transtuberculum approach was done using a standard binostril four-hand technique, with the patient positioned supine with the head turned to the right side and tilted to the left, fixed in a three-pin head clamp, under imaging guidance. The tumor was carefully dissected away from the optic apparatus while preserving the vessels, mainly the superior hypophyseal artery. The stalk was identified around the tumor and preserved. The third ventricle was entered and inspected at the end of the procedure and a near-total resection (a small residual in the right hypothalamus) with decompression of the optic apparatus was achieved. Reconstruction was done in a multilayered fashion, using collagen matrix and a nasoseptal flap. Patient had an uneventful postoperative stay and was discharged on POD 4, neurologically stable with no hormonal deficits. Pathology confirmed an adamantinomatous craniopharyngioma. Due to a small growth of the residual, patient underwent fractionated stereotactic radiation (50.4Gy in 28 sessions). He presented with panhypopituitarism 2 years after radiation therapy. At 3-month follow-up, his vision was back to normal and 6-year postoperative magnetic resonance imaging showed no signs of recurrence. The link to the video can be found at: https://youtu.be/chG7XIz7a_A .

  7. Fever of unknown origin as a presenting manifestation of craniopharyngioma in a child

    Directory of Open Access Journals (Sweden)

    Madhumita Nandi and Rakesh Kumar Mondal

    2010-01-01

    Full Text Available An unusual case of cranio-pharyngioma which presented with prolonged fever described here. Investigation revealed that the child was suffering from leaking craniopharyngioma with hypo-pituitarism Fever was due to chemical meningitis following cranio-pharyngioma as evident from the CSF findings. Craniopharyngioma can cause prolonged or recurrent fever due to various reasons. Prolonged fever as the sole manifestation of cranio-pharyngioma has been rarely reported in literature and this is probably first such report in Indian children.

  8. Craniopharyngioma arising in a Rathke's cleft cyst: case report.

    Science.gov (United States)

    Alomari, Ahmed K; Kelley, Brian J; Damisah, Eyiyemisi; Marks, Asher; Hui, Pei; DiLuna, Michael; Vortmeyer, Alexander

    2015-03-01

    Craniopharyngioma is one of the most common non-glial intracranial tumors of childhood. Its relation to Rathke's cleft cyst (RCC) is controversial, and both lesions have been hypothesized to lie on a continuum of cystic ectodermal lesions of the sellar region. The authors report on a 7-year-old boy who presented with decreased visual acuity, presumably of at least 2 years' duration, and was found to have a 5.2-cm sellar lesion with rim enhancement. Histological examination of the resected lesion showed a mixture of areas with simple RCC morphology with focal squamous metaplasia and areas with typical craniopharyngioma morphology. Immunohistochemical staining with CK20 and Ki 67 differentially highlighted the 2 morphological components. Testing for beta-catenin and BRAF mutations was negative in the craniopharyngioma component, precluding definitive molecular classification. Follow-up imaging showed minimal residual enhancement and the patient will be closely followed up with serial MRI. Given the clinical and histological findings in the case, a progressive transformation of the RCC to craniopharyngioma seems to be the most plausible explanation for the co-occurrence of the 2 lesion types in this patient. An extensive review of previously proposed theories of the relationship between craniopharyngioma and RCC is also presented.

  9. The limits of transsellar/transtuberculum surgery for craniopharyngioma.

    Science.gov (United States)

    Koutourousiou, Maria; Fernandez-Miranda, Juan C; Wang, Eric W; Snyderman, Carl H; Gardner, Paul A

    2018-06-01

    The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging brain tumors to treat. Although surgery remains the first line of therapy and offers the best chance of radical resection and oncological cure, the high recurrence tendency of craniopharyngiomas, even after apparent total removal, often makes adjuvant treatment essential. The endoscopic endonasal approach (EEA) has been recently introduced as a treatment option for both pediatric and adult craniopharyngiomas, rapidly gaining wide acceptance over the traditional transcranial approaches. Although the primary role of EEA over traditional transcranial approaches has been slowly accepted in the literature, little has been written about the limitations and potential contraindications of this approach in the treatment of craniopharyngiomas. This article presents the advantages and highlights the limitations of endoscopic transsellar/transtuberculum surgery for craniopharyngiomas. In every case, surgery should be tailored to individuals based on their age and comorbidities, presenting symptoms, tumor characteristics, prior treatment and treatment tolerance, as well as the surgeon's preference based on personal experience and comfort.

  10. Expanded Endonasal Endoscopic Approach for Resection of an Infrasellar Craniopharyngioma.

    Science.gov (United States)

    Abou-Al-Shaar, Hussam; Blitz, Ari M; Rodriguez, Fausto J; Ishii, Masaru; Gallia, Gary L

    2016-11-01

    Craniopharyngiomas are uncommon benign locally aggressive epithelial tumors mostly located in the sellar and suprasellar regions. An infrasellar origin of these tumors is rare. The authors report a 22-year-old male patient with a purely infrasellar adamantinomatous craniopharyngioma centered in the nasopharynx with extension into the posterior nasal septum, sphenoid sinus, and clivus. Gross total resection was achieved using an expanded endonasal endoscopic transethmoidal, transsphenoidal, transpterygoid, and transclival approach. Follow-up at one year demonstrated no evidence of disease recurrence. Infrasellar craniopharyngioma should be included in the differential diagnosis of sinonasal masses even in the absence of sellar extension. Expanded endonasal endoscopic approaches provide excellent access to and visualization of such lesions and may obviate the need for postoperative radiotherapy when gross total resection is achieved. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Microsurgical Resection of Suprasellar Craniopharyngioma-Technical Purview.

    Science.gov (United States)

    Nanda, Anil; Narayan, Vinayak; Mohammed, Nasser; Savardekar, Amey R; Patra, Devi Prasad

    2018-04-01

    Objectives  Complete surgical resection is an important prognostic factor for recurrence and is the best management for craniopharyngioma. This operative video demonstrates the technical nuances in achieving complete resection of a suprasellar craniopharyngioma. Design and Setting  The surgery was performed in a middle-aged lady who presented with the history of progressive bitemporal hemianopia and excessive sleepiness over 8 months. On imaging, suprasellar craniopharyngioma was identified. The tumor was approached through opticocarotid cistern and lamina terminalis. Exposure of bilateral optic nerves, right internal carotid artery, anterior cerebral artery, and its perforator branches was then afforded and the tumor was gross totally resected. Results  The author demonstrates step-by-step technique of microsurgical resection of suprasellar craniopharyngioma. The narrow corridor to deeper structures, intricacies of multiple perforator vessels, and the technique of arachnoid and capsule dissection are the main challenging factors for the gross total resection of craniopharyngioma. The tumor portion which lies under the ipsilateral optic nerve is a blind spot region with a high chance of leaving residual tumor. Mobilization of optic nerve may endanger visual function too. The use of handheld mirror ['mirror-technique'] helps in better visualization of this blind spot and achieve complete excision. Conclusions  The technical pearls of craniopharyngioma surgery include the optimum utilization of translamina terminalis route, wide opening of the cisterns, meticulous separation of deep perforator vessels, capsular mobilization/traction avoidance, and the use of "mirror-technique" for blind-spot visualization. These surgical strategies help to achieve complete resection without causing neurological deficit. The link to the video can be found at: https://youtu.be/9wHJ4AUpG50 .

  12. [Acute visual loss in pregnancy caused by craniopharyngioma].

    Science.gov (United States)

    Grillo-Mallo, E; Jiménez-Benito, J; Diéz-Feijóo, E; Alonso Alonso, I; Ferrero Collado, A; Muñoz Quiñones, S

    2014-04-01

    A 38-year-old female, at 20-weeks gestation, experienced a sudden visual loss and visual-field abnormalities. The neuroimaging tests showed a craniopharyngioma. Surgical removal was performed with a successful outcome as regards the pregnancy and visual function. It is known that pituitary adenomas may grow during pregnancy; however this is unusual in craniopharyngiomas. They usually present with visual problems due to their suprasellar topography. Surgery is the treatment of choice, the outcome essentially depending on its complete resection. Copyright © 2010 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  13. Haemorrhagic Presentation of a Craniopharyngioma in a Pregnant Woman

    Directory of Open Access Journals (Sweden)

    Cesare Zoia

    2014-01-01

    Full Text Available Objective. Craniopharyngioma is a rare tumour, and, consequently, acute clinical presentation and diagnosis, during pregnancy, of this pathology are quite difficult to find. Only few cases are reported in the literature, and no one describes these two conditions in association. Methods. We report a particular case of craniopharyngioma presenting both of the above conditions. Results. The patient was successfully operated with endoscopic technique. Conclusions. Rare and difficult cases, created by the superposition of different clinical conditions, need multidisciplinary management, with collaboration, integration, and cooperation between different medical specialists.

  14. All-trans retinoic acid inhibits craniopharyngioma cell growth: study on an explant cell model.

    Science.gov (United States)

    Li, Qiang; You, Chao; Zhou, Liangxue; Sima, Xiutian; Liu, Zhiyong; Liu, Hao; Xu, Jianguo

    2013-05-01

    The ratio between FABP5 and CRABPII determines cellular response to physiological level of retinoic acid; tumor cells undergo proliferation with high level of FABP5 and apoptosis with high level of CRABPII. We intended to study FABP5 and CRABPII expression in craniopharyngiomas, to establish craniopharyngioma cell model using explants method, and to study the effect of pharmacological dose of retinoic acid on craniopharyngioma cells. Expression of FABP5 and CRABPII in craniopharyngioma tissue from 20 patients was studied using immunohistochemistry. Primary craniopharyngioma cell cultures were established using tissue explants method. Craniopharyngioma cells were treated using various concentrations of all-trans retinoic acid, and cell growth curve, apoptosis, expression of FABP5, CRABPII and NF-κB were assayed in different groups. FABP5/CRABPII ratio was significantly higher in adamatinomatous group than that in papillary group. Cell cultures were established in 19 cases (95 %). Pharmacological level retinoic acid inhibited cell growth and induced cellular apoptosis in dose dependent manner, and apoptosis rate cells treated with 30 μM retinoic acid for 24 h was 43 %. Also, retinoic acid increased CRABPII, and decreased FABP5 and NF-κB expression in craniopharyngioma cells. High FABP5/CRABPII ratio is observed in adamatinomatous craniopharyngioma. Retinoic acid at pharmacological level induced craniopharyngioma cell apoptosis via increasing FABP5/CRABPII ratio and inhibiting NF-κB signaling pathway. Our study demonstrated that all-trans retinoic acid might be a candidate for craniopharyngioma adjuvant chemotherapy in future.

  15. Orbitopterional Craniotomy Resection of Pediatric Suprasellar Craniopharyngioma.

    Science.gov (United States)

    LeFever, Devon; Storey, Chris; Guthikonda, Bharat

    2018-04-01

    The orbitopterional approach provides an excellent combination of basal access and suprasellar access. This approach also allows for less brain retraction when resecting larger suprasellar tumors that are more superiorly projecting due to a more frontal and inferior trajectory. In this operative video, the authors thoroughly detail an orbitopterional craniotomy utilizing a one-piece modified orbitozygomatic technique. This technique involves opening the craniotomy through a standard pterional incision. The craniotomy is performed using the standard three burr holes of a pterional approach; however, the osteotomy is extended anteriorly through the frontal process of the zygomatic bone as well as through the supraorbital rim. In this operative video atlas, the authors illustrate the operative anatomy, as well as surgical strategy and techniques to resect a large suprasellar craniopharyngioma in a 4-year-old male. Other reasonable approach options for a lesion of this size would include a standard pterional approach, a supraorbital approach, or expanded endoscopic transsphenoidal approach. The lesion was quite high and thus, the supraorbital approach may confine access to the superior portion of the tumor. While recognizing that some groups may have chosen the endoscopic expanded transsphenoidal approach for this lesion, the authors describe more confidence in achieving the goal of a safe and maximal resection with the orbitopterional approach. The link to the video can be found at: https://youtu.be/eznsK16BzR8 .

  16. Endoscopic Endonasal Surgery for Purely Intrathird Ventricle Craniopharyngioma.

    Science.gov (United States)

    Nishioka, Hiroshi; Fukuhara, Noriaki; Yamaguchi-Okada, Mitsuo; Yamada, Shozo

    2016-07-01

    Extended endoscopic transsphenoidal surgery (EETS) is a safe and effective treatment for many suprasellar craniopharyngiomas, including those with third-ventricle involvement. Craniopharyngioma entirely within the third ventricle (purely intraventricular type), however, is generally regarded unsuitable for treatment with EETS. Three patients underwent total removal of a purely intraventricular craniopharyngioma with inferior extension via EETS by direct incision of the bulging, stretched ventricular floor and fine dissection from the ventricular wall. In 2 patients with an anteriorly displaced chiasm, the space between the chiasm and pituitary stalk created a wide corridor to the ventricle, whereas in the third case, in which the infrachiasmal space was somewhat narrowed, partial sacrifice of the pituitary gland was necessary to obtain sufficient space. Despite preservation of the stalk in 2 patients, hypopituitarism and diabetes insipidus developed after surgery. There was no other complication including obesity. Selected patients with purely intraventricular craniopharyngioma can be treated effectively and safely with EETS. Those with inferior extension in the interpeduncular fossa and anterior displacement of the chiasm may be suitable candidates. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. BRAF V600E mutations in papillary craniopharyngioma

    Science.gov (United States)

    Brastianos, Priscilla K.; Santagata, Sandro

    2016-01-01

    Papillary craniopharyngioma is an intracranial tumor that results in high levels of morbidity. We recently demonstrated that the vast majority of these tumors harbor the oncogenic BRAF V600E mutation. The pathologic diagnosis of papillary craniopharyngioma can now be confirmed using mutation specific immunohistochemistry and targeted genetic testing. Treatment with targeted agents is now also a possibility in select situations. We recently reported a patient with a multiply recurrent papillary craniopharyngioma in whom targeting both BRAF and MEK resulted in a dramatic therapeutic response with a marked anti-tumor immune response. This work shows that activation of the MAPK pathway is the likely principal oncogenic driver of these tumors. We will now investigate the efficacy of this approach in a multicenter phase II clinical trial. Post-treatment resection samples will be monitored for the emergence of resistance mechanisms. Further advances in the non-invasive diagnosis of papillary craniopharyngioma by radiologic criteria and by cell-free DNA testing could someday allow neo-adjuvant therapy for this disease in select patient populations. PMID:26563980

  18. Diagnostics, Treatment, and Follow-Up in Craniopharyngioma

    Science.gov (United States)

    Müller, Hermann L.

    2011-01-01

    Craniopharyngiomas are partly cystic embryogenic malformations of the sellar and parasellar region, with up to half the 0.5–2.0 new cases per million population per year occur in children and adolescents. Diagnosis profile for pediatric and adult craniopharyngioma is characterized by a combination of headache, visual impairment, and polyuria/polydipsia, which can also include significant weight gain. In children, growth retardation, and/or premature puberty often occur later or postoperatively. Recommended therapy with favorable tumor localization is complete resection; with unfavorable tumor localization (optic nerve and/or hypothalamic involvement), consensus is still pending whether a limited resection followed by local irradiation is more prudent. Even though overall survival rates are high (92%), recurrences after complete resection and progressions after incomplete resection can be expected. Accordingly, a randomized multinational trial (KRANIOPHARYNGEOM 2007) has been established to identify optimal diagnosis, treatment (particularly the ideal time point of irradiation after incomplete resection), and quality of life strategies of this chronic disease – most notably the morbid hypothalamic obesity in ∼50% of long-term survivors. We report on craniopharyngioma origins, its pathological manifestations, and specific challenges these sequelae pose regarding diagnosis, treatment, and life-long multi-discipline quality of life management for both adult and childhood craniopharyngioma patients. PMID:22654824

  19. Suprasellar Anaplastic Meningioma Masquerading As Craniopharyngioma: A Case Report

    Directory of Open Access Journals (Sweden)

    Eman Abdelzaher

    2014-06-01

    Full Text Available Anaplastic meningiomas are uncommon. We report the clinical, radiological and pathological features of an anaplastic meningioma in a young male Egyptian patient presenting as a suprasellar solid/cystic enhancing mass resembling a craniopharyngioma. [J Interdiscipl Histopathol 2014; 2(3.000: 154-157

  20. Morbidity and mortality in patients with craniopharyngioma after surgery.

    LENUS (Irish Health Repository)

    Crowley, R K

    2010-10-01

    Craniopharyngioma (CP) is a benign tumour of the suprasellar region that is associated with increased morbidity and mortality in comparison with other causes of hypopituitarism. We aimed to establish the rate and causes of mortality and morbidity in patients with CP who attended our centre.

  1. Treatment of cystic craniopharyngioma with phosphorus-32 intracavitary irradiation

    Science.gov (United States)

    Zhao, Rong; Deng, Jinglan; Liang, Xiaoyan; Zeng, Jin; Chen, Xiaoyuan

    2013-01-01

    Purpose The aim of the study was to evaluate the effect of phosphorus-32 colloid ([32P]) intracavitary irradiation on the treatment of patients with cystic craniopharyngiomas. Methods Twenty patients with predominantly cystic craniopharyngiomas were admitted from 1981 to 2006. Eleven patients had [32P] intracavitary irradiation by stereotactic injection or Ommaya cyst instillation as the primary treatment, and the remaining nine had the same internal irradiation as an adjuvant treatment after tumor resection. A calculated irradiation dose of 400~500 Gy per once was delivered to the cyst wall. Conclusion The patients were followed up ranging from 36 to 336 months; no operative morbidity or mortality was found from [32P] intracavitary irradiation. Fourteen patients (70%) had tumor progression and required further two to four times intracavitary irradiation. All 20 cases achieved tumor shrinkage or stabilization with effective outcome 3–6 months after the last [32P] therapy. For patients with cystic craniopharyngioma, [32P] administration by stereo-tactic injection or Ommaya cyst instillation is a safe and helpful option, which could improve the life quality, prolong the life span, and enhance the survival rate of cystic craniopharyngioma patients. PMID:19904543

  2. Pre- and intraoperative volume determination of craniopharyngioma cysts

    Energy Technology Data Exchange (ETDEWEB)

    Georgi, P.; Strauss, L.; Sturm, V.; Ostertag, H.; Sinn, H.; Rommel, T.

    1980-08-01

    Exact cystic volume measurement is a prerequisite to proper /sup 90/Y dosage in the therapy of intracavitary monocystic craniopharyngiomas. The method of intraoperative volume measurement by a radionuclide dilution technique is compared to results abtained by preoperative using computer tomography. Both methods gave congruous results. It is pointed out that gamma camera scintigrams are essential for the early detection of complications.

  3. Pituitary necrosis and vasospasm following removal of craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Linda Ratanaprasatporn

    2015-03-01

    Full Text Available We report a case of vasospasm complicating delayed pituitary necrosis after craniopharyngioma resection in an 18-year old female. This is the first reported case that utilizes aggressive blood pressure management, fluid optimization, and rheologic doses of mannitol to successfully treat severe symptomatic vasospasm.

  4. Craniopharyngioma in the third ventricle: necropsy findings and histogenesis.

    Science.gov (United States)

    Kunishio, K; Yamamoto, Y; Sunami, N; Asari, S; Akagi, T; Ohtsuki, Y

    1987-01-01

    A case of craniopharyngioma confined within the third ventricle with necropsy is reported. A stalk-like structure in this tumour was present in the wall of the third ventricle at its base. It is suggested that this tumour might have arisen from the remnants of Rathke's pouch persisting in the tuber cinereum. Images PMID:3655812

  5. Do craniopharyngioma molecular signatures correlate with clinical characteristics?

    Science.gov (United States)

    Omay, Sacit Bulent; Chen, Yu-Ning; Almeida, Joao Paulo; Ruiz-Treviño, Armando Saul; Boockvar, John A; Stieg, Philip E; Greenfield, Jeffrey P; Souweidane, Mark M; Kacker, Ashutosh; Pisapia, David J; Anand, Vijay K; Schwartz, Theodore H

    2018-05-01

    OBJECTIVE Exome sequencing studies have recently demonstrated that papillary craniopharyngiomas (PCPs) and adamantinomatous craniopharyngiomas (ACPs) have distinct genetic origins, each primarily driven by mutually exclusive alterations: either BRAF ( V600E), observed in 95% of PCPs, or CTNNB1, observed in 75%-96% of ACPs. How the presence of these molecular signatures, or their absence, correlates with clinical, radiographic, and outcome variables is unknown. METHODS The pathology records for patients who underwent surgery for craniopharyngiomas between May 2000 and March 2015 at Weill Cornell Medical College were reviewed. Craniopharyngiomas were identified and classified as PCP or ACP. Patients were placed into 1 of 3 groups based on their genomic mutations: BRAF mutation only, CTNNB1 mutation only, and tumors with neither of these mutations detected (not detected [ND]). Demographic, radiological, and clinical variables were collected, and their correlation with each genomic group was tested. RESULTS Histology correlated strongly with mutation group. All BRAF tumors with mutations were PCPs, and all CTNNB1 with mutations and ND tumors were ACPs. Preoperative and postoperative clinical symptoms and radiographic features did not correlate with any mutation group. There was a statistically significant relationship (p = 0.0323) between the age group (pediatric vs adult) and the mutation groups. The ND group tumors were more likely to involve the sella (p = 0.0065). CONCLUSIONS The mutation signature in craniopharyngioma is highly predictive of histology. The subgroup of tumors in which these 2 mutations are not detected is more likely to occur in children, be located in the sella, and be of ACP histology.

  6. Recurrent papillary craniopharyngioma with BRAFV600E mutation treated with neoadjuvant-targeted therapy.

    Science.gov (United States)

    Rostami, Elham; Witt Nyström, Petra; Libard, Sylwia; Wikström, Johan; Casar-Borota, Olivera; Gudjonsson, Olafur

    2017-11-01

    Craniopharyngiomas are histologically benign but locally aggressive tumors in the sellar region that may cause devastating neurological and endocrine deficits. They tend to recur following surgery with high morbidity; hence, postoperative radiotherapy is recommended following sub-total resection. BRAFV600E mutation is the principal oncogenic driver in the papillary variant of craniopharyngiomas. Recently, a dramatic tumor reduction has been reported in a patient with BRAFV600E mutated, multiply recurrent papillary craniopharyngioma using a combination therapy of BRAF inhibitor dabrafenib and MEK inhibitor trametinib. Here, we report on near-radical reduction of a growing residual BRAFV600E craniopharyngioma using the same neoadjuvant therapy.

  7. The neuroradiological study of craniopharyngiomas; A comparison with non-functioning pituitary macroadenomas

    Energy Technology Data Exchange (ETDEWEB)

    Oikawa, Susumu; Takemae, Toshiki; Kobayashi, Shigeaki (Shinshu Univ., Matsumoto, Nagano (Japan). Faculty of Medicine)

    1992-06-01

    The neuroradiological manifestations of 10 surgically verified craniopharyngiomas are retrospectively studied and compared with those of 8 non-functioning pituitary macroadenomas. The saucer-like appearance of the sella turcica on a craniogram was noted in 2 of the 10 craniopharyngiomas and in 2 of the 8 pituitary adenomas. A ballooning of the sella turcica was observed in one of the 10 craniopharyngiomas and in 5 of the 8 pituitary adenomas. Calcification on CT scan was presented in only 5 craniopharyngiomas. On a plain CT scan, no craniopharyngioma manifested any high-density mass except calcification. Four pituitary adenomas showed a slightly high density, though. A cystic lesion was revealed in all the craniopharyngiomas and in 4 pituitary adenomas. All the craniopharyngiomas except for one intrasellar type and all the pituitary adenomas were enhanced with a contrast medium. The solid portion in one craniopharyngioma and in one pituitary adenoma was high-intensity on T[sub 1]-weighted MRI. The cystic portion in 4 craniopharyngiomas and in 2 pituitary adenomas manifested a high intensity on a T[sub 1]-weighted image. A normal pituitary gland located on the floor of the sella was disclosed in all the craniopharyngiomas on a sagittal T[sub 1]-weighted image; however in all the pituitary adenomas, no normal pituitary gland was found at least not on the floor of the sella. The presence of a normal pituitary gland on the sellar floor on sagittal T[sub 1]-weighted MRI may be a new differential diagnostic point between craniopharyngioma and pituitary macroadenoma. Calcification, cystic formation, and density on a plain CT scan seem to be useful evidence for diagnosis. (author).

  8. Somnolence in adult craniopharyngioma patients is a common, heterogeneous condition that is potentially treatable.

    LENUS (Irish Health Repository)

    Crowley, R K

    2012-02-01

    CONTEXT AND OBJECTIVE: Somnolence and obesity are prevalent in craniopharyngioma patients. We hypothesized that somnolence was because of obstructive sleep apnoea in craniopharyngioma patients. DESIGN, PATIENTS AND MEASUREMENTS: We assessed prevalence of somnolence and sleep apnoea in 28 craniopharyngioma and 23 obese controls attending a tertiary referral centre, by means of the Epworth Sleepiness Score (ESS) and polysomnography. All subjects with sleep apnoea were offered continuous positive airway pressure therapy (CPAP) or modafinil. All craniopharyngioma patients, with unexplained somnolence, were offered modafinil. RESULTS: Somnolence was reported by 20\\/28 (71.5%) craniopharyngioma patients and 4\\/23 (17%) obese subjects (P < 0.001). Median ESS was 7.5 (IQR 6, 10.7) in craniopharyngioma patients and 4.0 (4,8) in controls, P < 0.01. Eleven somnolent craniopharyngioma patients had obstructive sleep apnoea, in whom treatment led to a reduction in ESS by 6.4 +\\/- 1.4, P = 0.01. Among the remaining nine patients, five were offered modafinil therapy, of whom four had benefit, three were not compliant with hormone replacement, and one died before intervention. There was no difference in the prevalence of obstructive sleep apnoea between craniopharyngioma (n = 13, 46%) and obese subjects (n = 14, 61%, P = 0.4). Body mass index (BMI) does not correlate with apnoea hypopnoea index [apnoea - hypopnoea index (AHI), r = 0.25, P = 0.08], which suggests that obesity alone does not explain the prevalence of sleep apnoea in craniopharyngioma patients. CONCLUSIONS: Somnolence is common in craniopharyngioma patients and in the majority is because of obstructive sleep apnoea. An additional group of somnolent craniopharyngioma patients benefits from modafinil.

  9. Treatment of craniopharyngioma estimated by follow-up CT

    Energy Technology Data Exchange (ETDEWEB)

    Kubota, T.; Ito, H.; Aizumi, S.; Yamamoto, S.; (Kanazawa Univ. (Japan). School of Medicine)

    1981-12-01

    Follow-up CT scans were taken from 12 cases of craniopharyngiomas after various treatment. Preoperative CT findings of craniopharyngiomas could be classified into three types. Type 1 was a non-enhanced or a thinly ring-like enhanced large cystic mass. Type 2 was a thickly enhanced large cystic mass with small solid mass. Type 3 was a large solid mass. Postoperative follow-up CT findings were as follows: Type 1 had a favorable postoperative course because the tumor tissues of the thin cystic wall seemed to collapse only with the procedure of cystic fluid aspiration. Recurrence frequently took place in patients of Type 2 and 3 if the tumor couldn't be radically removed or radiotherapy was not given after partial resection. Radiotherapy was most effective in these cases.

  10. Pathological changes in cystic craniopharyngiomas following intracavital 90Yttrium treatment

    International Nuclear Information System (INIS)

    Szeifert, G.T.; Julow, J.; Slowik, F.; Balint, K.; Lanyi, F.; Pasztor, E.

    1990-01-01

    Radiosurgery, using 90 Y injected directly into the cavity of cystic craniopharyngiomas produces remarkable reduction of tumour size and diminishes cyst fluid production. The authors have studied the histology of biopsy and autopsy material obtained from seven patients presented with cystic craniopharyngiomas. Histological examination was carried out before and after 90 Y silicate implantation. As an effect of 90 Y irradiation, histology of samples taken from the cyst wall revealed that the lining epithelial cell layer became destroyed and the cyst wall shrunk. Large amount of collagen fibres with focal hyaline degeneration was present. Proliferation of intimal cells and subendothelial connective tissue narrowing small vessel lumina also occurred. Considering that fibrotic tissue is more susceptible to shrink, the fibrosis induced by irradiation together with destruction of the squamous epithelium and vascular changes, might explain the reduction of the cyst volume and diminished fluid secretion after 90 Y treatment. (Authors)

  11. Treatment of craniopharyngioma estimated by follow-up CT

    International Nuclear Information System (INIS)

    Kubota, Toshihiko; Ito, Haruhide; Aizumi, Shinichi; Yamamoto, Shinjiro

    1981-01-01

    Follow-up CT scans were taken from 12 cases of craniopharyngiomas after various treatment. Preoperative CT findings of craniopharyngiomas could be classified into three types. Type 1 was a non-enhanced or a thinly ring-like enhanced large cystic mass. Type 2 was a thickly enhanced large cystic mass with small solid mass. Type 3 was a large solid mass. Postoperative follow-up CT findings were as follows: Type 1 had a favorable postoperative course because the tumor tissues of the thin cystic wall seemed to collapse only with the procedure of cystic fluid aspiration. Recurrence frequently took place in patients of Type 2 and 3 if the tumor couldn't be radically removed or radiotherapy was not given after partial resection. Radiotherapy was most effective in these cases. (author)

  12. Management strategy for treatment of vasospasm following transsphenoidal excision of craniopharyngioma.

    Science.gov (United States)

    Nash, Robert; Elwell, V; Brew, S; Powell, M; Grieve, J P

    2016-11-01

    We describe two cases of clinical and radiographic vasospasm after transsphenoidal resection of a craniopharyngioma. We review the literature on the association of vasospasm and craniopharyngioma and examine management options. Given the lack of evidence for the optimal management of these patients, treatment in concordance with protocols for vasospasm due to subarachnoid haemorrhage is recommended.

  13. First experiences with neuropsychological effects of oxytocin administration in childhood-onset craniopharyngioma.

    Science.gov (United States)

    Hoffmann, Anika; Özyurt, Jale; Lohle, Kristin; Reichel, Julia; Thiel, Christiane M; Müller, Hermann L

    2017-04-01

    The hypothalamic hormone oxytocin plays a major role in regulation of behavior and body composition. Quality of survival is frequently impaired in childhood craniopharyngioma patients due to sequelae such as behavioral deficits and severe obesity caused by tumor or treatment-related hypothalamic lesions. In our pilot cross-sectional study, we analyzed emotion recognition abilities and oxytocin concentrations in saliva and urine before and after single nasal administration of 24 IU oxytocin in 10 craniopharyngioma patients. Four craniopharyngioma presented with grade I lesions (limited to anterior hypothalamic areas) and 6 craniopharyngioma with grade II lesions (involving mammillary bodies and posterior hypothalamic areas). Emotional tasks were assessed before and after administration of oxytocin using the Geneva multimodal emotion portrayals corpus and the Multidimensional Mood Questionnaire. All patients presented with detectable levels of oxytocin before administration. Nasal administration of oxytocin was well-tolerated and resulted in increased oxytocin concentrations in saliva and urine. After oxytocin administration, craniopharyngioma patients with postsurgical lesions limited to the anterior hypothalamus area showed improvements in emotional identifications compared to craniopharyngioma patients with lesions of anterior and posterior hypothalamic areas. Focusing on correct assignments to positive and negative emotion categories, craniopharyngioma patients improved assignment to negative emotions. Oxytocin might have positive effects on emotion perception in craniopharyngioma patients with specific lesions of the anterior hypothalamic area. Further studies on larger cohorts are warranted.

  14. High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma

    NARCIS (Netherlands)

    Pereira, Alberto M.; Schmid, Eva M.; Schutte, Pieter J.; Voormolen, Joan H. C.; Biermasz, Nienke R.; van Thiel, Sjoerd W.; Corssmit, Eleonora P. M.; Smit, Jan W. A.; Roelfsema, Ferdinand; Romijn, Johannes A.

    2005-01-01

    The treatment of craniopharyngiomas is associated with long-term morbidity. To assess the long-term functional outcome and mortality rates after treatment for craniopharyngiomas, we audited our data with special focus on cardiovascular, neurological and psychosocial morbidity. Between 1965 and 2002,

  15. Rare case of malignant craniopharyngioma reactive to adjunctive stereotactic radiotherapy and chemotherapy; Case report and review.

    Science.gov (United States)

    Nomura, Shunsunke; Aihara, Yasuo; Amano, Kosaku; Eguchi, Seiichiro; Chiba, Kentaro; Komori, Takashi; Kawamata, Takakazu

    2018-06-19

    Malignant craniopharyngioma or anaplastic craniopharyngioma was first reported in 1987 by Akachi. It has a malignant clinical and histological feature; remarkably rapid progression, atypical pathology like squamous cell carcinoma and poor prognosis. To date seventeen cases of malignant craniopharyngioma have been reported and of these cases, most were of secondary malignant tumor in nature. With respect to traditional benign craniopharyngioma, adjunctive treatment after gross total removal is not necessary, but in the case of malignant types of the tumor, adjunctive treatment is important. This paper presents the first case of malignant craniopharyngioma reactive to adjunctive Gamma knife stereotactic radiosurgery and chemotherapy. Malignant craniopharyngioma is very rare, and we report Gamma knife stereotactic radiosurgery and chemotherapy (Carboplatine and etoposide chemotherapy), as well as Temozolomide chemotherapy were effective and could control progression of the tumor temporarily. Since adjunctive Gamma knife stereotactic radiosurgery and chemotherapy of malignant craniopharyngioma cases affects follow-up strategies, we propose supporting the need to a revision to the WHO classification regarding malignancy evaluation of craniopharyngioma. Copyright © 2018 Elsevier Inc. All rights reserved.

  16. Sleep-wake and melatonin pattern in craniopharyngioma patients.

    Science.gov (United States)

    Pickering, Line; Jennum, Poul; Gammeltoft, Steen; Poulsgaard, Lars; Feldt-Rasmussen, Ulla; Klose, Marianne

    2014-06-01

    To assess the influence of craniopharyngioma or consequent surgery on melatonin secretion, and the association with fatigue, sleepiness, sleep pattern and sleep quality. Cross-sectional study. A total of 15 craniopharyngioma patients were individually matched to healthy controls. In this study, 24-h salivary melatonin and cortisol were measured. Sleep-wake patterns were characterised by actigraphy and sleep diaries recorded for 2 weeks. Sleepiness, fatigue, sleep quality and general health were assessed by Multidimensional Fatigue Inventory, Pittsburgh Sleep Quality Index, Epworth Sleepiness Scale and Short-Form 36. Patients had increased mental fatigue, daytime dysfunction, sleep latency and lower general health (all, P≤0.05), and they tended to have increased daytime sleepiness, general fatigue and impaired sleep quality compared with controls. The degree of hypothalamic injury was associated with an increased BMI and lower mental health (P=0.01). High BMI was associated with increased daytime sleepiness, daytime dysfunction, mental fatigue and lower mental health (all, P≤0.01). Low midnight melatonin was associated with reduced sleep time and efficiency (P≤0.03) and a tendency for increased sleepiness, impaired sleep quality and physical health. Midnight melatonin remained independently related to sleep time after adjustment for cortisol. Three different patterns of melatonin profiles were observed; normal (n=6), absent midnight peak (n=6) and phase-shifted peak (n=2). Only patients with absent midnight peak had impaired sleep quality, increased daytime sleepiness and general and mental fatigue. Craniopharyngioma patients present with changes in circadian pattern and daytime symptoms, which may be due to the influence of the craniopharyngioma or its treatment on the hypothalamic circadian and sleep regulatory nuclei. © 2014 European Society of Endocrinology.

  17. Extended Transsphenoidal Endoscopic Endonasal Surgery of Suprasellar Craniopharyngiomas.

    Science.gov (United States)

    Fomichev, Dmitry; Kalinin, Pavel; Kutin, Maxim; Sharipov, Oleg

    2016-10-01

    The endoscopic extended transsphenoidal approach for suprasellar craniopharyngiomas may be a really alternative to the transcranial approach in many cases. The authors present their experience with this technique in 136 patients with craniopharyngiomas. From the past 7 years 204 patients with different purely supradiaphragmatic tumors underwent removal by extended endoscopic transsphenoidal transtuberculum transplanum approach. Most of the patients (136) had craniopharyngiomas (suprasellar, intra-extraventricular). The patients were analyzed according to age, sex, tumor size, growth and tumor structure, and clinical symptoms. Twenty-five patients had undergone a previous surgery. The mean follow-up was 42 months (range, 4-120 months). The operation is always performed with the bilateral endoscopic endonasal anterior extended transsphenoidal approach. A gross-total removal was completed in 72%. Improvement of vision or absence of visual deterioration after operation was observed in 89% of patients; 11% had worsening vision after surgery. Endocrine dysfunction did not improve after surgery, new hypotalamopituitary dysfunction (anterior pituitary dysfunction or diabetes insipidus) or worsening of it was observed in 42.6%. Other main complications included transient new mental disorder in 11%, temporary neurological postoperative deficits in 3.7%, bacterial meningitis in 16%, cerebrospinal fluid leaks in 8.8%. The recurrence rate was 20% and the lethality was 5.8%. Resection of suprasellar craniopharyngiomas using the extended endoscopic approach is a more effective and less traumatic technology, able to provide resection of the tumor along with high quality of life after surgery, and relatively rare postoperative complications and mortality. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. Adult Craniopharyngioma: Case Series, Systematic Review, and Meta-Analysis.

    Science.gov (United States)

    Dandurand, Charlotte; Sepehry, Amir Ali; Asadi Lari, Mohammad Hossein; Akagami, Ryojo; Gooderham, Peter

    2017-12-18

    The optimal therapeutic approach for adult craniopharyngioma remains controversial. Some advocate for gross total resection (GTR), while others advocate for subtotal resection followed by adjuvant radiotherapy (STR + XRT). To conduct a systematic review and meta-analysis assessing the rate of recurrence in the follow-up of 3 yr in adult craniopharyngioma stratified by extent of resection and presence of adjuvant radiotherapy. MEDLINE (1946-July 1, 2016) and EMBASE (1980-June 30, 2016) were systematically reviewed. From1975 to 2013, 33 patients were treated with initial surgical resection for adult onset craniopharyngioma at our center and were reviewed for inclusion in this study. Data from 22 patients were available for inclusion as a case series in the systematic review. Eligible studies (n = 21) were identified from the literature in addition to a case series of our institutional experience. Three groups were available for analysis: GTR, STR + XRT, and STR. The rates of recurrence were 17%, 27%, and 45%, respectively. The risk of developing recurrence was significant for GTR vs STR (odds ratio [OR]: 0.24, 95% confidence interval [CI]: 0.15-0.38) and STR + XRT vs STR (OR: 0.20, 95% CI: 0.10-0.41). Risk of recurrence after GTR vs STR + XRT did not reach significance (OR: 0.63, 95% CI: 0.33-1.24, P = .18). This is the first and largest systematic review focusing on the rate of recurrence in adult craniopharyngioma. Although the rates of recurrence are favoring GTR, difference in risk of recurrence did not reach significance. This study provides guidance to clinicians and directions for future research with the need to stratify outcomes per treatment modalities. Copyright © 2017 by the Congress of Neurological Surgeons

  19. Combined Interhemispheric and Transsylvian Approach for Resection of Craniopharyngioma.

    Science.gov (United States)

    Inoue, Tomohiro; Ono, Hideaki; Tamura, Akira; Saito, Isamu

    2018-04-01

    We present a 37-year-old male case of cystic suprasellar huge craniopharyngioma, who presented with significant memory disturbance due to obstructive hydrocephalus. Combined interhemispheric and pterional approach was chosen to resect huge suprasellar tumor. Interhemispheric trans-lamina terminalis approach was quite effective to resect third ventricular tumor, while pterional approach was useful to dissect tumor out of basilar perforators and stalk. The link to the video can be found at: https://youtu.be/BoYIPa96kdo .

  20. Visual complications following irradiation for pituitary adenomas and craniopharyngiomas

    International Nuclear Information System (INIS)

    Harris, J.R.; Levene, M.B.

    1976-01-01

    Of 55 patients with pituitary adenomas or craniopharyngiomas treated with irradiation, a retrospective study revealed that 5 sustained a visual loss compatible with radiation damage to the optic nerve. No patient who received less than 250 rads/day fractions showed such visual loss. Within the range of total dosages used in this series, total dose was not an important determinant of this complication. The time to occurrence of visual disturbance ranged from 5 to 34 months following therapy

  1. Long-term visual outcomes of craniopharyngioma in children.

    Science.gov (United States)

    Wan, Michael J; Zapotocky, Michal; Bouffet, Eric; Bartels, Ute; Kulkarni, Abhaya V; Drake, James M

    2018-05-01

    Visual function is a critical factor in the diagnosis, monitoring, and prognosis of craniopharyngiomas in children. The aim of this study was to report the long-term visual outcomes in a cohort of pediatric patients with craniopharyngioma. The study design is a retrospective chart review of craniopharyngioma patients from a single tertiary-care pediatric hospital. 59 patients were included in the study. Mean age at presentation was 9.4 years old (range 0.7-18.0 years old). The most common presenting features were headache (76%), nausea/vomiting (32%), and vision loss (31%). Median follow-up was 5.2 years (range 1.0-17.2 years). During follow-up, visual decline occurred in 17 patients (29%). On Kaplan Meier survival analysis, 47% of the cases of visual decline occurred within 4 months of diagnosis, with the remaining cases occurring sporadically during follow-up (up to 8 years after diagnosis). In terms of risk factors, younger age at diagnosis, optic nerve edema at presentation, and tumor recurrence were found to have statistically significant associations with visual decline. At final follow-up, 58% of the patients had visual impairment in at least one eye but only 10% were legally blind in both eyes (visual acuity 20/200 or worse or < 20° of visual field). Vision loss is a common presenting symptom of craniopharyngiomas in children. After diagnosis, monitoring vision is important as about 30% of patients will experience significant visual decline. Long-term vision loss occurs in the majority of patients, but severe binocular visual impairment is uncommon.

  2. Clinical equipoise: protons and the child with craniopharyngioma

    International Nuclear Information System (INIS)

    Conroy, Ruth; Owen, Catherine; Ahern, Verity; Gomes, Lavier; Buchsbaum, Jeremy

    2015-01-01

    Childhood craniopharyngioma is a complex condition to manage. Survival figures are high but the potential for long-term morbidity is great. There is much debate regarding the best management for these tumours with increasing interest in the use of proton beam therapy. We have therefore reviewed our radiotherapy (RT) practice at Westmead Hospital and the literature regarding the use of protons for these children. Three children have received fractionated stereotactic RT for craniopharyngioma at Westmead Hospital since 2007. Each RT plan was reviewed and additional organs at risk were contoured to enable comparison with published proton data. Planning target volume coverage was similar with all modalities: with the conformity index ranging from 0.70 to 0.78 in our patients compared with 0.50–0.84 in the published data. RT dose to temporal lobes, hippocampi and whole brain was also similar with protons and photons. Proton beam therapy may give lower dose to the Circle of Willis than stereotactic RT. Currently there is no clear evidence that proton beam therapy will improve survival or reduce morbidity for children with craniopharyngioma. However, proton therapy has the potential to reduce RT dose to the Circle of Willis, which may reduce the risk of future cerebrovascular complications. We propose that more resources should be allocated to ensuring these patients are managed by experienced multidisciplinary teams through the continuum from diagnosis to long-term follow-up.

  3. Clinical equipoise: Protons and the child with craniopharyngioma.

    Science.gov (United States)

    Conroy, Ruth; Gomes, Lavier; Owen, Catherine; Buchsbaum, Jeffrey; Ahern, Verity

    2015-06-01

    Childhood craniopharyngioma is a complex condition to manage. Survival figures are high but the potential for long-term morbidity is great. There is much debate regarding the best management for these tumours with increasing interest in the use of proton beam therapy. We have therefore reviewed our radiotherapy (RT) practice at Westmead Hospital and the literature regarding the use of protons for these children. Three children have received fractionated stereotactic RT for craniopharyngioma at Westmead Hospital since 2007. Each RT plan was reviewed and additional organs at risk were contoured to enable comparison with published proton data. Planning target volume coverage was similar with all modalities: with the conformity index ranging from 0.70 to 0.78 in our patients compared with 0.50-0.84 in the published data. RT dose to temporal lobes, hippocampi and whole brain was also similar with protons and photons. Proton beam therapy may give lower dose to the Circle of Willis than stereotactic RT. Currently there is no clear evidence that proton beam therapy will improve survival or reduce morbidity for children with craniopharyngioma. However, proton therapy has the potential to reduce RT dose to the Circle of Willis, which may reduce the risk of future cerebrovascular complications. We propose that more resources should be allocated to ensuring these patients are managed by experienced multidisciplinary teams through the continuum from diagnosis to long-term follow-up. © 2014 The Royal Australian and New Zealand College of Radiologists.

  4. Contrast enhancement in the craniopharyngioma cyst wall caused by irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Eldevik, O.P.; Gabrielsen, T.O.; Altinok, D. [Univ. of Michigan Medical Center, Ann Arbor, MI (United States). Div. of Neuroradiology

    1998-03-01

    Purpose: To report the occurrence, degree, frequency, and duration of contrast enhancement in the craniopharyngioma cyst wall as caused by irradiation. Material and Methods: Eight patients with cystic craniopharyngiomas had comparable CT or MR studies before and after either external irradiation of the cyst (n=5) or {sup 32}P instillation into the cyst (n=3). Results: A minimal to marked increase in cyst-wall enhancement was seen at 66-157 days after {sup 32}P instillation in 3/3 cases and at 112-495 days after external irradiation in 3/5 cases. The increased enhancement was always associated with a variable interim increase in the thickness of the enhanced cyst wall. Conclusion: CT or MR imaging after the irradiation of cystic craniopharyngiomas usually demonstrated an increase in thickness and enhancement in the cyst wall. Such changes may be due to the irradiation alone and do not necessarily represent infection or increased neoplastic activity. The timing is uncertain as to the earliest possible development of such changes and their maximum duration, but they were seen as early as 66 days post-irradiation and as late as 495 days post-irradiation. (orig.).

  5. Rathke's Cleft Cyst as Origin of a Pediatric Papillary Craniopharyngioma.

    Science.gov (United States)

    Schlaffer, Sven-Martin; Buchfelder, Michael; Stoehr, Robert; Buslei, Rolf; Hölsken, Annett

    2018-01-01

    A 6-year old patient presented with an intra and suprasellar cystic lesion accompanied with impairment of the hypothalamic-pituitary axis and partial hypopituitarism. The most likely cause of sellar lesions in this age group are adamantinomatous craniopharyngioma (adaCP) or Rathke´s cleft cysts (RCCs). AdaCP are characterized by CTNNB1 mutations accompanied with aberrant nuclear beta-catenin expression. RCC show neither nuclear beta-catenin expression nor BRAF mutation. The latter is a hallmark of papillary craniopharyngiomas (papCP) that exhibit remarkable histological similarity with metaplasia of RCC. Diagnosis of the patient was elucidated by CTNNB1 and BRAF mutation screening, utilizing different approaches, as well as histological examination of markers, e.g., beta-catenin, claudin-1, EpCAM and the mutated BRAFV600E protein, which are known to be differentially expressed in sellar lesions. The case presented reveals extraordinary aspects for two reasons. Firstly, the lesion appeared clinically, on MRI, intraoperatively and histologically as RCC with prominent squamous metaplasia, but showing an expression pattern of markers also found in papCP, whilst exhibiting a hitherto undescribed BRAF V 600 E mutation. This important result documents a supposable transition of RCC metaplasia into a papillary craniopharyngioma (papCP). Secondly, this intriguing case shows unexpectedly that although papCP usually occurs almost exclusively in adults, it can also arise in childhood.

  6. Contrast enhancement in the craniopharyngioma cyst wall caused by irradiation

    International Nuclear Information System (INIS)

    Eldevik, O.P.; Gabrielsen, T.O.; Altinok, D.

    1998-01-01

    Purpose: To report the occurrence, degree, frequency, and duration of contrast enhancement in the craniopharyngioma cyst wall as caused by irradiation. Material and Methods: Eight patients with cystic craniopharyngiomas had comparable CT or MR studies before and after either external irradiation of the cyst (n=5) or 32 P instillation into the cyst (n=3). Results: A minimal to marked increase in cyst-wall enhancement was seen at 66-157 days after 32 P instillation in 3/3 cases and at 112-495 days after external irradiation in 3/5 cases. The increased enhancement was always associated with a variable interim increase in the thickness of the enhanced cyst wall. Conclusion: CT or MR imaging after the irradiation of cystic craniopharyngiomas usually demonstrated an increase in thickness and enhancement in the cyst wall. Such changes may be due to the irradiation alone and do not necessarily represent infection or increased neoplastic activity. The timing is uncertain as to the earliest possible development of such changes and their maximum duration, but they were seen as early as 66 days post-irradiation and as late as 495 days post-irradiation. (orig.)

  7. Craniopharyngioma: identification of different semiological patterns by magnetic resonance

    International Nuclear Information System (INIS)

    Molla, E.; Marti-Bonmati, L.; Casillas, C.; Poyatos, C.; Menor, F.; Arana, E.

    1998-01-01

    To study craniopharyngiomas using different MR sequences to detect semiological patterns that aid in the characterization of the different components. We performed a retrospective MR study of 17 patients with confirmed craniopharyngioma. T1-weighted spin-echo, proton density-weighted, T2-weighted gradient-echo, T1-weighted (after administration of gadolinium), T1-weighted inversion recovery and phase and opposed-phase gradient echo sequences were employed to distinguish the different patterns. The semiologic patterns considered in MR were: solid-tissue, blood, protein, fat and fluid. A solid pole was detected in all the patients. There was a cystic component in 88.2% of cases; the protein pattern was observed in 52.9%, blood in 29.4%, fluid in 23.5% and fat in 11.7%. The coexistence of three patterns was detected in 29.4% and of two patterns in 58.8%. The calcium pattern was viewed in 75% of the patients studied with CT, with four patterns coexisting in 25%, three patterns in 41.6% and two patterns in 25%. MR detects different semiologic components in craniopharyngiomas, although it is necessary to employ certain unusual sequences in order to distinguish some patterns from others. (Author) 22 refs

  8. Rathke's Cleft Cyst as Origin of a Pediatric Papillary Craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Sven-Martin Schlaffer

    2018-02-01

    Full Text Available A 6-year old patient presented with an intra and suprasellar cystic lesion accompanied with impairment of the hypothalamic-pituitary axis and partial hypopituitarism. The most likely cause of sellar lesions in this age group are adamantinomatous craniopharyngioma (adaCP or Rathke´s cleft cysts (RCCs. AdaCP are characterized by CTNNB1 mutations accompanied with aberrant nuclear beta-catenin expression. RCC show neither nuclear beta-catenin expression nor BRAF mutation. The latter is a hallmark of papillary craniopharyngiomas (papCP that exhibit remarkable histological similarity with metaplasia of RCC. Diagnosis of the patient was elucidated by CTNNB1 and BRAF mutation screening, utilizing different approaches, as well as histological examination of markers, e.g., beta-catenin, claudin-1, EpCAM and the mutated BRAFV600E protein, which are known to be differentially expressed in sellar lesions. The case presented reveals extraordinary aspects for two reasons. Firstly, the lesion appeared clinically, on MRI, intraoperatively and histologically as RCC with prominent squamous metaplasia, but showing an expression pattern of markers also found in papCP, whilst exhibiting a hitherto undescribed BRAFV600E mutation. This important result documents a supposable transition of RCC metaplasia into a papillary craniopharyngioma (papCP. Secondly, this intriguing case shows unexpectedly that although papCP usually occurs almost exclusively in adults, it can also arise in childhood.

  9. Long-term outcomes for adult craniopharyngioma following radiation therapy

    International Nuclear Information System (INIS)

    Masson-Cote, Laurence; Masucci, Giuseppina Laura; Millar, Barbara-Ann; Laperriere, Normand J.; Atenafu, Eshetu G.; Cusimano, Michael; Croul, Sidney; Mason, Warren; Sahgal, Arjun; Dept. of Radiation Oncology, Sunnybrook Health Sciences Center, Univ. of Toronto, Toronto

    2013-01-01

    Background. We report long-term outcomes in adult patients with craniopharyngioma following surgery and radiation therapy (RT). Material and methods. Fifty-three patients treated with RT (median, 50 Gy in 25 fractions) between 1980 and 2009 with pathologically confirmed craniopharyngioma were reviewed (53% solid and 47% cystic/solid). The median age was 53 years (range, 22-76), 53% were female, 83% were sub-totally resected, 6% were gross totally resected and 11% had a biopsy and/or cyst aspiration alone. RT was delivered adjuvantly in 53% of patients as opposed to salvage intent upon progression. Results. Median follow-up was seven years (86 months, range, 8-259). The 5- and 10-year progression-free survival (PFS) rates were 85% and 69%, overall survival (OS) rates were 76% and 70%, and cause-specific survival (CSS) rates were both 88%, respectively. Both univariable and multivariable analysis identified age ( 2 surgeries prior to RT (p =0.01). Neither the intent of radiation or tumor type (cystic vs. solid/cystic) were prognostic or predictive. New endocrinopathies and visual dysfunction were observed in 53% and 17% of patients post-surgery, and in 11% and 6% post-RT, respectively. Conclusion. We report long-term favorable PFS, CSS and OS for craniopharyngioma post-RT. We observe age as a significant prognostic factor, however, timing of radiation was not

  10. Excess morbidity and mortality in patients with craniopharyngioma: a hospital-based retrospective cohort study.

    Science.gov (United States)

    Wijnen, Mark; Olsson, Daniel S; van den Heuvel-Eibrink, Marry M; Hammarstrand, Casper; Janssen, Joseph A M J L; van der Lely, Aart J; Johannsson, Gudmundur; Neggers, Sebastian J C M M

    2018-01-01

    Most studies in patients with craniopharyngioma did not investigate morbidity and mortality relative to the general population nor evaluated risk factors for excess morbidity and mortality. Therefore, the objective of this study was to examine excess morbidity and mortality, as well as their determinants in patients with craniopharyngioma. Hospital-based retrospective cohort study conducted between 1987 and 2014. We included 144 Dutch and 80 Swedish patients with craniopharyngioma identified by a computer-based search in the medical records (105 females (47%), 112 patients with childhood-onset craniopharyngioma (50%), 3153 person-years of follow-up). Excess morbidity and mortality were analysed using standardized incidence and mortality ratios (SIRs and SMRs). Risk factors were evaluated univariably by comparing SIRs and SMRs between non-overlapping subgroups. Patients with craniopharyngioma experienced excess morbidity due to type 2 diabetes mellitus (T2DM) (SIR: 4.4, 95% confidence interval (CI): 2.8-6.8) and cerebral infarction (SIR: 4.9, 95% CI: 3.1-8.0) compared to the general population. Risks for malignant neoplasms, myocardial infarctions and fractures were not increased. Patients with craniopharyngioma also had excessive total mortality (SMR: 2.7, 95% CI: 2.0-3.8), and mortality due to circulatory (SMR: 2.3, 95% CI: 1.1-4.5) and respiratory (SMR: 6.0, 95% CI: 2.5-14.5) diseases. Female sex, childhood-onset craniopharyngioma, hydrocephalus and tumour recurrence were identified as risk factors for excess T2DM, cerebral infarction and total mortality. Patients with craniopharyngioma are at an increased risk for T2DM, cerebral infarction, total mortality and mortality due to circulatory and respiratory diseases. Female sex, childhood-onset craniopharyngioma, hydrocephalus and tumour recurrence are important risk factors. © 2018 European Society of Endocrinology.

  11. Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Chen, C.J. [Dept. of Diagnostic Radiology, Chang Gung Memorial Hospital and University, Taipei (Taiwan)

    2001-09-01

    Craniopharyngiomas are usually confined to the sellar and suprasellar regions; infrasellar craniopharyngioma is rare. From an embryological point of view, this unusual localisation can be explained by Erdheim's theory that these tumours can arise anywhere along the craniopharyngeal canal (CPC). However, there has been no proof of this theory, because the CPC is usually obliterated during the 12th week of gestation. I present a case of supra- and infrasellar craniopharyngiomas with a persistent CPC. Imaging demonstrated an intimate relationship between the infrasellar tumour and the CPC, supporting Erdheim's view. (orig.)

  12. Natural pregnancy and normal delivery after intracapsular irradiation for symptomatic craniopharyngioma

    International Nuclear Information System (INIS)

    Kodama, Takafumi; Matsukado, Yasuhiko; Miura, Masaki; Takada, Akira

    1986-01-01

    Treatment for craniopharyngioma, particularly in adults and cases of recurrence, is still fraught with problems. One difficulty is that postoperative hormonal function may be abnormal even after successful surgery. We have treated craniopharyngioma primarily with simple evacuation and intracapsular irradiation, using 198 Au-colloid. Only a few cases of natural pregnancy and normal delivery following treatment for craniopharyngioma with hypothalamic extension have been reported. We will describe and discuss a case in which natural pregnancy and normal delivery occurred twice after such treatment. (author)

  13. The anesthetic, critical care and surgical challenges in the management of craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Sukhminder Jit Singh Bajwa

    2011-01-01

    Full Text Available Among the childhood brain neoplasms, craniopharyngioma constitutes about 2-6% of all primary intracranial tumors. Craniopharyngioma poses a multitude of challenges to the neurosurgeon, endocrinologist, oncologist, intensivist and the anesthesiologist. The morbidity and mortality due to radical surgical treatment is quite high, to the extent of 40-50%. The conservative approach with limited surgical intervention and radiotherapy assistance is taken by some neurosurgeons, but its usefulness is very doubtful. We are reporting a case of craniopharyngioma in an 18-year-old female who had progressive loss of vision and was successfully managed with combined radical surgery and medical therapy.

  14. De novo Craniopharyngioma of the Fourth Ventricle: Case Report and Review of Literature.

    Science.gov (United States)

    Algahtani, Abdulhadi Y; Algahtani, Hussein A; Jamjoom, Abdulhakim B; Samkari, Alaa M; Marzuk, Yousef I

    2018-01-01

    Craniopharyngiomas usually involve the sella and suprasellar space. Their occurrence in the posterior fossa without extension to the suprasellar region is uncommon with only 16 cases reported in the literature. We report a case of a primary posterior fossa craniopharyngioma that was managed by complete excision with a good recovery. Our case was unique in that the craniopharyngioma occurred in the fourth ventricle and extended downward to the level of C1, a manifestation that was reported only twice in the past. The literature on the topic is reviewed.

  15. A Craniopharyngioma Associated With Elevated Cerebrospinal Fluid HCG Concentrations Misdiagnosed as a Germinoma

    Directory of Open Access Journals (Sweden)

    Weijun Gu

    2018-06-01

    Full Text Available Craniopharyngiomas and germinomas are both rare cranial tumors that most commonly present during childhood or adolescence. Although these tumors have different origins, their clinical and radiological features may be similar. In this article, we report the case of a 35-year female patient with clinical and radiological findings and increased human chorionic gonadotrophin (HCG levels in the cerebrospinal fluid (CSF that were consistent with a germinoma. However, pathological analysis revealed a craniopharyngioma. This case report indicates that HCG, which is regarded as a specific tumor marker for germinomas in the differential diagnosis of intracranial lesions, is also detectable in other kinds of suprasellar tumors, such as craniopharyngiomas.

  16. 12 Years delayed postoperative spinal recurrence of craniopharyngioma. Case report and literature review.

    Science.gov (United States)

    Marchesini, Nicolò; Soda, Christian; Ricci, Umberto Maria; Sedia, Mattia; Sala, Francesco; Pinna, Giampietro

    2017-12-04

    A case of delayed spinal adamantinomatous craniopharyngioma recurrence is presented. A 54-year-old male patient was admitted in our Emergency Department complaining of urinary disorders and leg pains. He underwent surgical removal of intraventricular craniopharyngioma 12 years previously. On MR imaging a well-circumscribed intradural cistyc mass at the T12 level was reavealed. A T11 and T12 laminotomy was performed and total removal of the tumour was achieved. Histology examination showed adamantinous craniopharyngioma. The authors believe that this represents the third case described of spinal craniofaryngioma recurrence and the first involving the dorsal spine.

  17. MELATONIN SECRETION AND HYPERSOMNIA IN CHILDREN AND ADOLESCENTS AFTER CRANIOPHARYNGIOMA RESECTION

    Directory of Open Access Journals (Sweden)

    E. Yu. Il'ina

    2012-01-01

    Full Text Available Craniopharyngioma is a rare embryonic tumor of the chiasm-sellar region with low malignancy rate but high frequency of recurrence. In spite of the favorable outcome and satisfactory results of treatment the patients quality of life worsens due to complications associated with anatomic localization of craniopharyngioma close to the optic nerve, optic chiasm, hypophysis and hypothalamus. The development of hypothalamic obesity is observed in 25–60% of children and adolescents after surgical treatment of craniopharyngioma, which is usually accompanied with significant disturbances of circadian rhythm and has the most important influence on patients quality of life.

  18. Natural pregnancy and normal delivery after intracapsular irradiation for symptomatic craniopharyngioma. Case report

    Energy Technology Data Exchange (ETDEWEB)

    Kodama, Takafumi; Matsukado, Yasuhiko; Miura, Masaki; Takada, Akira

    1986-01-01

    Treatment for craniopharyngioma, particularly in adults and cases of recurrence, is still fraught with problems. One difficulty is that postoperative hormonal function may be abnormal even after successful surgery. We have treated craniopharyngioma primarily with simple evacuation and intracapsular irradiation, using /sup 198/Au-colloid. Only a few cases of natural pregnancy and normal delivery following treatment for craniopharyngioma with hypothalamic extension have been reported. We will describe and discuss a case in which natural pregnancy and normal delivery occurred twice after such treatment.

  19. Vasospasm After Craniopharyngioma Surgery: Can We Prevent It?

    Science.gov (United States)

    Singh, Apinderpreeet; Salunke, Pravin; Rangan, Vasundhara; Ahuja, Chirag K; Bhadada, Sanjay

    2017-05-01

    Vasospasm after craniopharyngioma surgery, although rare, has been reported. Hypotheses regarding possible causative factors, including major vessel handling during surgery and tumor cyst fluid spillage, do not explain vasospasm occurring in the late postoperative period. We have attempted to consider the probable pathogenic mechanisms of this complication and measures to prevent it. Of 60 patients operated for craniopharyngiomas over a period of 4 years, 6 who had clinical and radiologically demonstrable vasospasm were identified. Each case was analyzed retrospectively, and relevant variables were studied. Five of the 6 patients developed vasospasm in the second or third week after surgery. Only 2 of these patients had a favorable outcome. There was significant altered fluid balance during this period; however, patients remained misleadingly eunatremic. This most often coincided with the transition period from syndrome of inappropriate antidiuretic hormone to diabetes insipidus (DI) and the period after steroid taper to minimal dose. Major vessel handling during radical craniopharyngioma surgery is likely to predispose them to spasm, accentuated by rapid shifts of fluid and electrolytes during different phases of DI. This is further complicated by a relative hypocortisolic state caused by tendency to taper off steroids early. Hypocortisolism masks DI leading to dehydration and possibly vasospasm. Once vasospasm develops, it is not easy to reverse. Radiologic reversal with intra-arterial nimodipine may not translate into a good clinical response. Therefore, prudence lies in its prevention. Close monitoring of fluids and electrolytes with optimal steroid cover is necessary until stabilization of DI to prevent this complication. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Combined endoscopic approach in the management of suprasellar craniopharyngioma.

    Science.gov (United States)

    Deopujari, Chandrashekhar E; Karmarkar, Vikram S; Shah, Nishit; Vashu, Ravindran; Patil, Rahul; Mohanty, Chandan; Shaikh, Salman

    2018-05-01

    Craniopharyngiomas are dysontogenic tumors with benign histology but aggressive behavior. The surgical challenges posed by the tumor are well recognized. Neuroendoscopy has recently contributed to its surgical management. This study focuses on our experience in managing craniopharyngiomas in recent years, highlighting the role of combined endoscopic trans-ventricular and endonasal approach. Ninety-two patients have been treated for craniopharyngioma from 2000 to 2016 by the senior author. A total of 125 procedures, microsurgical (58) and endoscopic (67), were undertaken. Combined endoscopic approach was carried out in 18 of these patients, 16 children and 2 young adults. All of these patients presented with a large cystic suprasellar mass associated with hydrocephalus. In the first instance, they were treated with a transventricular endoscopic procedure to decompress the cystic component. This was followed by an endonasal transsphenoidal procedure for excision within the next 2 to 6 days. All these patients improved after the initial cyst decompression with relief of hydrocephalus while awaiting remaining tumor removal in a more elective setting. Gross total resection could be done in 84% of these patients. Diabetes insipidus was the most common postsurgical complication seen in 61% patients in the immediate period but was persistent in only two patients at 1-year follow-up. None of the children in this group developed morbid obesity. There was one case of CSF leak requiring repair after initial surgery. Peri-operative mortality was seen in one patient secondary to ventriculitis. The patients who benefit most from the combined approach are those who present with raised intracranial pressure secondary to a large tumor with cyst causing hydrocephalus. Intraventricular endoscopic cyst drainage allows resolution of hydrocephalus with restoration of normal intracranial pressure, gives time for proper preoperative work up, and has reduced incidence of CSF leak after

  1. Long-term outcomes for adult craniopharyngioma following radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Masson-Cote, Laurence; Masucci, Giuseppina Laura; Millar, Barbara-Ann; Laperriere, Normand J. [Dept. of Radiation Oncology, Princess Margaret Hospital, Univ. of Toronto, Toronto (Canada); Atenafu, Eshetu G. [Dept. of Biostatistics, Princess Margaret Hospital, Univ. of Toronto, Toronto (Canada); Cusimano, Michael [Dept. of Surgery, Div. of Neurosurgery, St. Michaels Hospital, Toronto (Canada); Croul, Sidney [Dept. of Pathology, Univ. of Toronto, Toronto (Canada); Mason, Warren [Dept. of Medicine, Princess Margaret Hospital, Univ. of Toronto, Toronto (Canada); Sahgal, Arjun [Dept. of Radiation Oncology, Princess Margaret Hospital, Univ. of Toronto, Toronto (Canada), E-mail: Arjun.sahgal@rmp.uhn.on.ca; Dept. of Radiation Oncology, Sunnybrook Health Sciences Center, Univ. of Toronto, Toronto (Canada)

    2013-01-15

    Background. We report long-term outcomes in adult patients with craniopharyngioma following surgery and radiation therapy (RT). Material and methods. Fifty-three patients treated with RT (median, 50 Gy in 25 fractions) between 1980 and 2009 with pathologically confirmed craniopharyngioma were reviewed (53% solid and 47% cystic/solid). The median age was 53 years (range, 22-76), 53% were female, 83% were sub-totally resected, 6% were gross totally resected and 11% had a biopsy and/or cyst aspiration alone. RT was delivered adjuvantly in 53% of patients as opposed to salvage intent upon progression. Results. Median follow-up was seven years (86 months, range, 8-259). The 5- and 10-year progression-free survival (PFS) rates were 85% and 69%, overall survival (OS) rates were 76% and 70%, and cause-specific survival (CSS) rates were both 88%, respectively. Both univariable and multivariable analysis identified age (<53 or {>=}53) as a prognostic factor for OS (p =0.0003) and CSS (p =0.05). PFS was observed to be worse in patients with >2 surgeries prior to RT (p =0.01). Neither the intent of radiation or tumor type (cystic vs. solid/cystic) were prognostic or predictive. New endocrinopathies and visual dysfunction were observed in 53% and 17% of patients post-surgery, and in 11% and 6% post-RT, respectively. Conclusion. We report long-term favorable PFS, CSS and OS for craniopharyngioma post-RT. We observe age as a significant prognostic factor, however, timing of radiation was not.

  2. Molecular pathology of adamantinomatous craniopharyngioma: review and opportunities for practice.

    Science.gov (United States)

    Apps, John Richard; Martinez-Barbera, Juan Pedro

    2016-12-01

    Since the first identification of CTNNB1 mutations in adamantinomatous craniopharyngioma (ACP), much has been learned about the molecular pathways and processes that are disrupted in ACP pathogenesis. To date this understanding has not translated into tangible patient benefit. The recent development of novel techniques and a range of preclinical models now provides an opportunity to begin to support treatment decisions and develop new therapeutics based on molecular pathology. In this review the authors summarize many of the key findings and pathways implicated in ACP pathogenesis and discuss the challenges that need to be tackled to translate these basic science findings for the benefit of patients.

  3. Unilateral Autosomal Recessive Anophthalmia in a Patient with Cystic Craniopharyngioma

    Science.gov (United States)

    Kumar, Amandeep; Bansal, Ankit; Garg, Ajay; Sharma, Bhawani S.

    2014-01-01

    Abstract Anophthalmia is a rare ocular malformation. It is a genetically determined disorder and is typically associated with syndromes. However, sporadic nonsyndromic familial as well as non-familial cases of anophthalmia have also been reported. Non-syndromic familial cases are usually bilateral and have been attributed to autosomal recessive, autosomal dominant, and X-linked inheritance patterns. The authors hereby report a rare case of autosomal recessive unilateral anophthalmia in a patient with no other associated congenital anomaly. Patient was operated for craniopharyngioma. The clinical, radiological and intraoperative findings are discussed. PMID:27928292

  4. Retreatment of Recurrent Cystic Craniopharyngioma With Chromic Phosphorus P 32

    Science.gov (United States)

    Kumar, P.P.; Good, R.R.; Skultety, F.M.; Jones, E.O.; Chu, W.K.

    1986-01-01

    A cystic craniopharyngioma in a two-year-old boy recurred six months after surgery and postoperative external-beam radiotherapy. Successful retreatment was accomplished with radioisotope injection of 0.5 mCi of chromic phosphorus P 32 into the intracranial cyst, which delivered approximately 300.00 Gy to the cyst wall. The patient's symptoms were relieved, and he is without evidence of disease or cystic fluid accumulation four years after intracavitary 32P irradiation. ImagesFigure 1Figure 2Figure 3 PMID:3735454

  5. Visual outcome in cystic craniopharyngiomas treated with intracavitary phosphorus-32

    International Nuclear Information System (INIS)

    Anderson, D.R.; Trobe, J.D.; Taren, J.A.; Gebarski, S.S.

    1989-01-01

    Seven patients with cystic craniopharyngiomas were treated with stereotactic instillation of radioactive phosphorus-32 (32P). Five patients had been previously treated with various combinations of surgery and external beam irradiation, whereas two had the 32 P instillation as a primary therapy. Visual acuity improved in 13 eyes and remained stable in 1. Visual fields normalized in three patients, improved in two, and remained stable in two. Two patients received single treatments with 32 P, whereas five required multiple instillations for recurrent cyst expansion

  6. A craniopharyngioma in a seven-year-old dog

    Energy Technology Data Exchange (ETDEWEB)

    Eckersley, G N; Kriek, N P.J. [Medical Univ. of Southern Africa (MEDUNSA), Pretoria (South Africa); Geel, J K [Witwatersrand Univ., Johannesburg (South Africa)

    1991-06-01

    A seven-year-old male Border Collie was presented with a history of lethargy, episodic circling, incoordination and polydypsia. Physical examination revealed depression, obesity and bradycardia. A neurological examination indicated the possible presence of a space-occupying lesion in the brain. Results of the clinical investigation revealed hyposthenuria, sinus bradycardia and increased concentration of protein in the cerobrospinal fluid. A computerised axial tomography scan revealed a mass in the region of the hypophysis. The dog was euthanased and a post mortem examination confirmed the presence of a craniopharyngioma. 17 refs., 3 figs.

  7. A craniopharyngioma in a seven-year-old dog

    International Nuclear Information System (INIS)

    Eckersley, G.N.; Kriek, N.P.J.; Geel, J.K.

    1991-01-01

    A seven-year-old male Border Collie was presented with a history of lethargy, episodic circling, incoordination and polydypsia. Physical examination revealed depression, obesity and bradycardia. A neurological examination indicated the possible presence of a space-occupying lesion in the brain. Results of the clinical investigation revealed hyposthenuria, sinus bradycardia and increased concentration of protein in the cerobrospinal fluid. A computerised axial tomography scan revealed a mass in the region of the hypophysis. The dog was euthanased and a post mortem examination confirmed the presence of a craniopharyngioma. 17 refs., 3 figs

  8. Endovascular Treatment for Fusiform Dilation of Internal Carotid Artery Following Craniopharyngioma Resection: A Case Illustration.

    Science.gov (United States)

    Li, Qiang; Wang, Chaohua; Xu, Jianguo; You, Chao

    2015-09-01

    Fusiform dilation of the internal carotid artery complicates aggressive craniopharyngioma resection and occurs mainly in children. We report a case to describe the availability of endovascular treatment for this rare entity. A 13-year-old boy presented with headache for 2 years after resection of craniopharyngioma. A fusiform dilation of the right carotid artery was found and was coiled using stent-assisted technique. Follow-up showed satisfactory outcome and disappearance of headache. To our knowledge, this is the first report regarding endovascular treatment for fusiform dilation of the internal carotid artery after craniopharyngioma resection. Stent-assisted coiling is a useful approach for fusiform dilation of the internal carotid artery following craniopharyngioma surgery. © The Author(s) 2014.

  9. Collision tumors of the sella: coexistence of pituitary adenoma and craniopharyngioma in the sellar region

    Science.gov (United States)

    2013-01-01

    Collision tumors of the sellar region are relatively uncommon and consist mainly of more than one type of pituitary adenoma or a cyst or cystic tumor. The association of a pituitary adenoma and a craniopharyngioma is particularly rare. This study describes a rare occurrence in which a pituitary adenoma and a craniopharyngioma coexisted in the sellar region. The case involves a 47-year-old woman who underwent transsphenoidal surgery with subtotal tumor resection and reoperation using an interhemispheric transcallosal approach for total microsurgical resection of the tumor because the visual acuity in her left eye had re-deteriorated. Histopathological and immunohistochemical examinations of the excised tissue revealed a pituitary adenoma in the first operation and a craniopharyngioma in the second operation. Retrospective analysis found the coexistence of a pituitary adenoma and a craniopharyngioma, known as a collision tumor. Instead of the transsphenoidal approach, a craniotomy should be performed, to explore the suprasellar region. PMID:23919255

  10. Paraneoplastic limbic encephalitis associated with mixed olfactory neuroblastoma and craniopharyngioma: A case report and literature review.

    Science.gov (United States)

    Nagafuji, Hiroshi; Yokoi, Hidenori; Fujiwara, Masachika; Sato, Dai; Saito, Koichiro

    2018-06-01

    Paraneoplastic limbic encephalitis (PLE) is a rare disorder of the nervous system associated with malignant disease. It has a subacute onset with the following symptoms: cognitive dysfunction, seizures, irritability, hallucinations, and short-term memory loss. Herein, we report the case of a 35-year-old man with PLE, an olfactory neuroblastoma (ONB) admixed with craniopharyngioma, and serum anti-Hu antibodies. The patient presented with generalized seizures, short-term memory loss, and a polypoid mass located high in the nasal cavity. He underwent surgical resection of the tumor and postoperative chemoradiotherapy with concurrent intra-arterial cisplatin administration. Pathological examination indicated an ONB admixed with craniopharyngioma. The patient's neurological symptoms gradually diminished after surgery. No evidence of recurrence was observed during a 4-year follow-up. We reported a histologically unusual heterogeneous tumor that comprised ONB and craniopharyngioma. This is the first reported case of PLE with anti-Hu antibodies possibly associated with ONB admixed with craniopharyngioma.

  11. Effects of gamma knife radiosurgery for pediatric craniopharyngiomas

    International Nuclear Information System (INIS)

    Tanaka, Takayuki; Kobayashi, Tatsuya; Kida, Yoshihisa

    1996-01-01

    Seven cases of pediatric craniopharyngiomas have been treated by gamma knife and followed-up for a mean 24.1 months between May, 1991 and March, 1995. They included 4 boys and 3 girls with a mean age of 8.9 years. Initial signs and symptoms were: headaches in 2 cases, decrease in visual acuity in 6 cases, visual field deficit in 5 cases, hypopituitary function in 5 cases, and diabetes insipidus in one case. Prior to the radiosurgery, surgical therapy had been performed in 7 cases, conventional radiotherapy in one case, and chemotherapy in one case. Tumor were located in the chiasmal region in 3 cases and the suprasellar region in 4 cases. Mean tumor diameter was 18.5 mm. Mean and marginal irradiation dosages were 25.9 Gy and 13.4 Gy. Repeated MRI indicated marked shrinkage of tumors was obtained in all 7 cases. Follow up showed neurological signs and symptoms improved in 3 cases and remained unchanged in 4 cases, without any side-effects. Hormonal study indicated TSH decreased about one year after gamma knife radiosurgery. It is considered that gamma knife radiosurgery will be a safe and effective treatment for pediatric craniopharyngiomas in combination with microsurgery. (author)

  12. Predicting Behavioral Problems in Craniopharyngioma Survivors after Conformal Radiation Therapy

    Science.gov (United States)

    Dolson, Eugenia P.; Conklin, Heather M.; Li, Chenghong; Xiong, Xiaoping; Merchant, Thomas E.

    2009-01-01

    Background Although radiation therapy is a primary treatment for craniopharyngioma, it can exacerbate existing problems related to the tumor and pre-irradiation management. Survival is often marked by neurologic deficits, panhypopituitarism, diabetes insipidus, cognitive deficiencies and behavioral and social problems. Procedure The Achenbach Child Behavior Checklist (CBCL) was used to evaluate behavioral and social problems during the first five years of follow-up in 27 patients with craniopharyngioma treated with conformal radiation therapy. Results All group averages for the CBCL scales were within the age-typical range at pre-irradiation baseline. Extent of surgical resection was implicated in baseline differences for the Internalizing, Externalizing, Behavior Problem and Social scores. Significant longitudinal changes were found in Internalizing, Externalizing, Behavior Problem and School scores that correlated with tumor and treatment related factors. Conclusions The most common variables implicated in post-irradiation behavioral and social problems were CSF shunting, presence of an Ommaya reservoir, diabetes insipidus, and low pre-irradiation growth hormone levels. PMID:19191345

  13. Computerized tomographic evaluations of 33 consecutive cases of craniopharyngiomas

    International Nuclear Information System (INIS)

    Nagasawa, Shiro; Takeuchi, Juji; Yamashita, Junkoh; Handa, Hajime

    1983-01-01

    Although craniopharyngiomas are widely known to exhibit three basic CT characteristics; calcification, cyst(s) and contrast enhancement (CE), several cases with atypical CT manifestations have been reported lately. These atypical manifestations can be classified into unusual extensions of the tumors, high dense cyst and marked homogeneous CE. The CT scans obtained in our recent series of 33 craniopharyngiomas were evaluated to analyze tumoral extensions, cyst density and CE. Cystic tumors were much more frequent than solid tumors (Table 1); 13 cases (39%) were exclusively cystic, 15 cases (45%) equally cystic and solid, and 5 case (15%) solid. Calcifications were present in 80 % in children and 39% in adults. Unusual extensions were noted in 7 cases (Fig. 2,3,4); anteriorly to the frontal fossa (5 cases, 15 %), laterally to the temporal fossa (1 case, 3%) and posteriorly to the posterior fossa (1 case, 3%). All these tumors were either pure cystic or equally cystic and solid. Cystic portions were detected in the tumors of 28 cases (Table 2). Although the majority of the cystic portions (22 cases, 79%) were low density, iso-dense cysts were observed in 6 cases (21%). Out of 20 cases exhibiting solid portions in the tumors, high density areas (calcified masses) were detected in 3 cases and mixed density areas in 17 cases (Table 3). While these 17 cases showed CE, marked homogeneous CE were noted in 5 cases (29%)

  14. MR and CT diagnosis of carotid pseudoaneurysm in children following surgical resection of craniopharyngioma

    International Nuclear Information System (INIS)

    Lakhanpal, S.K.; Glasier, C.M.; James, C.A.; Angtuaco, E.J.C.

    1995-01-01

    We report the cases of two children who underwent CT, MR, MRA and angiography in the diagnosis of postoperative aneurysmal dilatation of the supraclinoid carotid arteries following surgical resection of craniopharyngioma. Craniopharyngiomas are relatively common lesions, accounting for 6-7 % of brain tumors in children. They are histologically benign, causing symptoms by their growth within the sella and suprasellar cistern with compression of adjacent structures, especially the pituitary gland, hypothalamus and optic nerves, chiasm, and tracts. (orig.)

  15. MR and CT diagnosis of carotid pseudoaneurysm in children following surgical resection of craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Lakhanpal, S.K. [Dept. of Radiology, Univ. of Arkansas for Medical Sciences and Arkansas Children`s Hospital, Little Rock, AR (United States); Glasier, C.M. [Dept. of Radiology, Univ. of Arkansas for Medical Sciences and Arkansas Children`s Hospital, Little Rock, AR (United States); James, C.A. [Dept. of Radiology, Univ. of Arkansas for Medical Sciences and Arkansas Children`s Hospital, Little Rock, AR (United States); Angtuaco, E.J.C. [Dept. of Radiology, Univ. of Arkansas for Medical Sciences and Arkansas Children`s Hospital, Little Rock, AR (United States)

    1995-06-01

    We report the cases of two children who underwent CT, MR, MRA and angiography in the diagnosis of postoperative aneurysmal dilatation of the supraclinoid carotid arteries following surgical resection of craniopharyngioma. Craniopharyngiomas are relatively common lesions, accounting for 6-7 % of brain tumors in children. They are histologically benign, causing symptoms by their growth within the sella and suprasellar cistern with compression of adjacent structures, especially the pituitary gland, hypothalamus and optic nerves, chiasm, and tracts. (orig.)

  16. New outlook on the diagnosis, treatment and follow-up of childhood-onset craniopharyngioma.

    Science.gov (United States)

    Müller, Hermann L; Merchant, Thomas E; Puget, Stephanie; Martinez-Barbera, Juan-Pedro

    2017-05-01

    Childhood-onset craniopharyngiomas are rare embryonic tumours of low-grade histological malignancy. Novel insights into the molecular pathogenesis of human adamantinomatous craniopharyngioma have started to unveil the possibility of testing novel treatments targeting pathogenic pathways. Hypothalamic involvement and/or treatment-related lesions result in impaired physical and social functionality and in severe neuroendocrine sequelae. Quality of survival in patients with craniopharyngioma with hypothalamic involvement is impaired by severe obesity, physical fatigue and non-optimal psychosocial development. Patients with craniopharyngioma involving hypothalamic structures have reduced 20-year overall survival, but overall and progression-free survival are not related to the degree of surgical resection. Irradiation is effective in the prevention of tumour progression and recurrence. For favourably localized craniopharyngiomas, the preferred treatment of choice is to attempt complete resection with preservation of visual, hypothalamic and pituitary function. For unfavourably localized tumours in close proximity to optic and/or hypothalamic structures, a radical neurosurgical strategy attempting complete resection is not recommended owing to potential severe sequelae. As expertise has been shown to have an impact on post-treatment morbidity, medical societies should establish criteria for adequate professional expertise for the treatment of craniopharyngioma. On the basis of these criteria, health authorities should organize the certification of centres of excellence that are authorized to treat and care for patients with this chronic disease.

  17. Spontaneous alteration from Rathke's cleft cyst to craniopharyngioma--possible involvement of transformation between these pathologies.

    Science.gov (United States)

    Ogawa, Yoshikazu; Watanabe, Mika; Tominaga, Teiji

    2014-12-01

    Both Rathke's cleft cyst and craniopharyngioma are considered to arise from the remnants of Rathke's diverticulum despite the quite different histological characteristics. These two lesions may consist of a disease spectrum extending from Rathke's cleft cyst to craniopharyngioma. However, in spite of increasing evidence of these intermediate histologies, very few cases of the actual transformation from Rathke's cleft cyst to craniopharyngioma have been reported in the same patient. A 47-year-old man suffered from recurrent visual dysfunction. Aspiration and partial cystectomy was performed to a suprasellar massive cystic lesion. The histological diagnosis was Rathke's cleft cyst with a small component of squamous metaplasia. Seven months later, the cyst was re-expanded. The cyst wall was irregularly thickened. Re-operation was performed, and the thickened anterior wall was widely removed. Postoperative histological examination showed multiplication of stratified squamous epithelia forming a papillary arrangement. Ki-67 staining showed positive cells randomly distributed not only in the basal layer but also in various epithelial layers, with a labeling index of more than 20 %. The histological diagnosis was squamous papillary type of craniopharyngioma with high potential of proliferation. Subsequent immunohistochemical examinations showed positive reaction to cytokeratin 8 only in the initial epithelium and negative in the latter epithelium. The present case was thought as an actual evidence of the proposed link between Rathke's cleft cyst and craniopharyngioma. Cytokeratin 8 could be the important examination to differentiate Rathke's cleft cyst from craniopharyngioma.

  18. Does the calcification of adamantinomatous craniopharyngioma resemble the calcium deposition of osteogenesis/odontogenesis?

    Science.gov (United States)

    Song-Tao, Qi; Xiao-Rong, Yan; Jun, Pan; Yong-Jian, Deng; Jin, Liang; Guang-Long, Huang; Yun-Tao, Lu; Jian, Ruan; Xiang-Zhao, Li; Jia-Ming, Xu

    2014-02-01

    Calcification in adamantinomatous craniopharyngioma (ACP) is troublesome for surgical intervention. The aim of this study was to examine the osteogenic proteins that play important roles in the calcium deposition of the odontogenic/osteogenic tissues in craniopharyngioma. Craniopharyngiomas (n = 89) were investigated for the presence and expression pattern of the osteoinductive/odontoinductive factor bone morphogenetic protein-2 (Bmp2) and two osteoblastic differentiation makers, Runt-related transcription factor-2 (Runx2) and Osterix, using immunohistochemistry and Western blotting. Our results showed that Bmp2, Runx2 and Osterix levels increased in cases with high calcification and correlated positively with the degree of calcification in ACP, whereas they showed little or no expression in squamous papillary craniopharyngioma. In ACP, Bmp2 was expressed primarily in the stellate reticulum and whorl-like array cells; Runx2 and Osterix tended to be expressed in calcification-related epithelia, including whorl-like array cells and epithelia in/around wet keratin and calcification lesions. Our study indicated, for the first time, that osteogenic factor Bmp2 may play an important role in the calcification of ACP via autocrine or paracrine mechanisms. Given the presence of osteogenic markers (Runx2 and Osterix), craniopharyngioma cells could differentiate into an osteoblast-like lineage, and the process of craniopharyngioma calcification resembles that which occurs in osteogenesis/odontogenesis. © 2014 John Wiley & Sons Ltd.

  19. Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

    Science.gov (United States)

    Sughrue, Michael E.; Yang, Isaac; Kane, Ari J.; Fang, Shanna; Clark, Aaron J.; Aranda, Derrick; Barani, Igor J.

    2010-01-01

    Craniopharyngiomas are locally aggressive tumors which typically are focused in the sellar and suprasellar region near a number of critical neural and vascular structures mediating endocrinologic, behavioral, and visual functions. The present study aims to summarize and compare the published literature regarding morbidity resulting from treatment of craniopharyngioma. We performed a comprehensive search of the published English language literature to identify studies publishing outcome data of patients undergoing surgery for craniopharyngioma. Comparisons of the rates of endocrine, vascular, neurological, and visual complications were performed using Pearson’s chi-squared test, and covariates of interest were fitted into a multivariate logistic regression model. In our data set, 540 patients underwent surgical resection of their tumor. 138 patients received biopsy alone followed by some form of radiotherapy. Mean overall follow-up for all patients in these studies was 54 ± 1.8 months. The overall rate of new endocrinopathy for all patients undergoing surgical resection of their mass was 37% (95% CI = 33–41). Patients receiving GTR had over 2.5 times the rate of developing at least one endocrinopathy compared to patients receiving STR alone or STR + XRT (52 vs. 19 vs. 20%, χ2P < 0.00001). On multivariate analysis, GTR conferred a significant increase in the risk of endocrinopathy compared to STR + XRT (OR = 3.45, 95% CI = 2.05–5.81, P < 0.00001), after controlling for study size and the presence of significant hypothalamic involvement. There was a statistical trend towards worse visual outcomes in patients receiving XRT after STR compared to GTR or STR alone (GTR = 3.5% vs. STR 2.1% vs. STR + XRT 6.4%, P = 0.11). Given the difficulty in obtaining class 1 data regarding the treatment of this tumor, this study can serve as an estimate of expected outcomes for these patients, and guide decision making until these data are available. PMID

  20. Partial removal and post-operative irradiation for craniopharyngioma

    International Nuclear Information System (INIS)

    Nishioka, Takeshi; Shirato, Hiroki; Arimoto, Takuro; Kamata, Tadashi; Suzuki, Keishiro; Kitahara, Toshihiro.

    1992-01-01

    From 1971 to 1990, sixteen patients with craniopharyngioma were treated by postoperative radiotherapy (RT). Fourteen patients underwent partial removal and RT as primary treatment. In two patients, partial removal and RT were performed for treatment of recurrence after total removal. Total dose was between 40 Gy and 60 Gy. Five year survival and 5-year relapse free rates after RT were 100% and 71.7%, respectively. Computed tomography raised suspicion of radiation necrosis in two patients, each treated with two opposing lateral ports. The following hormonal replacements were needed: adrenal in 73% of the patients, thyroid in 66%, growth hormone in 50% of the children, and antidiuretic hormone in 21%. The amounts of thyroid and adrenal hormones for substitution have increased gradually with elapsed time after RT. To minimize these complications and to achieve tumor control, a multiport technique including radiosurgery, and a total dose of 50-55 Gy with 2 Gy per fraction is suggested to be optimal. (author)

  1. Hypothalamic obesity after craniopharyngioma: mechanisms, diagnosis, and treatment

    Directory of Open Access Journals (Sweden)

    Robert H. Lustig

    2011-11-01

    Full Text Available Obesity is a common complication after craniopharyngioma therapy, occurring in up to 75% of survivors. Its weight gain is unlike that of normal obesity, in that it occurs even with caloric restriction, and attempts at lifestyle modification are useless to prevent or treat the obesity. The pathogenesis of this condition involves the inability to transduce afferent hormonal signals of adiposity, in effect mimicking a state of CNS starvation. Efferent sympathetic activity drops, resulting in malaise and reduced energy expenditure, and vagal activity increases, resulting in increased insulin secretion and adipogenesis. Lifestyle intervention is essentially useless in this syndrome, termed hypothalamic obesity. Pharmacologic treatment is also difficult, consisting of adrenergics to mimic sympathetic activity, or suppression of insulin secretion with octreotide, or both. Recently, bariatric surgery (Roux-en-Y gastric bypass, laparoscopic gastric banding, truncal vagotomy have also been attempted with variable results. Early and intensive management is required to mitigate the obesity and its negative consequences.

  2. Partial removal and post-operative irradiation for craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Nishioka, Takeshi; Shirato, Hiroki (Obihiro Kousei Hospital, Hokkaido (Japan)); Arimoto, Takuro; Kamata, Tadashi; Suzuki, Keishiro; Kitahara, Toshihiro

    1992-12-01

    From 1971 to 1990, sixteen patients with craniopharyngioma were treated by postoperative radiotherapy (RT). Fourteen patients underwent partial removal and RT as primary treatment. In two patients, partial removal and RT were performed for treatment of recurrence after total removal. Total dose was between 40 Gy and 60 Gy. Five year survival and 5-year relapse free rates after RT were 100% and 71.7%, respectively. Computed tomography raised suspicion of radiation necrosis in two patients, each treated with two opposing lateral ports. The following hormonal replacements were needed: adrenal in 73% of the patients, thyroid in 66%, growth hormone in 50% of the children, and antidiuretic hormone in 21%. The amounts of thyroid and adrenal hormones for substitution have increased gradually with elapsed time after RT. To minimize these complications and to achieve tumor control, a multiport technique including radiosurgery, and a total dose of 50-55 Gy with 2 Gy per fraction is suggested to be optimal. (author).

  3. Dosimetry of P-32 radiocolloid for treatment of cystic craniopharyngioma

    International Nuclear Information System (INIS)

    Sadeghi, Mahdi; Moradi, Somayeh; Shahzadi, Sohrab; Pourbeigi, Hossien

    2008-01-01

    Full text: In Tajrish Shohada hospital, patients with either cystic craniopharyngiomas or cystic astrocytomas have been treated with P-32 radiocolloid by Stereotactic Procedure. The total activity was prescribed for deliver dose between 200 Gy to 300 Gy to the cyst wall thicknesses of 1 mm to 3 mm. In this project, MD-55-2 radiochromic film was utilized to determine the dose distribution around the source. The film dosimetry data was compared with Monte Carlo simulated values calculated with MCNP4C code. In addition, the clinical and dosimetric factors such as the volume of cyst, method of radiocolloid injection was evaluated. The required activity and distribution of radial dose in and out of cyst wall have been investigated. (author)

  4. Distinct patterns of primary and motile cilia in Rathke's cleft cysts and craniopharyngioma subtypes.

    Science.gov (United States)

    Coy, Shannon; Du, Ziming; Sheu, Shu-Hsien; Woo, Terri; Rodriguez, Fausto J; Kieran, Mark W; Santagata, Sandro

    2016-12-01

    Cilia are highly conserved organelles, which serve critical roles in development and physiology. Motile cilia are expressed in a limited range of tissues, where they principally regulate local extracellular fluid dynamics. In contrast, primary cilia are expressed by many vertebrate cell types during interphase, and are intimately involved in the cell cycle and signal transduction. Notably, primary cilia are essential for vertebrate hedgehog pathway activity. Improved detection of motile cilia may assist in the diagnosis of some pathologic entities such as Rathke's cleft cysts, whereas characterizing primary cilia in neoplastic tissues may implicate cilia-dependent signaling pathways as critical for tumorigenesis. We show that immunohistochemistry for the nuclear transcription factor FOXJ1, a master regulator of motile ciliogenesis, robustly labels the motile ciliated epithelium of Rathke's cleft cysts. FOXJ1 expression discriminates Rathke's cleft cysts from entities in the sellar/suprasellar region with overlapping histologic features such as craniopharyngiomas. Co-immunohistochemistry for FOXJ1 and markers that highlight motile cilia such as acetylated tubulin (TUBA4A) and the small GTPase ARL13B further enhance the ability to identify diagnostic epithelial cells. In addition to highlighting motile cilia, ARL13B immunohistochemistry also robustly highlights primary cilia in formalin-fixed paraffin-embedded sections. Primary cilia are present throughout the neoplastic epithelium of adamantinomatous craniopharyngioma, but are limited to basally oriented cells near the fibrovascular stroma in papillary craniopharyngioma. Consistent with this differing pattern of primary ciliation, adamantinomatous craniopharyngiomas express significantly higher levels of SHH, and downstream targets such as PTCH1 and GLI2, compared with papillary craniopharyngiomas. In conclusion, motile ciliated epithelium can be readily identified using immunohistochemistry for FOXJ1, TUBA4A, and

  5. Analysis and Long-Term Follow-Up of the Surgical Treatment of Children With Craniopharyngioma.

    Science.gov (United States)

    Cheng, Jing; Shao, Qiang; Pan, Zhiyong; You, Jin

    2016-11-01

    To investigate the relationship between the operative approach, clinical pathological factors, and curative effect of the surgical treatment in the patients with craniopharyngioma; to provide a theoretical basis for determining the prognosis and reducing the recurrence rate during the long-term postoperative follow-up in children. This was a retrospective analysis of the clinical data of 92 children who underwent surgical treatment in our department from May 2011 to January 2005. Long-term follow-up was performed from 12 months to 8 years. The pterional approach was used in 49 patients, the interhemispheric approach in 20 patients, the corpus callosum approach in 16 patients, and the butterfly approach in 7 patients. Pathological classification was performed by hematoxylin and eosin stain staining of the pathological tissues and evaluated according to the different surgical approaches, MRI calcification status, calcification type, pathological type, whether radiotherapy was performed, postoperative recurrence, and death. For the pterion approach resection, there was near total resection in 46 patients (93.9%) with the lowest recurrence rate. The operative approach and postoperative recurrence rates were compared; the difference was statistically significant (P 0.05). There was not a significant difference between the MRI classification and postoperative recurrence rate (P >0.05). Comparing the degree of tumor calcification with the recurrence rate after operation and the mortality rate, the difference was statistically significant (P craniopharyngioma and squamous papillary craniopharyngioma in 2 groups following operation were compared, and the differences were statistically significant (P craniopharyngioma relapse rate is higher, which could be because invasion of craniopharyngioma only occurs with adamantimous craniopharyngioma. Postoperative radiotherapy can significantly prolong the recurrence time and reduce the mortality rate of patients with

  6. Psychological well-being and independent living of young adults with childhood-onset craniopharyngioma.

    Science.gov (United States)

    Memmesheimer, Rodica Mia; Lange, Karin; Dölle, Michael; Heger, Sabine; Mueller, Iris

    2017-08-01

    To assess the psychological well-being and social integration of adults with craniopharyngioma diagnosed in childhood. A cross-sectional study of a nationwide cohort of young adults with craniopharyngioma in Germany was performed. A structured questionnaire covered the sociodemographic, clinical data, and subjective effects of the condition on social integration. Psychological well-being was assessed using the Hospital Anxiety and Depression Scale (HADS). Results were compared to young adults with type 1 diabetes mellitus (T1DM). The study included 59 participants (29 females, 30 males; mean age 25y 2mo [SD 5y 10mo]), mean age at first surgery 10y 2mo [SD 3y 7mo]. Compared to the T1DM group, significantly more young people with craniopharyngioma aged 25 to 35 years lived at their parents' homes (craniopharyngioma 43.34%; T1DM 13.7%; χ 2 =4.14, p=0.049), and fewer lived in a relationship (craniopharyngioma 8.69%; T1DM 54.7%; χ 2 =15.74, p<0.001). The HADS revealed a score for depression above the cut-off in 20.69 per cent of young adults with craniopharyngioma and in 6 per cent of young adults with T1DM (χ 2 =13.42, p<0.001). Young adults with craniopharyngioma reported subjective disadvantages in professional and social integration. Further, they presented with reduced well-being and increased depression rates. Better psychosocial support and self-management education might reduce the long-term burden of the disease. © 2017 Mac Keith Press.

  7. [Transient enlargement of craniopharyngioma cysts after stereotactic radiotherapy and radiosurgery].

    Science.gov (United States)

    Mazerkina, N A; Savateev, A N; Gorelyshev, S K; Konovalov, A N; Trunin, Yu Yu; Golanov, A V; Medvedeva, O A; Kalinin, P L; Kutin, M A; Astafieva, L I; Krasnova, T S; Ozerova, V I; Serova, N K; Butenko, E I; Strunina, Yu V

    Stereotactic radiotherapy/radiosurgery (RT/ES) is an effective technique for treating craniopharyngiomas (CPs). However, enlargement of the cystic part of the tumor occurs in some cases after irradiation. The enlargement may be transient and not require treatment or be a true relapse requiring treatment. In this study, we performed a retrospective analysis of 79 pediatric patients who underwent stereotactic RT or RS after resection of craniopharyngioma. Five-year relapse-free survival after complex treatment of CP was 86%. In the early period after irradiation, 3.5 months (2.7-9.4) on average, enlargement of the cystic component of the tumor was detected in 10 (12.7%) patients; in 9 (11.4%) of them, the enlargement was transient and did not require treatment; in one case, the patient underwent surgery due to reduced visual acuity. In 8 (10.1%) patients, an increase in the residual tumor (a solid component of the tumor in 2 cases and a cystic component of the tumor in 6 cases) occurred in the long-term period after irradiation - after 26.3 months (16.6-48.9) and did not decrease during follow-up in none of the cases, i.e. continued growth of the tumor was diagnosed. A statistical analysis revealed that differences in the terms of transient enlargement and true continued growth were statistically significant (pcraniopharyngioma cyst in the early period (up to 1 year) after RT/RS is usually transient and does not require surgical treatment (except cases where worsening of neurological symptoms occurs, or occlusive hydrocephalus develops).

  8. Intracapsular irradiation therapy of craniopharyngiomas with radioactive gold

    International Nuclear Information System (INIS)

    Kodama, Takafumi; Matsukado, Yasuhiko; Uemura, Shozaburo

    1981-01-01

    Sixteen cases out of 27 patients with craniopharyngiomas were arbitrarily subjected to combined treatment of simple surgical evacuation and intracapsular irradiation with 198-Au. Follow-up studies were performed on 15 cases and they ranged from 6 months to 11 years. One patient was omitted from the study because of a short postoperative period. Immediate postoperative morbidity and the endocrine functions at the end of the follow-up study were compared with those of the patients who underwent extensive surgical resection of the tumors. Intracapsular irradiation with 198-Au was found to have satisfactory effects in the treatment of cystic craniopharyngioma, especially in recurrent cases of initially solid tumors, with respect to the preservation of the endocrine functions and the daily activity of the patients. The immediate postoperative hazards in the patients' care were also much less and they were found to be easily manageable. The patients, who had been followed up for over 5 years, maintained an occupational IQ score in the normal range and the patients under school age were all able to continue their school lives. One of the female patients, who had been married after the treatment, could have two children without any specific replacement therapy, and another patient in childhood who had shown physical retardation due to HGH deficiency, showed favorable results with crescormon administration in comparison with cases of extensive resection. Although the dosimetric value of 198-Au should be varied according to the size and thickness of the capsule, it was found that 15 to 30 mCi of 198-Au was the appropriate dosis for treatment. (author)

  9. Computerized tomographic evaluations of 37 cases of craniopharyngiomas

    International Nuclear Information System (INIS)

    Nagasawa, Shiro; Yamashita, Junkoh; Handa, Hajime; Takeuchi, Juji.

    1983-01-01

    Although craniopharyngiomas are well known to exhibit three basic CT characteristics: Calcification, cysts, and contrast enhancement (CE), several cases with atypical CT manifestations have been reported lately. These atypical manifestations can be classified into three groups: Unusual extensions of the tumors, high-density cysts, and marked homogeneous CE. The CT scans obtained in our recent series of 37 craniopharyngiomas were evaluated in an attempt to analyze the tumoral extensions, the cyst densities, and the CE, and were compared with those of other, rarer parasellar lesions. Cystic tumors were much more frequent than solid tumors (Table 1); 14 cases (38%) were exclusively cystic; 17 cases (46%), equally cystic and solid, and 6 cases (16%), solid. Calcifications were present in 86% of the children and 43% of the adults. Unusual extensions were noted in 7 cases (Fig. 1, 2): Anteriorly to the frontal fossa (5 cases, 14%), laterally to the temporal fossa (1 case, 3%), or posteriorly to the posterior fossa (1 case, 3%). All these tumors were either purely cystic or equally cystic and solid. Cystic portions were detected in the tumors of 31 cases (Table 2). Although the majority of the cystic portions (25 cases, 81%) were low in density, isodense cysts were observed in 6 cases (19%). Out of 23 cases exhibiting solid portions in the tumors, high-density areas (calcified masses) were detected in 3 cases and mixed-density areas in 20 cases. While these 20 cases all showed CE, marked homogeneous CE were noted in 5 cases (25%). (J.P.N.)

  10. Craniopharyngiomas: identification of different semiological patterns with MRI

    Energy Technology Data Exchange (ETDEWEB)

    Molla, Enrique [Magnetic Resonance and Radiology Service, Valencia (Spain); Department of Morphologic Sciences, University of Valencia (Spain); Marti-Bonmati, Luis [Hospital Universitario Doctor Peset, Valencia (Spain); Magnetic Resonance and Radiology Service, Valencia (Spain); Revert, Antonio [Department of Radiology, Hospital La Ribera, Alzira, 46017 Valencia (Spain); Arana, Estanislao; Dosda, Rosa [Magnetic Resonance and Radiology Service, Valencia (Spain); Menor, Francisco [Department of Radiology, Infantil La Fe University Hospital, 46017 Valencia (Spain); Poyatos, Cecilio [Department of Radiology, Doctor Peset University Hospital, Valencia (Spain)

    2002-07-01

    Our objectives were to analyze different semiological patterns in craniopharyngiomas studied with CT and MR sequences. Retrospective study of 26 patients with confirmed craniopharyngiomas. All cases were examined with CT and MR imaging using a variety of pulse sequences (spin echo, inversion recovery, gradient echo in-phase and opposed-phase). The analyzed component patterns were classified as solid, calcium, proteic-like, cerebrospinal fluid (CSF)-like, hematic-like, and fatty patterns. The different patterns were related by means of contingency tables and the Fisher exact test and also to epidemiological findings and tumor size. A solid pole was detected in all patients, whereas a cystic component was present in 92.3% of the cases. Calcification was visualized in 65.3%, proteic-like in 53.8%, CSF-like in 23%, hematic-like in 19.2%, and fatty component in 15.3%. There were no statistical associations between patterns, with the exception that in no case did CSF-like and proteic-like patterns coexist (P=0.004). Tumor size was related to components. Hematic-like (17.0{+-}18.9 vs 3.9{+-}2.6 mm, non-present vs present) and CSF-like (16.9{+-}19.6 vs 6.5{+-}4.0 mm) patterns were observed most frequently in smaller tumors, whereas larger tumors usually had proteic-like (5.9{+-}5.4 vs 21.1{+-}21.0 mm) and calcified (4.6{+-}1.9 vs 19.1{+-}19.9 mm) patterns. Computed tomography and a combination of different MR images frequently allow the detection of different semiological patterns in these tumors. Semiological patterns were correlated only to tumor size. (orig.)

  11. Sequential pathological changes during malignant transformation of a craniopharyngioma: A case report and review of the literature

    Science.gov (United States)

    Negoto, Tetsuya; Sakata, Kiyohiko; Aoki, Takachika; Orito, Kimihiko; Nakashima, Shinji; Hirohata, Masaru; Sugita, Yasuo; Morioka, Motohiro

    2015-01-01

    Background: Malignant transformation of craniopharyngiomas is quite rare, and the etiology of transformation remains unclear. The prognosis of malignantly transformed craniopharyngiomas is very poor. Case Description: A 36-year-old male had five craniotomies, five transsphenoidal surgeries, and two radiation treatments until 31 years of age after diagnosis of craniopharyngioma at 12 years of age. All serial pathological findings indicated adamantinomatous craniopharyngioma including those of a surgery performed for tumor regrowth at 31 years of age. However, when the tumor recurred approximately 5 years later, the pathological findings showed squamous metaplasia. The patient received CyberKnife surgery, but the tumor rapidly regrew within 4 months. The tumor was resected with the cavernous sinus via a dual approach: Transcranial and transsphenoidal surgery with an extracranial-intracranial bypass using the radial artery. Pathologic examination of a surgical specimen showed that it consisted primarily of squamous cells; the lamina propria was collapsed, and the tumor cells had enlarged nuclei and clarification of the nucleolus. The tumor was ultimately diagnosed as malignant transformation of craniopharyngioma. After surgery, he received combination chemotherapy (docetaxel, cisplatin, and fluorouracil). The tumor has been well controlled for more than 12 months. Conclusion: Serial pathological changes of the craniopharyngioma and a review of the 20 cases reported in the literature suggest that radiation of the squamous epithelial cell component of the craniopharyngioma led to malignant transformation via squamous metaplasia. We recommend aggressive surgical removal of craniopharyngiomas and avoidance of radiotherapy if possible. PMID:25883842

  12. Eating behavior, weight problems and eating disorders in 101 long-term survivors of childhood-onset craniopharyngioma

    NARCIS (Netherlands)

    Hoffmann, Anika; Postma, Frank P.; Sterkenburg, Anthe S.; Gebhardt, Ursel; Mueller, Hermann L.

    Background: As a result of hypothalamic involvement and/or treatment-related hypothalamic damage, up to 75% of childhood craniopharyngioma patients develop hypothalamic obesity. Methods: Eating behavior was analyzed in 101 survivors of childhood craniopharyngioma, recruited from 1980 to 2001 in the

  13. Dramatic Response of BRAF V600E Mutant Papillary Craniopharyngioma to Targeted Therapy.

    Science.gov (United States)

    Brastianos, Priscilla K; Shankar, Ganesh M; Gill, Corey M; Taylor-Weiner, Amaro; Nayyar, Naema; Panka, David J; Sullivan, Ryan J; Frederick, Dennie T; Abedalthagafi, Malak; Jones, Pamela S; Dunn, Ian F; Nahed, Brian V; Romero, Javier M; Louis, David N; Getz, Gad; Cahill, Daniel P; Santagata, Sandro; Curry, William T; Barker, Fred G

    2016-02-01

    We recently reported that BRAF V600E is the principal oncogenic driver of papillary craniopharyngioma, a highly morbid intracranial tumor commonly refractory to treatment. Here, we describe our treatment of a man age 39 years with multiply recurrent BRAF V600E craniopharyngioma using dabrafenib (150mg, orally twice daily) and trametinib (2mg, orally twice daily). After 35 days of treatment, tumor volume was reduced by 85%. Mutations that commonly mediate resistance to MAPK pathway inhibition were not detected in a post-treatment sample by whole exome sequencing. A blood-based BRAF V600E assay detected circulating BRAF V600E in the patient's blood. Re-evaluation of the existing management paradigms for craniopharyngioma is warranted, as patient morbidity might be reduced by noninvasive mutation testing and neoadjuvant-targeted treatment. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  14. Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma.

    Science.gov (United States)

    Müller, Hermann L

    2016-05-01

    Craniopharyngiomas are rare embryonic malformations of the sellar/parasellar region with low histological grade. Here, we review findings on the diagnosis, treatment, clinical course, follow-up, and prognosis of craniopharyngioma patients. Clinical manifestations develop from increased intracranial pressure, anterior visual pathway damage, and hypothalamic/pituitary deficiencies. If the tumor is favorably localized (no anatomical involvement with the hypothalamic and optical structures) therapy of choice is complete resection, meticulously performed to preserve hypothalamic and optic functions. In patients with unfavorable tumor involvement, optimal therapy is limited hypothalamus-sparing surgical strategy, followed by judicious irradiation dosage to minimize recurrences and progression. Surgical lesions and/or anatomical involvement of posterior hypothalamic areas result in serious sequelae, mainly hypothalamic syndrome. Craniopharyngioma is a chronic disease and must be managed as such, providing ongoing care of pediatric and adult patients by experienced multidisciplinary teams in the context of multicenter trials.

  15. From cerebral salt wasting to diabetes insipidus with adipsia: case report of a child with craniopharyngioma.

    Science.gov (United States)

    Raghunathan, Veena; Dhaliwal, Maninder Singh; Gupta, Aditya; Jevalikar, Ganesh

    2015-03-01

    Craniopharyngioma is associated with a wide and interesting variety of sodium states both by itself and following surgical resection. These are often challenging to diagnose, especially given their dynamic nature during the perioperative course. We present the case of a boy with craniopharyngioma who had hyponatremia due to cerebral salt wasting preoperatively, developed diabetes insipidus (DI) intraoperatively and proceeded to develop hypernatremia with adipsic DI. Cerebral salt wasting is a rare presenting feature of craniopharyngioma. Postoperative DI can be associated with thirst abnormalities including adipsia due to hypothalamic damage; careful monitoring and a high index of suspicion are required for its detection. Adipsic DI is a difficult condition to manage; hence a conservative surgical approach is suggested.

  16. Predictors of visual outcome in patients operated for craniopharyngioma - a Danish national study

    DEFF Research Database (Denmark)

    Jacobsen, Mads Forslund; Thomsen, Ann Sofia Skou; Bach-Holm, Daniella

    2018-01-01

    Purpose Craniopharyngioma often causes visual loss due to the close relation to the anterior visual pathways. This study investigates the incidence and predictors of visual outcomes in patients with craniopharyngioma. Methods Data from sixty-six patients who underwent surgery for craniopharyngioma...... from 2009 to 2013 in Denmark were reviewed. Primary outcomes were visual acuity (VA) and visual field (VF) defects from pre-and postoperative visits. Secondary outcomes were optic nerve atrophy (OA) and papilledema. Results Fifty-eight patients were included. The VA of the patients 1-year after surgery...... = 0.011 and p = 0.011, respectively). Patients undergoing surgery within a week or less after their first ophthalmological examination had a significant improvement in VA (−0.36; 95%CI: −0.62 to −0.09; p = 0.0099). Patients undergoing surgery using a subfrontal approach also showed improvement in VA...

  17. Nonalcoholic fatty liver disease in long-term survivors of childhood-onset craniopharyngioma

    Directory of Open Access Journals (Sweden)

    So Yoon Jung

    2017-09-01

    Full Text Available Purpose Hypothalamic obesity in childhood-onset (CO- craniopharyngioma patients may predispose to nonalcoholic fatty liver disease (NAFLD. This study reviewed the characteristics of NAFLD associated with CO-craniopharyngioma. Methods This study retrospectively reviewed 75 patients who underwent surgery for craniopharyngioma while younger than 15 years of age between 2000 and 2016. Results Elevated aspartate aminotransferase (AST or alanine aminotransferase (ALT above 40 IU/L was observed in 51 of the 75 (68% CO-craniopharyngioma patients. Imaging studies were performed in 32 patients with elevated liver enzymes. The estimated prevalence of NAFLD in CO-craniopharyngioma was 47%. NAFLD was detected in 22 patients (male 59%, 4.3±4.0 years after first surgery. The mean age at the time of the initial operation was 9.1±2.9 years. Six patients (27.3% were diagnosed within 1 year. Among the 19 patients with initial height and weight data, the body mass index (BMI z-score (BMI_Z at the time of diagnosis with NAFLD was 1.37±1.01 (range, -0.75 to 3.18, with 4 patients (18.2% being overweight and 9 (40.9% being obese. BMI_Z increased above BMI_Z at the time of the operation in 13 patients (68.4%. The increment in BMI_Z was 1.13 (range, 0.10–2.84. Seventeen patients did not receive growth hormone. An insulin-like growth factor-I level <3rd percentile was observed in 19 patients. Conclusions NAFLD is common in survivors of CO-craniopharyngioma and may develop earlier. If the ALT or AST is above 40 IU/L, a diagnostic work-up should be started.

  18. Visceral adiposity index as an indicator of cardiometabolic risk in patients treated for craniopharyngioma.

    Science.gov (United States)

    Ferraù, Francesco; Spagnolo, Federica; Cotta, Oana Ruxandra; Cannavò, Laura; Alibrandi, Angela; Russo, Giuseppina Tiziana; Aversa, Tommaso; Trimarchi, Francesco; Cannavò, Salvatore

    2017-11-01

    Craniopharyngioma is associated with metabolic alterations leading to increased cardiovascular mortality. Recently, the visceral adiposity index has been proposed as a marker of visceral adipose tissue dysfunction and of the related cardiometabolic risk. The role of the visceral adiposity index has never been explored in craniopharyngioma patients. We assessed the cardiometabolic risk on the basis of the visceral adiposity index in craniopharyngioma patients. We evaluated data of 24 patients treated for craniopharyngioma in a single-centre. We investigated the relationship among patients' clinical and biochemical features, cardiovascular risk -assessed by the Framingham and the atherosclerotic cardiovascular disease risk scores-, visceral adiposity index and adipose tissue dysfunction severity. Increased visceral adiposity index was found in 8 patients (33%). Adipose tissue dysfunction resulted to be severe, moderate or mild in 5, 2 and 1 cases. Increased visceral adiposity index significantly correlated with the occurrence of metabolic syndrome (p 0.027), IRI (p 0.001), triglycerides (p < 0.001), HOMA-IR (p < 0.001) and with lower ISI-Matsuda (p 0.005) and HDL-cholesterol (p < 0.001). Higher degree of adipose tissue dysfunction associated with increased insulin resistance. No gender difference was found for visceral adiposity index, adipose tissue dysfunction severity, and cardiovascular risk scores. Patients with adulthood onset craniopharyngioma showed higher Framingham risk score (p 0.004), atherosclerotic cardiovascular disease 10-year (p < 0.001) and lifetime (p 0.018) risk scores than those with childhood onset disease. Visceral adiposity index is increased in one third of our patients with craniopharyngioma, even if metabolic syndrome does not occur. Increased visceral adiposity index and adipose tissue dysfunction severity correlate with insulin sensitivity parameters, do not correlate with Framingham or atherosclerotic cardiovascular

  19. Nonalcoholic fatty liver disease in long-term survivors of childhood-onset craniopharyngioma.

    Science.gov (United States)

    Jung, So Yoon; Lee, Yun Jeong; Lee, Hye Jin; Lee, Young Ah; Moon, Jin Soo; Ko, Jae Sung; Yang, Sei Won; Shin, Choong Ho

    2017-09-01

    Hypothalamic obesity in childhood-onset (CO-) craniopharyngioma patients may predispose to nonalcoholic fatty liver disease (NAFLD). This study reviewed the characteristics of NAFLD associated with CO-craniopharyngioma. This study retrospectively reviewed 75 patients who underwent surgery for craniopharyngioma while younger than 15 years of age between 2000 and 2016. Elevated aspartate aminotransferase (AST) or alanine aminotransferase (ALT) above 40 IU/L was observed in 51 of the 75 (68%) CO-craniopharyngioma patients. Imaging studies were performed in 32 patients with elevated liver enzymes. The estimated prevalence of NAFLD in CO-craniopharyngioma was 47%. NAFLD was detected in 22 patients (male 59%, 4.3±4.0 years after first surgery). The mean age at the time of the initial operation was 9.1±2.9 years. Six patients (27.3%) were diagnosed within 1 year. Among the 19 patients with initial height and weight data, the body mass index (BMI) z-score (BMI_Z) at the time of diagnosis with NAFLD was 1.37±1.01 (range, -0.75 to 3.18), with 4 patients (18.2%) being overweight and 9 (40.9%) being obese. BMI_Z increased above BMI_Z at the time of the operation in 13 patients (68.4%). The increment in BMI_Z was 1.13 (range, 0.10-2.84). Seventeen patients did not receive growth hormone. An insulin-like growth factor-I level <3rd percentile was observed in 19 patients. NAFLD is common in survivors of CO-craniopharyngioma and may develop earlier. If the ALT or AST is above 40 IU/L, a diagnostic work-up should be started.

  20. Transfrontal and Transsphenoidal Approaches to Pediatric Craniopharyngioma: A National Perspective.

    Science.gov (United States)

    Lin, Yimo; Hansen, Daniel; Sayama, Christina M; Pan, I-Wen; Lam, Sandi

    2017-01-01

    This study compared transsphenoidal (TS) and transfrontal (TF) approaches to craniopharyngioma utilizing a national database. The Kids' Inpatient Database (2003, 2006, and 2009) was surveyed for patients with a diagnosis of craniopharyngioma who underwent a subset of surgical interventions to compare TS and TF surgery. Demographics, hospital variables, and complications/comorbidities were analyzed with multivariate regression. 314 admissions (TS = 104, TF = 210) were identified. The mean age was 14.8 (TS) versus 9.8 (TF) years (p < 0.001). The mean number of diagnoses was 4.6 (TS) versus 6.2 (TF) (p < 0.001). Diabetes insipidus was associated with 38% (TS) and 69% (TF). Cerebrospinal fluid (CSF) leak affected 19% TS and 4% TF resections. Other complications and comorbidities included postoperative stroke (2% TS vs. 5% TF), panhypopituitarism (5 vs. 8%), death (0 vs. 1%), cranial nerve deficits (1 vs. 6%), thrombotic events (7 vs. 17%), and seizures (0 vs. 12%). 98% of patients were discharged home after a mean 6-day length of stay (LOS) after TS, whereas 90% of TF patients had a LOS of 12 days. TS cases were more likely to be privately insured (68%) and from higher income brackets (61%) than TF ones (56 and 2%, respectively) (p < 0.05). In multivariate regression models adjusting for age, sex, race, number of diagnoses, surgical approach, hospital volume, and insurance type, the TS approach was associated with an increased incidence of CSF leak (OR 10, p < 0.001). More documented diagnoses (OR 16-60, p < 0.01) and TF approach (OR 2.6, p < 0.01) were associated with an increased incidence of other complications and comorbidities. Age younger than 10 (β-coefficient 2.3, p = 0.01), more diagnoses (β-coefficient 1.2, p < 0.001), and TF approach (β- coefficient 3.0, p < 0.01) were associated with increased LOS. A higher number of diagnoses were associated with nonhome discharge destinations (β-coefficient 1.29, p < 0.001). TS surgery was associated with an increased

  1. Acute presentation of craniopharyngioma in children and adults in a Danish national cohort

    DEFF Research Database (Denmark)

    Nielsen, E H; Jørgensen, J O; Bjerre, Peter Bjørn

    2013-01-01

    We aimed to study the occurrence of acute-onset symptoms at initial presentation in a national Danish cohort of patients with childhood- or adult-onset craniopharyngioma, and to investigate potential risk factors for acute presentation. Medical records of 189 consecutive patients (39 children, 150...... adults) presenting with craniopharyngioma during the period 1985-2004 were reviewed, and data regarding initial symptoms, neuroimaging results, vision and pituitary function were systematically collected. Acute symptoms preceding hospital admission were noted. Subgroup analyses were based on age, gender...

  2. Magnetic resonance cystography with gadopenetate dimeglumine of a cystic craniopharyngioma in a child - a technical note

    Energy Technology Data Exchange (ETDEWEB)

    Greenberg, S.B. [St. Christopher' s Hospital for Children, Philadelphia, PA (United States); Arkansas Children' s Hospital, Department of Radiology, 800 Marshall Street, Little Rock, AR 72202 (United States); Magram, G. [St. Christopher' s Hospital for Children, Philadelphia, PA (United States)

    2000-02-01

    Large cystic craniopharyngiomas can be treated with chemotherapy injected directly into the cyst. Chemotherapy is toxic if it leaks from the cyst into the subarachnoid space. We present a child with a cystic craniopharyngioma following surgical placement of a catheter into the cystic component. Computed tomography following iodinated contrast injection into the cyst was inconclusive in determining the cyst wall integrity. Magnetic resonance following dilute gadopentetate dimeglumine injection into the cyst clearly defined a leak into the subarachnoid space. Coronal imaging was especially helpful. This technique is simple to perform and useful for clinical management. (orig.)

  3. Diagnostics, treatment, and follow-up in craniopharyngioma

    Directory of Open Access Journals (Sweden)

    Hermann L. Müller

    2011-11-01

    Full Text Available Craniopharyngiomas (CP are partly cystic embryogenic malformations of the sellar and parasellar region. With an overall incidence of 0.5 to 2.0 new cases / million population / year, approximately 30 to 50% of all cases represent childhood CP. Typical manifestations at diagnosis are headache, visual impairment, polyuria/polydypsia, growth retardation, and significant weight gain. Therapy of choice in patients with favorable tumor localization is complete resection with the intention to maintain optical nerve and hypothalamic-pituitary functions. In patients with unfavorable tumor localization (hypothalamic involvement, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%. Recurrences after complete resection and progressions of residual tumor after incomplete resection are frequent post-surgical events. Accordingly, the appropriate time point of irradiation after incomplete resection is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007. Quality of life is substantially reduced in approximately 50% of long-term survivors due to sequelae, notably morbid hypothalamic obesity. Both, childhood CP and adult onset CP should be recognized as chronic diseases requiring constant monitoring of the consequences and appropriate medical resources for treatment in order to provide optimal quality of survival for patients.

  4. Using the trans-lamina terminalis route via a pterional approach to resect a retrochiasmatic craniopharyngioma involving the third ventricle.

    Science.gov (United States)

    Weil, Alexander G; Robert, Thomas; Alsaiari, Sultan; Obaid, Sami; Bojanowski, Michel W

    2016-01-01

    Retrochiasmatic craniopharyngiomas involving the anterior third ventricle are challenging to access. Although the pterional approach is a common route for suprasellar lesions, when the craniopharyngioma extends behind the chiasma into the third ventricle, access is even more difficult, and the lamina terminalis may offer a good working window. The translamina terminalis approach provides direct access to the retrochiasmatic portion of the tumor with minimal brain retraction and no manipulation of the visual nerves. In this video, we emphasize the utility of using the lamina terminalis corridor to resect the retrochiasmatic intraventricular portion of a craniopharyngioma. The video can be found here: https://youtu.be/hrLNC0hDKe4 .

  5. Physiological growth hormone replacement and rate of recurrence of craniopharyngioma: the Genentech National Cooperative Growth Study.

    Science.gov (United States)

    Smith, Timothy R; Cote, David J; Jane, John A; Laws, Edward R

    2016-10-01

    OBJECTIVE The object of this study was to establish recurrence rates in patients with craniopharyngioma postoperatively treated with recombinant human growth hormone (rhGH) as a basis for determining the risk of rhGH therapy in the development of recurrent tumor. METHODS The study included 739 pediatric patients with craniopharyngioma who were naïve to GH upon entering the Genentech National Cooperative Growth Study (NCGS) for treatment. Reoperation for tumor recurrence was documented as an adverse event. Cox proportional-hazards regression models were developed for time to recurrence, using age as the outcome and enrollment date as the predictor. Patients without recurrence were treated as censored. Multivariate logistic regression was used to examine the incidence of recurrence with adjustment for the amount of time at risk. RESULTS Fifty recurrences in these 739 surgically treated patients were recorded. The overall craniopharyngioma recurrence rate in the NCGS was 6.8%, with a median follow-up time of 4.3 years (range 0.7-6.4 years.). Age at the time of study enrollment was statistically significant according to both Cox (p = 0.0032) and logistic (p craniopharyngioma after surgery in children, but long-term follow-up of GH-treated patients is required to establish a true natural history in the GH treatment era.

  6. A systematic review of cognitive performance in patients with childhood craniopharyngioma.

    Science.gov (United States)

    Özyurt, Jale; Müller, Hermann L; Thiel, Christiane M

    2015-10-01

    Craniopharyngiomas are rare brain tumors of the sellar/suprasellar region, often adversely affecting patients' physical and psychosocial functioning. Until a few years ago, knowledge on cognitive deficits in craniopharyngioma patients was based on little valid evidence, with considerable inconsistencies across studies. Findings from recent research, with partly larger sample sizes, add to existing evidence to provide a more clear and reliable picture. The current review aims to summarize and systemize current findings on cognitive deficits in childhood craniopharyngioma, taking account of patient- and treatment-related variables where possible. Those studies were included that reported results of childhood craniopharyngioma patients tested with formalized neuropsychological tests (irrespective of their age at study, group size ≥10). A systematic assignment of test results to subcomponents of broader cognitive domains (e.g. to specific memory systems and processes) allows for a first comprehensive overview of patterns of spared and impaired cognitive functions. We show that episodic memory recall in particular is impaired, largely sparing other memory components. In accordance with recent knowledge on mammillary function, patients with hypothalamic involvement appear to be at particular risk. Deficits in higher cognitive processes, relying on the integrity of the prefrontal cortex and its subcortical pathways, may also occur, but results are still inconsistent. To gain deeper insight into the pattern of deficits and their association with patient- and treatment-related variables, further multi-site research with larger cohorts is needed.

  7. BRAF-V600E mutant papillary craniopharyngioma dramatically responds to combination BRAF and MEK inhibitors.

    Science.gov (United States)

    Roque, Ashley; Odia, Yazmin

    2017-04-01

    We present a patient with BRAF-V600E mutant papillary craniopharyngioma successfully treated with combination BRAF (dabrafenib 150 mg twice daily) and MEK (trametinib 2 mg daily) inhibitors after her unresectable tumor proved refractory to radiation. Serial brain MRIs and PET revealed marked tumor reduction with gradual neurological improvement and permanent panhypopituitarism.

  8. Patterns of Care for Craniopharyngioma: Survey of members of the American Association of Neurological Surgeons

    Science.gov (United States)

    Hankinson, Todd C.; Palmeri, Nicholas O.; Williams, Sarah A.; Torok, Michelle R.; Serrano, Cesar A.; Foreman, Nicholas K.; Handler, Michael H.; Liu, Arthur K.

    2014-01-01

    Background/Significance Initial therapy for craniopharyngioma remains controversial. Population-based datasets indicate that traditional algorithms (GTR versus STR +/− XRT) are often not employed. We investigated neurosurgical practice patterns. Methods A ten-question survey was electronically distributed to members of the American Association of Neurological Surgeons. Responses were analyzed using standard statistical techniques. Results One hundred-two responses were collected, with a median 25 craniopharyngiomas managed per respondent. 36% estimated their practice included ≥75% pediatrics and 61% had an academic practice. 36% would recommend observation or radiation therapy for a suspected craniopharyngioma in the absence of a tissue diagnosis, with 46% of these indicating this recommendation in ≥10% of cases. Following STR, 35% always recommend XRT and 59% recommend it in over half of cases. However, following STR or biopsy alone, 18% and 11% never recommend XRT. There was no association between type of practice (i.e. academic or ≥75% pediatrics) and practice patterns. Conclusions This survey verifies that deviation from established algorithms is common, underscoring the clinical complexity of these patients and recent secondary data analyses This should influence clinical researchers to investigate outcomes for patients treated using alternative methods. This will lend insight into appropriate treatment options and contribute to quality of life outcomes studies for craniopharyngioma. PMID:24577430

  9. Results of radiotherapy in craniopharyngiomas analysed by the linear quadratic model

    Energy Technology Data Exchange (ETDEWEB)

    Guerkaynak, M. [Dept. of Radiation Oncology, Hacettepe Univ., Ankara (Turkey); Oezyar, E. [Dept. of Radiation Oncology, Hacettepe Univ., Ankara (Turkey); Zorlu, F. [Dept. of Radiation Oncology, Hacettepe Univ., Ankara (Turkey); Akyol, F.H. [Dept. of Radiation Oncology, Hacettepe Univ., Ankara (Turkey); Lale Atahan, I. [Dept. of Radiation Oncology, Hacettepe Univ., Ankara (Turkey)

    1994-12-31

    In 23 craniopharyngioma patients treated by limited surgery and external radiotherapy, the results concerning local control were analysed by linear quadratic formula. A biologically effective dose (BED) of 55 Gy, calculated with time factor and an {alpha}/{beta} value of 10 Gy, seemed to be adequate for local control. (orig.).

  10. Childhood Craniopharyngioma with Hypothalamic Obesity - No Long-term Weight Reduction due to Rehabilitation Programs

    NARCIS (Netherlands)

    Sterkenburg, A. S.; Hoffmann, A.; Gebhardt, U.; Waldeck, E.; Springer, S.; Mueller, H. L.

    2014-01-01

    Background: Severe obesity due to hypothalamic involvement has major impact on prognosis in long-term survivors of childhood craniopharyngioma. The long-term effects of rehabilitation efforts on weight development and obesity in these patients are not analyzed up to now. Patients and Methods: 108

  11. Symptomatic Cerebral Vasospasm and Delayed Cerebral Ischemia Following Transsphenoidal Resection of a Craniopharyngioma.

    Science.gov (United States)

    Ricarte, Irapuá Ferreira; Funchal, Bruno F; Miranda Alves, Maramélia A; Gomes, Daniela L; Valiente, Raul A; Carvalho, Flávio A; Silva, Gisele S

    2015-09-01

    Vasospasm has been rarely described as a complication associated with craniopharyngioma surgery. Herein we describe a patient who developed symptomatic vasospasm and delayed cerebral ischemia after transsphenoidal surgery for a craniopharyngioma. A 67-year-old woman became drowsy 2 weeks after a transsphenoidal resection of a craniopharyngioma. A head computed tomography (CT) was unremarkable except for postoperative findings. Electroencephalogram and laboratory studies were within the normal limits. A repeated CT scan 48 hours after the initial symptoms showed bilateral infarcts in the territory of the anterior cerebral arteries (ACA). Transcranial Doppler (TCD) showed increased blood flow velocities in both anterior cerebral arteries (169 cm/second in the left ACA and 145 cm/second in the right ACA) and right middle cerebral artery (164 cm/second) compatible with vasospasm. A CT angiography confirmed the findings. She was treated with induced hypertension and her level of consciousness improved. TCD velocities normalized after 2 weeks. Cerebral vasospasm should be considered in the differential diagnosis of patients with altered neurologic status in the postoperative period following a craniopharyngioma resection. Copyright © 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  12. Ghost cells in pilomatrixoma, craniopharyngioma, and calcifying cystic odontogenic tumor: histological, immunohistochemical, and ultrastructural study.

    Science.gov (United States)

    Rumayor, Alicia; Carlos, Román; Kirsch, Hernán Molina; de Andrade, Bruno A Benevenuto; Romañach, Mario J; de Almeida, Oslei Paes

    2015-04-01

    Pilomatrixoma, craniopharyngioma, and calcifying cystic odontogenic tumor are the main entities presenting ghost cells as an important histological feature, in spite their quite different clinical presentation; it seems that they share a common pathway in the formation of these cells. The aim of this study is to examine and compare the characteristics of ghost and other cells that form these lesions. Forty-three cases including 21 pilomatrixomas, 14 craniopharyngiomas, and eight calcifying cystic odontogenic tumors were evaluated by immunohistochemistry for cytokeratins, CD138, β-catenin, D2-40, Glut-1, FAS, CD10 and also by scanning electron microscopy. The CKs, CD138, β-catenin, Glut-1, FAS, and CD10 were more often expressed by transitional cells of craniopharyngioma and calcifying cystic odontogenic tumor, compared with pilomatrixoma. Basaloid cells of pilomatrixoma showed strong positivity for CD138 and CD10. Differences on expression pattern were identified in transitional and basal cells, as ghost cells were negative for most antibodies used, except by low expression for cytokeratins. By scanning electron microscopy, the morphology of ghost cells were similar in their fibrillar cytoplasm, but their pattern varied from sheets in pilomatrixoma to small clusters in craniopharyngioma and calcifying cystic odontogenic tumor. Mechanisms involved in formation of ghost cells are unknown, but probably they follow different pathways as protein expression in the basal/transitional cells was not uniform in the three tumors studied. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  13. Optic Tract Edema: A Highly Specific Magnetic Resonance Imaging Finding for the Diagnosis of Craniopharyngiomas

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    Hirunpat, S.; Tanomkiat, W.; Sriprung, H.; Chetpaophan, J. [Prince of Songkla Univ., Hat Yai (Thailand). Dept. of Radiology and Epidemiology Unit

    2005-07-01

    Purpose: To clarify the accuracy, sensitivity, and specificity of optic tract edema in the diagnosis of craniopharyngiomas. Material and Methods: Preoperative magnetic resonance images (MRIs) of 49 patients (between May 1996 and March 2003) who had a diagnosis of parasellar masses were blindly reviewed by two radiologists. The spread of edema surrounding the tumor on the coronal TSE T2-weighted images was analyzed. Sensitivity and specificity were calculated based on the numbers in this series and also pooled numbers from previous known reported series. Results: Edema along the optic tracts was detected in 7 of 1 craniopharyngiomas, giving a sensitivity of 63.6% (95% CI{approx_equal}30.8-89.1) for our series and 66.7% (95% CI{approx_equal}47.2-82.7) for the pooled numbers. The specificity was 00% (95% CI{approx_equal}90.7-100.0) for our series and 93.9% (95% CI{approx_equal}87.1-97.7) for the pooled numbers. None of the 28 pituitary macroadenomas, 4 meningiomas, 2 hypothalamic astrocytomas, 2 germinomas, mixed-germ cell tumor and arachnoid cyst in our study showed edema of the optic pathways. Conclusion: Optic tract edema, commonly seen in craniopharyngiomas, is a useful MR finding for distinguishing craniopharyngiomas from other parasellar tumors with considerable sensitivity and high specificity.

  14. Optic Tract Edema: A Highly Specific Magnetic Resonance Imaging Finding for the Diagnosis of Craniopharyngiomas

    International Nuclear Information System (INIS)

    Hirunpat, S.; Tanomkiat, W.; Sriprung, H.; Chetpaophan, J.

    2005-01-01

    Purpose: To clarify the accuracy, sensitivity, and specificity of optic tract edema in the diagnosis of craniopharyngiomas. Material and Methods: Preoperative magnetic resonance images (MRIs) of 49 patients (between May 1996 and March 2003) who had a diagnosis of parasellar masses were blindly reviewed by two radiologists. The spread of edema surrounding the tumor on the coronal TSE T2-weighted images was analyzed. Sensitivity and specificity were calculated based on the numbers in this series and also pooled numbers from previous known reported series. Results: Edema along the optic tracts was detected in 7 of 1 craniopharyngiomas, giving a sensitivity of 63.6% (95% CI≅30.8-89.1) for our series and 66.7% (95% CI≅47.2-82.7) for the pooled numbers. The specificity was 00% (95% CI≅90.7-100.0) for our series and 93.9% (95% CI≅87.1-97.7) for the pooled numbers. None of the 28 pituitary macroadenomas, 4 meningiomas, 2 hypothalamic astrocytomas, 2 germinomas, mixed-germ cell tumor and arachnoid cyst in our study showed edema of the optic pathways. Conclusion: Optic tract edema, commonly seen in craniopharyngiomas, is a useful MR finding for distinguishing craniopharyngiomas from other parasellar tumors with considerable sensitivity and high specificity

  15. Nonalcoholic fatty liver disease and fatigue in long-term survivors of childhood-onset craniopharyngioma

    NARCIS (Netherlands)

    Hoffmann, Anika; Bootsveld, Klaus; Gebhardt, Ursel; Daubenbuchel, Anna M. M.; Sterkenburg, Anthe S.; Muller, Hermann L.

    Objective: Hypothalamic obesity in childhood craniopharyngioma (CP) patients carries a high risk for development of metabolic syndrome. In metabolic syndrome, the development of nonalcoholic fatty liver disease (NAFLD) is known. The aim of this study is to detect the risk for NAFLD in

  16. Childhood Craniopharyngioma with Hypothalamic Obesity - No Long-term Weight Reduction due to Rehabilitation Programs

    NARCIS (Netherlands)

    Sterkenburg, A. S.; Hoffmann, A.; Gebhardt, U.; Waldeck, E.; Springer, S.; Mueller, H. L.

    Background: Severe obesity due to hypothalamic involvement has major impact on prognosis in long-term survivors of childhood craniopharyngioma. The long-term effects of rehabilitation efforts on weight development and obesity in these patients are not analyzed up to now. Patients and Methods: 108

  17. Fractionated stereotactic conformal radiotherapy following conservative surgery in the control of craniopharyngiomas

    International Nuclear Information System (INIS)

    Minniti, Giuseppe; Saran, Frank; Traish, Daphne; Soomal, Rubin; Sardell, Susan; Gonsalves, Adam; Ashley, Susan; Warrington, Jim; Burke, Kevin; Mosleh-Shirazi, Amin; Brada, Michael

    2007-01-01

    Purpose: To describe the technique and results of stereotactically guided conformal radiotherapy (SCRT) in patients with craniopharyngioma after conservative surgery. Methods and materials: Thirty-nine patients with craniopharyngioma aged 3-68 years (median age 18 years) were treated with SCRT between June 1994 and January 2003. All patients were referred for radiotherapy after undergoing one or more surgical procedures. Treatment was delivered in 30-33 daily fractions over 6-6.5 weeks to a total dose of 50 Gy using 6 MV photons. Outcome was assessed prospectively. Results: At a median follow-up of 40 months (range 3-88 months) the 3- and 5-year progression-free survival (PFS) was 97% and 92%, and 3- and 5-year survival 100%. Two patients required further debulking surgery for progressive disease 8 and 41 months after radiotherapy. Twelve patients (30%) had acute clinical deterioration due to cystic enlargement of craniopharyngioma following SCRT and required cyst aspiration. One patient with severe visual impairment prior to radiotherapy had visual deterioration following SCRT. Seven out of 10 patients with a normal pituitary function before SCRT had no endocrine deficits following treatment. Conclusion: SCRT as a high-precision technique of localized RT is suitable for the treatment of incompletely excised craniopharyngioma. The local control, toxicity and survival outcomes are comparable to results reported following conventional external beam RT. Longer follow-up is required to assess long-term efficacy and toxicity, particularly in terms of potential reduction in treatment related late toxicity

  18. History before diagnosis in childhood craniopharyngioma : associations with initial presentation and long-term prognosis

    NARCIS (Netherlands)

    Hoffmann, Anika; Boekhoff, Svenja; Gebhardt, Ursel; Sterkenburg, Anthe S.; Daubenbuechel, Anna M. M.; Eveslage, Maria; Mueller, Hermann L.

    2015-01-01

    Objective: Childhood craniopharyngiomas (CP) are often diagnosed after a long duration of history (DOH). Tumor size, hypothalamic involvement (HI), and obesity are associated with reduced overall survival (OS) and functional capacity (FC). The effect of DOH and specific symptoms in history on

  19. Child dermoid cyst mimicking a craniopharyngioma: the benefit of MRI T2-weighted diffusion sequence.

    Science.gov (United States)

    Amelot, Aymeric; Borha, Alin; Calmon, Raphael; Barbet, Patrick; Puget, Stephanie

    2018-02-01

    Brain dermoid cysts are very rare lesions. Although benign, these cysts may be associated with devastating complications due to mass effect or meningitis. The discovery of completely asymptomatic dermoid cysts in the pediatric population is exceedingly rare. Despite the advances in imaging modalities, it sometimes remains difficult to exclude the differential diagnosis of craniopharyngioma. We describe a 12-year-old boy addressed for suspicion of craniopharyngioma diagnosed by decreased visual acuity, bitemporal hemianopia and a CT scan showing a large hypodense suprasellar lesion with intralesional calcifications. Despite the unusual localization and size of this lesion, the absence of dermal sinus commonly found, and before visualizing a hyperintense mass on MRI-diffusion, the diagnosis of craniopharyngioma was ruled out in favor of a dermoid cyst. Radical excision was performed. In the suprasellar area, craniopharyngioma and dermoid cyst may have very similar radiological aspects: low density masses on CT scan and a hyperintense signal on T1-weighted MRI sequences with a variable signal on T2-weighted sequences. Hitherto, only two cases in literature have described suprasellar dermoid cyst. Their initial diagnosis was facilitated by the presence of a dermal sinus.

  20. Clinical study on microsurgical treatment for craniopharyngioma in a single consecutive institutional series of 335 patients.

    Science.gov (United States)

    Guo, Fuyou; Wang, Guoqing; Suresh, Vigneyshwar; Xu, Dingkang; Zhang, Xiaoyang; Feng, Mengzhao; Wang, Fang; Liu, Xianzhi; Song, Laijun

    2018-04-01

    The optimal management of craniopharyngioma is still controversial. The aim of this study is to explore microsurgical outcomes of craniopharyngioma in 335 cases. Clinical data of 335 consecutive patients with craniopharyngioma between March 2011 and March 2017 were retrospectively analyzed. Gross total resection (GTR) was achieved in 265 cases (79.1%), subtotal resection (STR) was obtained in 70 cases (20.9%). The GTR rate was 81.93% in pediatric group and 78.17% in adult group respectively, no significant difference regarding the GTR rate was found in adult group compared with in pediatric group (p > 0.05). However, there was a noticeable difference in the elevated hypothalamic obesity in children group compared with in adult group after operation (p  0.05). Additionally, there were no statistically significant differences for recurrence-free curves between GTR and STR plus adjuvant radiotherapy (p > 0.05). Present findings demonstrated that tumor recurrence and surgical times contribute to negative total resection for craniopharyngioma. Postoperative precise adjuvant radiotherapy was considered in selected cases if pursuit of GTR was rather dangerous under disadvantageous removal factors. Copyright © 2018 Elsevier B.V. All rights reserved.

  1. Craniopharyngioma: improving outcome by early recognition and treatment of acute complications

    International Nuclear Information System (INIS)

    Rajan, Balakrishnan; Ashley, Sue; Thomas, David G. T.; Marsh, Henry; Britton, Juliet; Brada, Michael

    1997-01-01

    Purpose: To assess the frequency, mode of presentation, treatment, and outcome of acute complications in patients with craniopharyngioma around the time of radiotherapy. Methods and Materials: A review was made of 188 patients with craniopharyngioma treated with conservative surgery and external beam radiotherapy at the Royal Marsden Hospital between 1950 and 1992. Results: Twenty six (14%) (95% confidence interval: 9-19%) patients with craniopharyngioma developed acute deterioration immediately before, during and 2 months after radiotherapy with visual deterioration (19 patients), hydrocephalus (7 patients), and global deficit (7 patients). Cystic enlargement with or without hydrocephalus was the most common cause of deterioration. No patient or disease characteristics were predictive of deterioration on univariate or multivariate analysis. Eighteen patients had surgical intervention at the time of deterioration and survived the immediate period. Six of seven patients who did not have surgical intervention died. All patients who survived the postcomplication period completed the full course of external beam radiotherapy. The 10-year progression-free survival of 162 patients without deterioration was 86%, and of 18 patients with acute deterioration who recovered after surgery, 77%. Conclusion: Patients with craniopharyngioma develop acute deterioration around the time of radiotherapy owing to cystic enlargement and/or hydrocephalus which does not represent tumor progression. Early recognition and appropriate surgical treatment followed by conventional full-dose radiotherapy are associated with good long-term outcome

  2. [Hypopituitarism mode in patients with craniopharyngioma in relation to tumor growth pattern].

    Science.gov (United States)

    Qi, S T; Peng, J X; Pan, J; Fan, J; Zhang, S C; Liu, Y; Bao, Y; Qiu, B H; Wu, X Y

    2018-01-02

    Objective: To investigate the pituitary hormone changes of patients with craniopharyngioma of different growth patterns during perioperative period and follow up time. Methods: Retrospective studies were performed on 212 cases of primary craniopharyngioma patient who received total tumor excision surgery in our hospital from January 2001 to May 2012. The characteristics of pituitary hormone and associated clinical manifestation during preoperative, perioperative and postoperative periods were analyzed according to the QST surgical classification. Results: One hundred and seventy-seven (83.5%) of patients present preoperative hypopituitarism, 36 of them were panhypopituitarism. The hypopituitarism condition was exacerbated during the early stage of post-operation period. The abnormal rates of HPA and HPT during the follow up were 60.1% and 58.3% respectively and hormone replacement treatment was needed for these patients. Craniopharyngioma of different growth patterns showed diversities in the characteristics of hypopituitarism. Conclusion: QST surgical classification was closely associated with the pattern of hypopituitarism, it can help to optimize treatment and prognosis estimation, and could be important criterion for improving the clinical practice of neuroendocrine monitoring, treatment and health education of patients with craniopharyngioma.

  3. Hypothalamic obesity after treatment for craniopharyngioma: the importance of the home environment

    NARCIS (Netherlands)

    Meijneke, Ruud W. H.; Schouten-van Meeteren, Antoinette Y. N.; de Boer, Nienke Y.; van Zundert, Suzanne; van Trotsenburg, Paul A. S.; Stoelinga, Femke; van Santen, Hanneke M.

    2015-01-01

    Abstract Hypothalamic obesity after treatment for craniopharyngioma is a well-recognized, severe problem. Treatment of hypothalamic obesity is difficult and often frustrating for the patient, the parents and the professional care-giver. Because hypothalamic obesity is caused by an underlying medical

  4. Pituitary adenoma, craniopharyngioma, and Rathke cleft cyst involving both intrasellar and suprasellar regions: differentiation using MRI

    International Nuclear Information System (INIS)

    Choi, S.H.; Kwon, B.J.; Na, D.G.; Kim, J.-H.; Han, M.H.; Chang, K.-H.

    2007-01-01

    Aims: To determine the differential magnetic resonance imaging (MRI) features of pituitary adenoma, craniopharyngioma, and Rathke cleft cyst involving both intrasellar and suprasellar regions. Materials and methods: The MRI images of 64 patients with pituitary adenoma (n = 38), craniopharyngioma (n = 13), or Rathke cleft cyst (n = 13) were retrospectively reviewed by three neuroradiologists. The following characteristics were evaluated: shape, volume, extent, component characteristics, signal intensities of solid portions on T2-weighted images, signal intensities of cystic portions on T1-weighted images, and enhancement patterns of solid portions and cyst walls of tumours. Fisher's exact test applied with Bonferroni correction was used for multiple comparison. A flowchart for differential diagnosis was constructed based on statistical analysis of the results. Results: A snowman shape, solid characteristics, and homogeneous enhancement of the solid portion were more common in pituitary adenomas (p < 0.017). A superiorly lobulated shape, third ventricle compression by superior tumour extension, mixed solid and cystic characteristics, and reticular enhancement of the solid portion were more common in craniopharyngiomas (p < 0.017). Finally, an ovoid shape, a small tumour volume, cystic characteristics, and no or thin cyst wall enhancement were more common in Rathke cleft cysts (p < 0.017). The flowchart yielded diagnostic accuracies as follows: 92.1% in pituitary adenoma; 92.3% in craniopharyngioma; 92.3% in Rathke cleft cyst; and 92.2% overall. Conclusion: A combination of MRI findings is helpful in the differential diagnosis of the three tumours involving both intrasellar and suprasellar regions

  5. Distinct Patterns of Primary and Motile Cilia in Rathke’s Cleft Cysts and Craniopharyngioma Subtypes

    Science.gov (United States)

    Coy, Shannon; Du, Ziming; Sheu, Shu-Hsien; Woo, Terri; Rodriguez, Fausto J.; Kieran, Mark W.; Santagata, Sandro

    2017-01-01

    Cilia are highly conserved organelles which serve critical roles in development and physiology. Motile cilia are expressed in a limited range of tissues, where they principally regulate local extracellular fluid dynamics. In contrast, primary cilia are expressed by many vertebrate cell types during interphase, and are intimately involved in the cell cycle and signal transduction. Notably, primary cilia are essential for vertebrate hedgehog pathway activity. Improved detection of motile cilia may assist in the diagnosis of some pathologic entities such as Rathke’s cleft cysts while characterizing primary cilia in neoplastic tissues may implicate cilia-dependent signaling pathways as critical for tumorigenesis. We show that immunohistochemistry for the nuclear transcription factor FOXJ1, a master regulator of motile ciliogenesis, robustly labels the motile ciliated epithelium of Rathke’s cleft cysts. FOXJ1 expression discriminates Rathke’s cleft cysts from entities in the sellar/suprasellar region with overlapping histologic features such as craniopharyngiomas. Co-immunohistochemistry for FOXJ1 and markers that highlight motile cilia such as acetylated tubulin (TUBA4A) and the small GTPase ARL13B further enhance the ability to identify diagnostic epithelial cells. In addition to highlighting motile cilia, ARL13B immunohistochemistry also robustly highlights primary cilia in formalin-fixed paraffin-embedded sections. Primary cilia are present throughout the neoplastic epithelium of adamantinomatous craniopharyngioma, but are limited to basally oriented cells near the fibrovascular stroma in papillary craniopharyngioma. Consistent with this differing pattern of primary ciliation, adamantinomatous craniopharyngiomas express significantly higher levels of SHH, and downstream targets such as PTCH1 and GLI2, compared to papillary craniopharyngiomas. In conclusion, motile ciliated epithelium can be readily identified using immunohistochemistry for FOXJ1, TUBA4A and

  6. A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer.

    Science.gov (United States)

    Fountas, Athanasios; Chai, Shu Teng; Ayuk, John; Gittoes, Neil; Chavda, Swarupsinh; Karavitaki, Niki

    2018-01-01

    Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1) and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH) suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed. Although an extremely rare clinical scenario, craniopharyngioma and acromegaly can co-exist; aetiopathogenic link between these two conditions is unlikely.Meticulous review of unexpected

  7. Radiation therapy rather than prior surgery reduces extent of resection during endonasal endoscopic reoperation for craniopharyngioma.

    Science.gov (United States)

    Younus, Iyan; Forbes, Jonathan A; Ordóñez-Rubiano, Edgar G; Avendano-Pradel, Rafael; La Corte, Emanuele; Anand, Vijay K; Schwartz, Theodore H

    2018-07-01

    Radiation therapy is often advocated for residual or recurrent craniopharyngioma following surgical resection to prevent local recurrence. However, radiation therapy is not always effective and may render tumors more difficult to remove. If this is the case, patients may benefit more from reoperation if gross total resection can be achieved. Nevertheless, there is little data on the impact of radiation on reoperations for craniopharyngioma. In this study, we sought to analyze whether a history of previous radiation therapy (RT) affected extent of resection in patients with recurrent craniopharyngiomas subsequently treated with reoperation via endoscopic endonasal approach (EEA). The authors reviewed a prospectively acquired database of EEA reoperations of craniopharyngiomas over 13 years at Weill Cornell, NewYork-Presbyterian Hospital. All procedures were performed by the senior author. The operations were separated into two groups based on whether the patient had surgery alone (group A) or surgery and RT (group B) prior to recurrence. A total of 24 patients (16 male, 8 female) who underwent surgery for recurrent craniopharyngioma were identified. The average time to recurrence was 7.64 ± 4.34 months (range 3-16 months) for group A and 16.62 ± 12.1 months (range 6-45 months) for group B (p < 0.05). The average tumor size at recurrence was smaller in group A (1.85 ± 0.72 cm; range 0.5-3.2) than group B (2.59 ± 0.91 cm; range 1.5-4.6; p = 0.00017). Gross total resection (GTR) was achieved in 91% (10/11) of patients in group A and 54% (7/13) of patients in group B (p = 0.047). There was a near significant trend for higher average Karnofsky performance status (KPS) score at last follow-up for group A (83 ± 10.6) compared with group B (70 ± 16.3, p = 0.056). While RT for residual or recurrent craniopharyngioma may delay time to recurrence, ability to achieve GTR with additional surgery is reduced. In the case of

  8. Prediction of BRAF mutation status of craniopharyngioma using magnetic resonance imaging features.

    Science.gov (United States)

    Yue, Qi; Yu, Yang; Shi, Zhifeng; Wang, Yongfei; Zhu, Wei; Du, Zunguo; Yao, Zhenwei; Chen, Liang; Mao, Ying

    2017-10-06

    OBJECTIVE Treatment with a BRAF mutation inhibitor might shrink otherwise refractory craniopharyngiomas and is a promising preoperative treatment to facilitate tumor resection. The aim of this study was to investigate the noninvasive diagnosis of BRAF-mutated craniopharyngiomas based on MRI characteristics. METHODS Fifty-two patients with pathologically diagnosed craniopharyngioma were included in this study. Polymerase chain reaction was performed on tumor tissue specimens to detect BRAF and CTNNB1 mutations. MRI manifestations-including tumor location, size, shape, and composition; signal intensity of cysts; enhancement pattern; pituitary stalk morphology; and encasement of the internal carotid artery-were analyzed by 2 neuroradiologists blinded to patient identity and clinical characteristics, including BRAF mutation status. Results were compared between the BRAF-mutated and wild-type (WT) groups. Characteristics that were significantly more prevalent (p < 0.05) in the BRAF-mutated craniopharyngiomas were defined as diagnostic features. The minimum number of diagnostic features needed to make a diagnosis was determined by analyzing the receiver operating characteristic (ROC) curve. RESULTS Eight of the 52 patients had BRAF-mutated craniopharyngiomas, and the remaining 44 had BRAF WT tumors. The clinical characteristics did not differ significantly between the 2 groups. Interobserver agreement for MRI data analysis was relatively reliable, with values of Cohen κ ranging from 0.65 to 0.97 (p < 0.001). A comparison of findings in the 2 patient groups showed that BRAF-mutated craniopharyngiomas tended to be suprasellar (p < 0.001), spherical (p = 0.005), predominantly solid (p = 0.003), and homogeneously enhancing (p < 0.001), and that patients with these tumors tended to have a thickened pituitary stalk (p = 0.014). When at least 3 of these 5 features were present, a tumor might be identified as BRAF mutated with a sensitivity of 1.00 and a specificity of 0

  9. Intracystic interferon-alpha in pediatric craniopharyngioma patients : An international multicenter assessment on behalf of SIOPE and ISPN

    NARCIS (Netherlands)

    Kilday, John-Paul; Caldarelli, Massimo; Massimi, Luca; Chen, Robert Hsin-Hung; Lee, Yi Yen; Liang, Muh-Lii; Parkes, Jeanette; Naiker, Thuran; van Veelen, Marie-Lise; Michiels, Erna; Mallucci, Conor; Pettorini, Benedetta; Meijer, Lisethe; Dorfer, Christian; Czech, Thomas; Diezi, Manuel; Schouten-van Meeteren, Antoinette Y. N.; Holm, Stefan; Gustavsson, Bengt; Benesch, Martin; Mueller, Hermann L.; Hoffmann, Anika; Rutkowski, Stefan; Flitsch, Joerg; Escherich, Gabriele; Grotzer, Michael; Spoudeas, Helen A.; Azquikina, Kristian; Capra, Michael; Jimenez-Guerra, Rolando; MacDonald, Patrick; Johnston, Donna L.; Dvir, Rina; Constantini, Shlomi; Kuo, Meng-Fai; Yang, Shih-Hung; Bartels, Ute

    2017-01-01

    Background: Craniopharyngiomas are frequent hypothalamo-pituitary tumors in children, presenting predominantly as cystic lesions. Morbidity from conventional treatment has focused attention on intracystic drug delivery, hypothesized to cause fewer clinical consequences. However, the efficacy of

  10. A novel paraneoplastic syndrome with acquired lipodystrophy and chronic inflammatory demyelinating polyneuropathy in an adolescent male with craniopharyngioma.

    Science.gov (United States)

    Lockemer, Hillary Elizabeth; Sumpter, Kathryn Maria; Cope-Yokoyama, Sandy; Garg, Abhimanyu

    2018-03-28

    Acquired lipodystrophy, craniopharyngioma and chronic inflammatory demyelinating polyneuropathy (CIDP) are individually rare disorders, and have never before been reported in a single patient. A 15-year-7 month old Caucasian male presented with lower extremity weakness, frequent falls and abnormal fat distribution occurring over the previous 1 year. He was diagnosed with CIDP, craniopharyngioma and acquired lipodystrophy. The patient underwent tumor debulking and cranial irradiation for the craniopharyngioma, and received monthly intravenous immunoglobulin for the CIDP. The patient initially had some resolution of the lipodystrophy phenotype, but subsequently the abnormal fat distribution recurred and the patient developed additional systemic abnormalities, including mild pancytopenia and hepatic fibrosis. Our patient represents a novel association of acquired lipodystrophy, craniopharyngioma, and CIDP, possibly due to an as yet unidentified paraneoplastic autoantibody.

  11. Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN

    NARCIS (Netherlands)

    Kilday, John-Paul; Caldarelli, Massimo; Massimi, Luca; Chen, Robert Hsin-Hung; Lee, Yi Yen; Liang, Muh-Lii; Parkes, Jeanette; Naiker, Thuran; van Veelen, Marie-Lise; Michiels, Erna; Mallucci, Conor; Pettorini, Benedetta; Meijer, Lisethe; Dorfer, Christian; Czech, Thomas; Diezi, Manuel; Schouten-van Meeteren, Antoinette Y. N.; Holm, Stefan; Gustavsson, Bengt; Benesch, Martin; Müller, Hermann L.; Hoffmann, Anika; Rutkowski, Stefan; Flitsch, Joerg; Escherich, Gabriele; Grotzer, Michael; Spoudeas, Helen A.; Azquikina, Kristian; Capra, Michael; Jiménez-Guerra, Rolando; MacDonald, Patrick; Johnston, Donna L.; Dvir, Rina; Constantini, Shlomi; Kuo, Meng-Fai; Yang, Shih-Hung; Bartels, Ute

    2017-01-01

    Background: Craniopharyngiomas are frequent hypothalamo-pituitary tumors in children, presenting predominantly as cystic lesions. Morbidity from conventional treatment has focused attention on intracystic drug delivery, hypothesized to cause fewer clinical consequences. However, the efficacy of

  12. Intralobar pulmonary sequestration: a masquarader in tuberculosis prevalent population

    International Nuclear Information System (INIS)

    Ashraf, A.; Iqbal, M.

    2009-01-01

    Intra pulmonary sequestration is a rare congenital disorder that is characterized by malformation of pulmonary tissue having no connection to normal tracheobronchial tree and pulmonary arteries. This is a case history of 20 years old man initially misdiagnosed as Tuberculosis and later diagnosis of intra-lobar pulmonary sequestration was confirmed. There are few reports of this rare disorder globally and we are reporting the first case of Intrapulmonary Sequestration in Pakistan. (author)

  13. Aberrant membranous expression of β-catenin predicts poor prognosis in patients with craniopharyngioma.

    Science.gov (United States)

    Li, Zongping; Xu, Jianguo; Huang, Siqing; You, Chao

    2015-12-01

    The objective of this study is to investigate β-catenin expression in craniopharyngioma patients and determine its significance in predicting the prognosis of this disease. Fifty craniopharyngioma patients were enrolled in this study. Expression of β-catenin in tumor specimens collected from these patients was examined through immunostaining. In addition, mutation of exon 3 in the β-catenin gene, CTNNB1, was analyzed using polymerase chain reaction, denaturing high-pressure liquid chromatography, and DNA sequencing. Based on these results, we explored the association between membranous β-catenin expression, clinical and pathologic characteristics, and prognoses in these patients. Of all craniopharyngioma specimens, 31 (62.0%) had preserved membranous β-catenin expression, whereas the remaining 19 specimens (38.0%) displayed aberrant expression. Statistical analysis showed a significant correlation between aberrant membranous β-catenin expression and CTNNB1 exon 3 mutation, as well as between aberrant membranous β-catenin expression and the histopathologic type of craniopharyngioma and type of resection in our patient population. Furthermore, aberrant membranous β-catenin expression was found to be associated with poor patient survival. Results of Kaplan-Meier survival analysis and Cox regression analysis further confirmed this finding. In conclusion, our study demonstrated that aberrant membranous β-catenin expression was significantly correlated with poor survival in patients with craniopharyngioma. This raises the possibility for use of aberrant membranous β-catenin expression as an independent risk factor in predicting the prognosis of this disease. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Craniopharyngioma presenting with severe hyponatremia, hyponatremia-induced myopathy, and panhypopituitarism: a case report.

    Science.gov (United States)

    Dilrukshi, M D S A; Sandakumari, G V N; Abeysundara, P K; Chang, T

    2017-02-05

    Craniopharyngiomas are rare intracranial tumors commonly presenting with neurological symptoms. Reports of severe hyponatremia as a presenting manifestation of a craniopharyngioma and hyponatremia-induced myopathy are rare. We report the case of a patient with craniopharyngioma presenting with severe hyponatremia, panhypopituitarism, and hyponatremia-induced myopathy. A 52-year-old Sri Lankan man presented with anorexia, nausea, fatigue, generalized muscle weakness, and cramps for 1 week. The onset of his illness had been preceded by vomiting and diarrhea for 1 day which he attributed to food poisoning. On examination, he had an apathetic disposition with a generalized "sallow complexion." He was not dehydrated. Apart from reduced muscle power (4/5) and hyporeflexia, the neurological examination was normal. His serum sodium was 102 mmol/l; potassium 4.1 mmol/l; chloride 63 mmol/l; plasma osmolality 272 mosm/KgH 2 O; urine osmolality 642 mosm/KgH 2 O; and urine sodium 79 mmol/l. His creatine phosphokinase was 12,400 U/l, lactate dehydrogenase 628 U/l, aspartate aminotransferase 360 U/l, and alanine aminotransferase 64 U/l. His hormone profile revealed panhypopituitarism. An electromyogram showed nonspecific abnormalities while a muscle biopsy did not show any pathology. Magnetic resonance imaging of his brain demonstrated a well-defined craniopharyngioma with suprasellar extension. His pituitary gland was compressed and the pituitary stalk was displaced by the tumor. He had marked improvement in muscle power and rapid reduction of serum creatine phosphokinase levels paralleling the correction of severe hyponatremia, even before the initiation of hormone replacement. This case illustrates the rare presentation of severe hyponatremia and hyponatremia-induced myopathy in patients with craniopharyngioma, awareness of which would facilitate early appropriate investigations and treatment.

  15. Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program

    Science.gov (United States)

    Zacharia, Brad E.; Bruce, Samuel S.; Goldstein, Hannah; Malone, Hani R.; Neugut, Alfred I.; Bruce, Jeffrey N.

    2012-01-01

    Craniopharyngioma is a rare primary central nervous system neoplasm. Our objective was to determine factors associated with incidence, treatment, and survival of craniopharyngiomas in the United States. We used the surveillance, epidemiology and end results program (SEER) database to identify patients who received a diagnosis of craniopharyngioma during 2004–2008. We analyzed clinical and demographic information, including age, race, sex, tumor histology, and treatment. Age-adjusted incidence rates and age, sex, and race-adjusted expected survival rates were calculated. We used Cox proportional hazards models to determine the association between covariates and overall survival. We identified 644 patients with a diagnosis of craniopharyngioma. Black race was associated with an age-adjusted relative risk for craniopharyngioma of 1.26 (95% confidence interval [CI], 0.98–1.59), compared with white race. One- and 3-year survival rates of 91.5% (95% CI, 88.9%–93.5%), and 86.2% (95% CI, 82.7%–89.0%) were observed for the cohort; relative survival rates were 92.1% (95% CI, 89.5%–94.0%) and 87.6% (95% CI, 84.1%–90.4%) for 1- and 3-years, respectively. In the multivariable model, factors associated with prolonged survival included younger age, smaller tumor size, subtotal resection, and radiation therapy. Black race, on the other hand, was associated with worse overall survival in the final model. We demonstrated that >85% of patients survived 3 years after diagnosis and that subtotal resection and radiation therapy were associated with prolonged survival. We also noted a higher incidence rate and worse 1- and 3-year survival rates in the black population. Future investigations should examine these racial disparities and focus on evaluating the efficacy of emerging treatment paradigms. PMID:22735773

  16. Phosphorus-32 intracavitary irradiation of cystic craniopharyngiomas: current technique and long-term results

    International Nuclear Information System (INIS)

    Pollock, Bruce E.; Lunsford, L. Dade; Kondziolka, Douglas; Levine, Geoffrey; Flickinger, John C.

    1995-01-01

    Purpose: The management of patients with craniopharyngiomas is often multifaceted and multidisciplinary. The purpose of this study was to examine the results of phosphorus-32 intracavitary irradiation in the treatment of patients with predominately cystic craniopharyngiomas. Methods and Materials: Thirty patients with cystic craniopharyngiomas underwent phosphorus-32 intracavitary irradiation at our center between 1981 and 1993. The median patient age was 26 years (range, 3-70 years). Thirteen patients had intracavitary irradiation as the primary surgery for their cystic tumors, whereas 17 patients had adjuvant intracavitary irradiation after microsurgical resection, fractionated radiotherapy, or both. Patients in the adjuvant treatment group were more likely to have preoperative anterior pituitary insufficiency (p = 0.008 Fischer exact test) and diabetes insipidus (p = 0.003 Fischer exact test). The median follow-up was 37 months (mean, 46 months, range, 7-116 months). Results: Phosphorus-32 intracavitary irradiation resulted in cyst regression in 28 of 32 treated cysts (88%). Ten patients (33%) have had tumor progression requiring further surgical intervention. Three patients (10%) died: two of tumor progression, and one of unrelated causes. Visual acuity and fields improved or remained stable in 63% of the patients. Fifteen patients had residual anterior pituitary function before intracavitary irradiation and 10 (67%) retained their preoperative endocrine status. New-onset diabetes insipidus occurred in 3 of 17 patients (18%) who had normal posterior pituitary function preoperatively. Fourteen of 20 adult patients (70%) continued to perform at their preoperative functional level; 3 of 5 pediatric patients who were age appropriate at the time of treatment continued to develop normally. No difference was noted between primary and adjuvant treatment patients with respect to cyst control, visual deterioration, or endocrine preservation after phosphorus-32

  17. Endoscopic Transsphenoidal Salvage Surgery for Symptomatic Residual Cystic Craniopharyngioma after Radiotherapy.

    Science.gov (United States)

    Emanuelli, Enzo; Frasson, Giuliana; Cazzador, Diego; Borsetto, Daniele; Denaro, Luca

    2018-04-01

    Objectives  Ideal treatment of craniopharyngiomas is still controversial. Radiotherapy (RT) is considered effective for recurrences or after subtotal tumor removal (STR). About 40 to 50% of patients may experience tumor cyst expansion soon after RT; in these cases, the role of salvage surgery is debated. Design  Operative video. Setting  Tertiary care center. Participants  An 11-year-old boy diagnosed with persistent craniopharyngioma. In 2015, the patient underwent right frontotemporal craniotomy for STR at another center, complicated by panhypopituitarism. Two years later, fractionated 54-Gy RT was performed on growing residual tumor. After 3 months, he was admitted to our hospital due to persistent malaise, vomiting, pulsating headache, and epistaxis. Ophthalmologic evaluation evidenced left homonymous hemianopsia. Results  A contrast-enhanced magnetic resonance imaging (MRI) showed a 27-mm cystic component enlarging from the cranial end of the persistent craniopharyngioma lesion, extending into the third ventricle. Biventricular hydrocephalus and brain midline shift to the right were present. Compared with the early post-RT MRI, the cystic component of the tumor demonstrated growth. The patient underwent external ventricular drainage placement for emergent treatment of hydrocephalus and endoscopic transsphenoidal surgery. After cystic content drainage, the lesion was completely removed with its capsule. A "gasket seal" technique was performed for skull base reconstruction, with autologous fascia lata, septal bone, and mucoperiosteum from inferior turbinate. Histologic examination confirmed the craniopharyngioma diagnosis. Postoperative MRI showed resolution of the hydrocephalus and complete tumor removal. Conclusion  Although shrinkage of cystic components of craniopharyngioma residuals may occur within 5 to 6 months after RT, salvage surgery is indicated in symptomatic patients. The link to the video can be found at https://youtu.be/4x6Qe76bf60 .

  18. A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer

    Directory of Open Access Journals (Sweden)

    Athanasios Fountas

    2018-04-01

    Full Text Available Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1 and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed.

  19. Nutritional and endocrinologic evaluation of patients with craniopharyngioma.

    Science.gov (United States)

    Nogueira, Mônica Cristina; Berbel Júnior, Alfredo Sérgio; Koenigkam-Santos, Marcel; Moreira, Ayrton Custódio; Nonino, Carla Barbosa; de Castro, Margaret

    2015-12-01

    Lesions of hypothalamus or adjacent brain structures by the craniopharyngioma (CP) and/or its treatment, as well as changes in orexigenic and anorexigenic hormones, are possible pathogenic factors for the obesity observed in CP patients. This study assessed anthropometric measurements, food intake, and biochemical markers of CP patients. Weight, height, skinfold thicknesses, circumferences, body composition, food intake evaluation, basal glucose, lipids, insulin, ghrelin, PYY, and HOMA-IR calculation were obtained from CP children (n = 10, 4F, aged 12 ± 4.2yr) and CP adults (n = 27,13F aged 42 ± 13 yr) and from 32 gender and age matched controls. Overweight/obesity was observed in 51.4% of the patients at the diagnosis and increased to 86.5% at the time of the study. Obesity was more frequent in patients with grade 2 hypothalamic involvement. Most anthropometric measurements were similar in patients and controls. Caloric intake was lower in CP adults, without difference between children. Lipid intake was higher in CP patients. Carbohydrate and protein intakes were lower in CP children, with no difference between adult groups. There were no differences in micronutrients intake as well as in insulin and PYY levels, and HOMA-IR between patients and controls. HDL-c was lower in CP adults and ghrelin higher in CP children. There is a high rate of overweight/obesity in CP patients at the diagnosis and throughout the follow-up period. Obesity was associated with degree of hypothalamic involvement but not with caloric intake. Obesity and a lipid rich diet may have contributed to the dyslipidemia observed in CP patients. Copyright © 2015 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.

  20. Predicting parental distress among children newly diagnosed with craniopharyngioma.

    Science.gov (United States)

    Peterson, Rachel K; Ashford, Jason M; Scott, Sarah M; Wang, Fang; Zhang, Hui; Bradley, Julie A; Merchant, Thomas E; Conklin, Heather M

    2018-06-22

    Childhood brain tumor diagnoses are stressful for families. Children diagnosed with craniopharyngioma (Cp) present with particularly challenging medical and cognitive problems due to tumor location and associated biophysiologic comorbidities. This study examined parental distress in a sample of families of patients with Cp treated with proton beam therapy to identify factors for targeting psychological intervention. Prior to (n = 96) and 1 year after (n = 73) proton therapy, parents of children diagnosed with Cp (9.81 ± 4.42 years at baseline; 49% male) completed a self-report measure of distress, the Brief Symptom Inventory (BSI). Children completed cognitive assessment measures at baseline; medical variables were extracted from the study database. At baseline, t-tests revealed parents reported higher levels of distress than normative expectations on Anxiety, Depression, Global Severity, and Positive Symptom Distress BSI scales (P < 0.05). Linear mixed effects models revealed parent report measures of child executive dysfunction and behavioral issues were more predictive of parental distress than patients' cognitive performance or medical status (P < 0.05). Models also revealed a significant reduction only in Anxiety over time (t = -2.19, P < 0.05). Extensive hypothalamic involvement at baseline predicted this reduction (P < 0.05). Parents experience significant distress before their child begins adjuvant therapy for Cp, though parental distress appears largely unrelated to medical complications and more related to parent perceptions of child cognitive difficulties (vs. child performance). Importantly, this may be explained by a negative parent reporting style among distressed parents. Knowledge of socio-emotional functioning in parents related to patient characteristics is important for optimization of psychological intervention. © 2018 Wiley Periodicals, Inc.

  1. Endoscopic Endonasal Transsphenoidal Surgery, A Reliable Method for Treating Primary and Recurrent/Residual Craniopharyngiomas: Nine Years of Experience.

    Science.gov (United States)

    Bal, Ercan; Öge, Kamil; Berker, Mustafa

    2016-10-01

    Craniopharyngioma resection is one of the most challenging surgical procedures. Herein, we describe our extended endoscopic endonasal transsphenoidal surgery (EETS) technique, and the results of 9 years of use on primary and recurrent/residual craniopharyngiomas. This study reviewed 28 EETSs in 25 patients with craniopharyngiomas between January 2006 and September 2015. The patients were divided into 2 groups, newly diagnosed patients (group A, n = 15), and patients having residual or recurrent tumors (group B, n = 10). There was no significant difference between the groups in terms of the largest tumor diameter (P = 0.495), and all patients underwent EETS. The clinical and ophthalmologic examinations, imaging studies, endocrinologic studies, and operative findings for these cases were reviewed retrospectively. The number of gross total resections in group A was 13/15, and 7/10 in group B. Three of the patients developed postoperative cerebrospinal fluid leakage (all in group A). There were no neurovascular or ophthalmologic complications, and no meningitis or mortality was observed. There has been a notable increase in the use of EETS in the treatment of craniopharyngiomas during the last decade. Despite its increased use in the treatment of primary craniopharyngiomas, its implementation for recurrent or residual craniopharyngiomas has been viewed with suspicion. In this study, the results have been presented separately for primary and recurrent/residual craniopharyngiomas, so that the results can be compared. Overall, EETS is a reliable and successful surgical treatment method for primary and recurrent/residual craniopharyngiomas. Copyright © 2016 Elsevier Inc. All rights reserved.

  2. The metabolic syndrome and its components in 178 patients treated for craniopharyngioma after 16 years of follow-up.

    Science.gov (United States)

    Wijnen, Mark; Olsson, Daniel S; van den Heuvel-Eibrink, Marry M; Hammarstrand, Casper; Janssen, Joseph A M J L; van der Lely, Aart-Jan; Johannsson, Gudmundur; Neggers, Sebastian J C M M

    2018-01-01

    Patients with craniopharyngioma are at an increased risk for cardio- and cerebrovascular mortality. The metabolic syndrome (MetS) is an important cardiometabolic risk factor, but barely studied in patients with craniopharyngioma. We aimed to investigate the prevalence of and risk factors for the MetS and its components in patients with craniopharyngioma. Cross-sectional study with retrospective data. We studied the prevalence of and risk factors for the MetS and its components in 110 Dutch (median age 47 years, range 18-92) and 68 Swedish (median age 50 years, range 20-81) patients with craniopharyngioma with ≥3 years of follow-up (90 females (51%); 83 patients with childhood-onset craniopharyngioma (47%); median follow-up after craniopharyngioma diagnosis 16 years (range 3-62)). In Dutch patients aged 30-70 years and Swedish patients aged 45-69 years, we examined the prevalence of the MetS and its components relative to the general population. Sixty-nine (46%) of 149 patients with complete data demonstrated the MetS. Prevalence of the MetS was significantly higher in patients with craniopharyngioma compared with the general population (40% vs 26% ( P  < 0.05) for Dutch patients; 52% vs 15% ( P  < 0.05) for Swedish patients). Multivariable logistic regression analysis identified visual impairment as a borderline significant predictor of the MetS (OR 2.54, 95% CI 0.95-6.81; P  = 0.06) after adjustment for glucocorticoid replacement therapy and follow-up duration. Age, female sex, tumor location, radiological hypothalamic damage, 90 Yttrium brachytherapy, glucocorticoid replacement therapy and follow-up duration significantly predicted components of the MetS. Patients with craniopharyngioma are at an increased risk for the MetS, especially patients with visual impairment. © 2018 European Society of Endocrinology.

  3. De novo adamantinomatous craniopharyngioma presenting anew in an elderly patient with previous normal CT and MRI studies: A case report and implications on pathogenesis

    Directory of Open Access Journals (Sweden)

    Amy Walker, B.S.

    2015-09-01

    Full Text Available Adamantinomatous craniopharyngiomas are histologically benign epithelial tumors which arise from embryonic remnants of the craniopharyngeal duct and Rathke’s pouch. They are thought to have a congenital origin and are histologically unique from papillary craniopharyngioma. We describe the case of an elderly male who presented with symptoms related to a large craniopharyngioma with previously normal brain magnetic resonance and computed tomography imaging studies. These findings dispute the embryogenic theory that craniopharyngiomas observed in adults develop from the persistent slow growth of embryonic remnants.

  4. Czaszkogardlak – rzadki łagodny guz nowotworowy - opis przypadku = Craniopharyngioma – rare benign tumor – case report

    Directory of Open Access Journals (Sweden)

    Kamila Woźniak

    2016-08-01

    • 2.        Katedra i Klinika Rehabilitacji, Collegium Medicum im. Ludwika Rydygiera w Bydgoszczy, Uniwersytet Mikołaja Kopernika w Toruniu     Streszczenie Czaszkogardlak (guz Erdheima, łac. craniopharyngioma to stosunkowo rzadki, łagodny guz nowotworowy o lokalizacji wewnątrzczaszkowej, wywodzący się z pozostałości kieszonki Rathkego. Nazwę craniopharyngioma wprowadził Harvey Cushing w 1932. Jedne z pierwszych prób operacyjnego leczenia guza podjął Ludvig Puusepp w 1923 roku. Objawy kliniczne zależą od lokalizacji guza. Są to przede wszystkim objawy wynikające z ucisku masy guza na sąsiadujące struktury anatomiczne. Autorzy przedstawiają opis przypadku klinicznego, leczonego operacyjnie w Klinice Neurochirurgii, Neurotraumatologii i Neurochirurgii Dziecięcej Szpitala Uniwersyteckiego nr 1 im. dr A. Jurasza w Bydgoszczy z rozpoznanym w badaniu histopatologicznym craniopharyngioma.   Słowa kluczowe: czaszkogardlak, craniopharyngioma, guz Erdheima, nowotwór łagodny.     Abstract Craniopharyngioma (Erdheima tumor is a relatively rare benign tumor of intracranial location, derived from the remnants of Rathke's pouch. The name craniopharyngioma introduced Harvey Cushing in 1932. One of the first attempts at surgical treatment of the tumor took Ludvig Puusepp in 1923. Clinical symptoms depend on the site of a tumor. These are primarily the symptoms resulting from the comppression of the tumor mass on adjacent anatomical structures. The authors present a clinical case operating treated in the Department of Neurosurgery, Neurotraumatology and Pediatric Neurosurgery, Dr Antoni Jurasz University Hospital No.1 in Bydgoszcz histopathological diagnosed with craniopharyngoma.   Key words: craniopharyngioma, Erdheima tumor, benign tumor.

  5. Long Term Sequelae of Pediatric Craniopharyngioma – Literature Review and 20 Years of Experience

    Science.gov (United States)

    Cohen, Michal; Guger, Sharon; Hamilton, Jill

    2011-01-01

    Craniopharyngioma are rare histologically benign brain tumors that develop in the pituitary–hypothalamic area. They may invade nearby anatomical structures causing significant rates of neurological, neurocognitive, and endocrinological complications including remarkable hypothalamic damage. Information regarding long term implications of the tumors and treatment in the pediatric population is accumulating, and treatment goals appear to be changing accordingly. In this review we aim to present data regarding long term complications of craniopharyngioma in children and adolescents and our experience from a large tertiary center. Hypothalamic dysfunction was noted to be the most significant complication, adversely affecting quality of life in survivors. Obesity, fatigue, and sleep disorders are the most notable manifestations of this dysfunction, and treatment is extremely difficult. Changes in management in recent years show a potential for improved long term outcomes; we found a trend toward less aggressive surgical management and increasing use of adjuvant treatment, accompanied by a decrease in complication rates. PMID:22645511

  6. Intracystic interferon-α treatment leads to neurotoxicity in craniopharyngioma: case report.

    Science.gov (United States)

    Sharma, Julia; Bonfield, Christopher M; Singhal, Ash; Hukin, Juliette; Steinbok, Paul

    2015-09-01

    Craniopharyngioma is a benign, cystic suprasellar tumor that can be treated with intracystic chemotherapy. Interferon-α (IFN-α) has been gaining popularity as an intracystic treatment for craniopharyngioma because of its efficacy and supposed benign neurotoxicity profile. In this case report the authors describe a patient who, while receiving intracystic IFN-α, suffered a neurological event, which was believed to be related to drug leakage outside the cyst. This is the first report of a focal neurological deficit potentially attributable to intracystic IFN-α therapy, highlighting the fact that IFN-α may have neurotoxic effects on the central nervous system. Given this case and the results of a literature review, the authors suggest that a positive leak test is a relative contraindication to intracystic IFN-α treatment.

  7. Intracavitary Irradiation as a Safe Alternative for Cystic Craniopharyngiomas: Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Alejandro Enriquez-Marulanda

    2016-01-01

    Full Text Available Craniopharyngioma treatment remains a challenge for clinicians and patients. There are many treatment alternatives; however one of them (intracavitary irradiation seeks to control this type of benign brain tumor using minimally invasive techniques, with the specific aim of avoiding causing significant damage to important structures surrounding the sellar/suprasellar region. We present the case of a 3-year-old patient with a predominantly cystic craniopharyngioma who underwent intracavitary irradiation by stereotactic placement. Using this approach, the patient showed a successful response with remission of headaches and hydrocephalus. A reduction in the size of the cyst was achieved, without deterioration of visual fields, with no hormonal supplementation being needed, and with no evidence of focal neurological signs.

  8. MR and CT findings of craniopharyngioma during and after radiation therapy

    International Nuclear Information System (INIS)

    Hamamoto, Y.; Niino, K.; Adachi, M.; Hosoya, T.

    2002-01-01

    Long-term changes in craniopharyngioma treated with radiation therapy (RT) were investigated by computed tomography (CT) and/or magnetic resonance (MR) imaging. Eight patients with craniopharyngioma were treated with incomplete resection or conservative surgical intervention followed by postoperative RT. The periods of tumor shrinkage were often long and varied (range: 6-68 months, mean: 29.1 months). Temporary enlargement of the solid component of a tumor usually occurs during RT and does not represent tumor progression. Cystic enlargement also occurs sometimes comparatively early after RT, and enlarged cysts often shrink with no treatment or with conservative treatment. These changes should be differentiated from tumor recurrence, with careful follow-up. After shrinkage, small solid or cystic nodules enhanced with contrast medium often remain. Long-term follow-up is necessary to differentiate uncontrolled tumors from controlled tumors with imaging modalities. (orig.)

  9. MR and CT findings of craniopharyngioma during and after radiation therapy

    Energy Technology Data Exchange (ETDEWEB)

    Hamamoto, Y.; Niino, K.; Adachi, M.; Hosoya, T. [Department of Radiology, Yamagata University School of Medicine, Iidanishi, Yamagata (Japan)

    2002-02-01

    Long-term changes in craniopharyngioma treated with radiation therapy (RT) were investigated by computed tomography (CT) and/or magnetic resonance (MR) imaging. Eight patients with craniopharyngioma were treated with incomplete resection or conservative surgical intervention followed by postoperative RT. The periods of tumor shrinkage were often long and varied (range: 6-68 months, mean: 29.1 months). Temporary enlargement of the solid component of a tumor usually occurs during RT and does not represent tumor progression. Cystic enlargement also occurs sometimes comparatively early after RT, and enlarged cysts often shrink with no treatment or with conservative treatment. These changes should be differentiated from tumor recurrence, with careful follow-up. After shrinkage, small solid or cystic nodules enhanced with contrast medium often remain. Long-term follow-up is necessary to differentiate uncontrolled tumors from controlled tumors with imaging modalities. (orig.)

  10. Neurological and psychophysiological sequelae following different treatments of craniopharyngioma in children

    International Nuclear Information System (INIS)

    Cavazzuti, V.; Fischer, E.G.; Welch, K.; Belli, J.A.; Winston, K.R.

    1983-01-01

    The authors present neurological findings and data from psychophysiological tests administered during the follow-up period of 35 patients with craniopharyngioma. Group I patients who were treated by irradiation alone or by radiotherapy and conservative surgical procedures (including biopsy, cyst aspiration, and shunting), showed significantly less frontal lobe and visual perceptual dysfunction than Group II patients, in whom radical tumor resection was attempted by a subfrontal exposure. Frontal lobe dysfunction was demonstrated in sorting tests by perseverative responses, inflexibility of behavior, and lack of inhibitory control, while intelligence quotients remained relatively unaffected. Immediate memory defects and decreased manual dexterity were present, to different degrees, in both treatment groups. On the basis of these preliminary data and a maximum follow-up period of 10 years, the authors conclude that primary irradiation of a craniopharyngioma appears associated with a lower morbidity rate and may avoid the frontal lobe disorders seen in the patients with extensive tumor resection

  11. Long term sequelae of pediatric craniopharyngioma - literature review and 20 years of experience

    Directory of Open Access Journals (Sweden)

    Michal eCohen

    2011-11-01

    Full Text Available Craniopharyngioma are rare histologically benign brain tumors that develop in the pituitary- hypothalamic area. They may invade nearby anatomical structures causing significant rates of neurological, neurocognitive and endocrinological complications including remarkable hypothalamic damage. Information regarding long term implications of the tumors and treatment in the pediatric population is accumulating, and treatment goals appear to be changing accordingly. In this review we aim to present data regarding long term complications of craniopharyngioma in children and adolescents and our experience from a large tertiary center. Hypothalamic dysfunction was noted to be the most significant complication, adversely affecting quality of life in survivors. Obesity, fatigue and sleep disorders are the most notable manifestations of this dysfunction, and treatment is extremely difficult. Changes in management in recent years show a potential for improved long term outcomes; we found a trend towards less aggressive surgical management and increasing use of adjuvant treatment, accompanied by a decrease in complication rates.

  12. Craniopharyngioma causing bilateral vision loss 4 months after unremarkable magnetic resonance imaging of the brain

    Directory of Open Access Journals (Sweden)

    Rainy Betts

    2015-01-01

    Full Text Available A 65-year-old man developed bilateral vision loss 4 months after magnetic resonance imaging demonstrated no lesion in the vicinity of the optic chiasm, hypothalamus, and suprasellar tissues. Repeat computed tomography 3 months later showed a predominantly cystic mass of the suprasellar cistern with extension into the anterior third ventricle, which histologically was a craniopharyngioma. The clinical course of this case fuels the controversy whether craniopharyngiomas arise from embryonic rests or can be acquired. From a clinical perspective, it raises questions about when to obtain imaging studies dedicated to the chiasm and the appropriate interval in which a scan should be repeated to exclude structural causes of bilateral vision loss.

  13. Craniopharyngioma in a patient with acromegaly due to a pituitary macroadenoma

    Science.gov (United States)

    El-Bilbeisi, Hazem; Ghannam, Mohammad; Nimri, Caramella F.; Ahmad, Azmi T.

    2010-01-01

    We present the first reported case of a craniopharyngioma as a second primary tumor in a patient with acromegaly due to a growth hormone (GH)-secreting pituitary adenoma. The patient was lost for follow-up for 18 years after trans-sphenoidal pituitary surgery for a GH-secreting pituitary adenoma. She presented with headaches and decreased visual acuity, and showed unsuppressed GH in an oral glucose load test with high IGF-1 levels. Brain MRI showed a suprasellar cystic mass and the patient underwent surgery for cyst drainage resulting in postoperative improvement in her vision. Biopsy of the mass confirmed the diagnosis of a craniopharyngioma. We stress the need for close follow-up of patients with acromegaly with adequate control of GH and IGF-1 levels. PMID:20864785

  14. Gastric bypass surgery for treatment of hypothalamic obesity after craniopharyngioma therapy.

    Science.gov (United States)

    Inge, Thomas H; Pfluger, Paul; Zeller, Meg; Rose, Susan R; Burget, Lukas; Sundararajan, Sumana; Daniels, Stephen R; Tschöp, Matthias H

    2007-08-01

    A 14-year-old boy presented with daytime somnolence, intermittent emesis and hypothyroidism. Neuroimaging revealed a calcified suprasellar intracranial mass, suspected to be a craniopharyngioma. Subtotal resection of the tumor confirmed the diagnosis. Extreme obesity (BMI >60 kg/m(2)) and hyperinsulinemia followed tumor resection and cranial irradiation. Dietary interventions were unsuccessful, and pharmacologic intervention (i.e. octreotide) only slowed the rate of weight gain. Radiography documented the suprasellar mass. Following surgical resection and radiotherapy, hypothalamic-pituitary deficiencies were found. Preprandial and postprandial excursions of insulin, active ghrelin and leptin were measured before and after gastric bypass surgery. Panhypopituitarism, hypothalamic obesity and hyperinsulinemia following craniopharyngioma therapy. Severe caloric restriction, octreotide, and pituitary hormone replacement did not produce weight loss. Gastric bypass surgery led to reduced food cravings, significant weight loss, and amelioration of obesity-related comorbidities. Correction of fasting hyperinsulinemia, normalization of postprandial insulin responses, and reductions in active ghrelin and leptin concentrations were also observed.

  15. Visual outcome, endocrine function and tumor control after fractionated stereotactic radiation therapy of craniopharyngiomas in adults

    DEFF Research Database (Denmark)

    Astradsson, Arnar; Munck Af Rosenschöld, Per; Feldt-Rasmussen, Ulla

    2017-01-01

    BACKGROUND: The purpose of this study was to examine visual outcome, endocrine function and tumor control in a prospective cohort of craniopharyngioma patients, treated with fractionated stereotactic radiation therapy (FSRT). MATERIAL AND METHODS: Sixteen adult patients with craniopharyngiomas were...... eligible for analysis. They were treated with linear accelerator-based FSRT during 1999-2015. In all cases, diagnosis was confirmed by histological analysis. The prescription dose to the tumor was 54 Gy (median, range 48-54) in 1.8 or 2.0 Gy per fraction, and the maximum radiation dose to the optic nerves.......7-13.1) for visual outcome, endocrine function, and tumor control, respectively. RESULTS: Visual acuity impairment was present in 10 patients (62.5%) and visual field defects were present in 12 patients (75%) before FSRT. One patient developed radiation-induced optic neuropathy at seven years after FSRT. Thirteen...

  16. Hybrid Microscopic-Endoscopic Surgery for Craniopharyngioma in Neurosurgical Suite: Technical Notes.

    Science.gov (United States)

    Ichikawa, Tomotsugu; Otani, Yoshihiro; Ishida, Joji; Fujii, Kentaro; Kurozumi, Kazuhiko; Ono, Shigeki; Date, Isao

    2016-01-01

    The best chance of curing craniopharyngioma is achieved by microsurgical total resection; however, its location adjacent to critical structures hinders complete resection without neurologic deterioration. Unrecognized residual tumor within microscopic blind spots might result in tumor recurrences. To improve outcomes, new techniques are necessary to visualize tissue within these blind spots. We examined the success of hybrid microscopic-endoscopic neurosurgery for craniopharyngioma in a neurosurgical suite. Four children with craniopharyngiomas underwent microscopic resection. When the neurosurgeon was confident that most of the visible tumor was removed but was suspicious of residual tumor within the blind spot, he or she used an integrated endoscope-holder system to inspect and remove any residual tumor. Two ceiling monitors were mounted side by side in front of the surgeon to display both microscopic and endoscopic views and to view both monitors simultaneously. Surgery was performed in all patients via the frontobasal interhemispheric approach. Residual tumors were observed in the sella (2 patients), on the ventral surface of the chiasm and optic nerve (1 patient), and in the third ventricle (1 patient) and were resected to achieve total resection. Postoperatively, visual function was improved in 2 patients and none exhibited deterioration related to the surgery. Simultaneous microscopic and endoscopic observation with the use of dual monitors in a neurosurgical suite was ergonomically optimal for the surgeon to perform microsurgical procedures and to avoid traumatizing surrounding vessels or neural tissues. Hybrid microscopic-endoscopic neurosurgery may contribute to safe, less-invasive, and maximal resection to achieve better prognosis in children with craniopharyngioma. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Longitudinal Investigation of Adaptive Functioning following Conformal Irradiation for Pediatric Craniopharyngioma and Low-Grade Glioma

    Science.gov (United States)

    Netson, Kelli L.; Conklin, Heather M.; Wu, Shengjie; Xiong, Xiaoping; Merchant, Thomas E.

    2013-01-01

    Purpose Children treated for brain tumors with conformal radiation therapy experience preserved cognitive outcomes. Early evidence suggests that adaptive functions or independent living skills may be spared. This longitudinal investigation prospectively examined intellectual and adaptive functioning during the first 5 years following irradiation for childhood craniopharyngioma and low-grade glioma (LGG). The effect of visual impairment on adaptive outcomes was investigated. Methods and Materials Children with craniopharyngioma (n=62) and LGG (n=77) were treated using conformal or intensity-modulated radiation therapy. The median age was 8.05 years (3.21 years –17.64 years) and 8.09 years (2.20 years–19.27 years), respectively. Serial cognitive evaluations including measures of intelligence quotient (IQ) and the Vineland Adaptive Behavior Scales (VABS) were conducted at pre-irradiation baseline, 6 months after treatment, and annually through 5 years. A total of 588 evaluations were completed during the follow-up period. Results Baseline assessment revealed no deficits in IQ and VABS indices for children with craniopharyngioma, with significant (p craniopharyngioma. Children with LGG performed below population norms (p < .05) at baseline on VABS Communication, Daily Living Indices, and the Adaptive Behavior Composite, with significant (p < .05) longitudinal decline limited to VABS Communication. Older age at irradiation was a protective factor against longitudinal decline. Severe visual impairment did not independently correlate with poorer adaptive outcomes for either tumor group. Conclusions There was relative sparing of post-irradiation functional outcomes over time in this sample. Baseline differences in functional abilities prior to the initiation of irradiation suggested that other factors influence functional outcomes above and beyond the effects of irradiation. PMID:23245284

  18. Recovery from anterograde and retrograde amnesia after percutaneous drainage of a cystic craniopharyngioma.

    Science.gov (United States)

    Ignelzi, R J; Squire, L R

    1976-01-01

    A case is reported of a cystic craniopharyngioma involving the floor and walls of the third ventricle. Pronounced anterograde and retrograde amnesia were documented preoperatively by formal testing. Rapid improvement in both new learning capacity and remote memory occurred after percutaneous twist drill drainage of the cystic portion of the tumour. The relevance of these observations to the amnesic syndrome and its neuropathological basis is discussed. Images PMID:1011035

  19. Diagnosis, treatment, clinical course, and prognosis of childhood-onset craniopharyngioma patients.

    Science.gov (United States)

    Müller, Hermann L

    2017-12-01

    For decades gross-total resection was the preferred treatment option in childhood-onset craniopharyngioma, assuming that radical strategies at the time of initial diagnosis and treatment would result in cure. Recent reports on long-term prognosis, novel treatment approaches, and molecular genetics provide new insights into more risk-adapted treatment strategies in order to prevent sequelae such as hypothalamic syndrome. A search for original articles published between 2000 and 2016 was performed in PubMed, Science Citation Index Expanded, EMBASE and Scopus. The search terms used were "craniopharyngioma", "hypothalamus", "pituitary", "obesity", "irradiation", and "neurosurgery". The clinical, neuroradiological and surgical definition of hypothalamic involvement is a fundamental factor related to postoperative poor outcome, progressive obesity and neuropsychological impairment after surgical removal. There is a need to change the previous "gold-standard" objective of a primary radical tumor removal in all cases by the new paradigm of a limited resection plus focused radiotherapy in patients with hypothalamic lesions. Hypothalamic involvement and treatment-related hypothalamic lesions are associated with the highest risk of postoperative sequelae. Three dimensional intensity modulated proton beam radiotherapy has potential advantage of over photon beam methods to focus and limit the radiation effects to optic and hypothalamic structures. Preclinical, in vivo mouse models of craniopharyngioma have potential advantage to investigate molecular pathways deregulated in the tumor and to test the use of specific drugs. As expertise has been shown to have impact on post-treatment morbidity, medical societies should establish criteria of adequate professional expertise for the treatment of craniopharyngioma.

  20. Predictors of visual outcome in patients operated for craniopharyngioma - a Danish national study.

    Science.gov (United States)

    Jacobsen, Mads Forslund; Thomsen, Ann Sofia Skou; Bach-Holm, Daniella; Doroudian, Ghazaleh; Nissen, Kamilla Rothe; Fugleholm, Kåre; Poulsgaard, Lars; Siersma, Volkert; Heegaard, Steffen

    2018-02-01

    Craniopharyngioma often causes visual loss due to the close relation to the anterior visual pathways. This study investigates the incidence and predictors of visual outcomes in patients with craniopharyngioma. Data from sixty-six patients who underwent surgery for craniopharyngioma from 2009 to 2013 in Denmark were reviewed. Primary outcomes were visual acuity (VA) and visual field (VF) defects from pre-and postoperative visits. Secondary outcomes were optic nerve atrophy (OA) and papilledema. Fifty-eight patients were included. The VA of the patients 1-year after surgery improved by -0.16 log(MAR) (95%CI: -0.30 to -0.02; p = 0.0266). Visual field (VF) defects worsened in 17 eyes (30%), remained stable in 21 eyes (37%) and improved in 19 eyes (33%). The presence of papilledema and the absence of OA were significantly correlated with an improvement in VA postoperatively (p = 0.011 and p = 0.011, respectively). Patients undergoing surgery within a week or less after their first ophthalmological examination had a significant improvement in VA (-0.36; 95%CI: -0.62 to -0.09; p = 0.0099). Patients undergoing surgery using a subfrontal approach also showed improvement in VA (p = 0.048). Tumour recurrence had a significantly worse VA outcome (p = 0.0074). Patients show a slight improvement in VA 1-year after operation for craniopharyngioma. The presence of papilledema and early surgical intervention is associated with a significant improvement in VA. Early involvement of a dedicated ophthalmologist is recommended to secure an early detection of a visual decline and potential tumour recurrence. © 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  1. Pituitary adenoma, craniopharyngioma, and Rathke cleft cyst involving both intrasellar and suprasellar regions: differentiation using MRI

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    Choi, S.H. [Department of Radiology, Seoul National University College of Medicine (Korea, Republic of); Kwon, B.J. [Department of Radiology, Seoul National University College of Medicine (Korea, Republic of)]. E-mail: bjkwon@radiol.snu.ac.kr; Na, D.G. [Department of Radiology, Seoul National University College of Medicine (Korea, Republic of); Kim, J.-H. [Department of Radiology, Seoul National University College of Medicine (Korea, Republic of); Han, M.H. [Department of Radiology, Seoul National University College of Medicine (Korea, Republic of); Clinical Research Institute, Seoul National University Hospital (Korea, Republic of); Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul (Korea, Republic of); Chang, K.-H. [Department of Radiology, Seoul National University College of Medicine (Korea, Republic of); Clinical Research Institute, Seoul National University Hospital (Korea, Republic of); Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul (Korea, Republic of)

    2007-05-15

    Aims: To determine the differential magnetic resonance imaging (MRI) features of pituitary adenoma, craniopharyngioma, and Rathke cleft cyst involving both intrasellar and suprasellar regions. Materials and methods: The MRI images of 64 patients with pituitary adenoma (n = 38), craniopharyngioma (n = 13), or Rathke cleft cyst (n = 13) were retrospectively reviewed by three neuroradiologists. The following characteristics were evaluated: shape, volume, extent, component characteristics, signal intensities of solid portions on T2-weighted images, signal intensities of cystic portions on T1-weighted images, and enhancement patterns of solid portions and cyst walls of tumours. Fisher's exact test applied with Bonferroni correction was used for multiple comparison. A flowchart for differential diagnosis was constructed based on statistical analysis of the results. Results: A snowman shape, solid characteristics, and homogeneous enhancement of the solid portion were more common in pituitary adenomas (p < 0.017). A superiorly lobulated shape, third ventricle compression by superior tumour extension, mixed solid and cystic characteristics, and reticular enhancement of the solid portion were more common in craniopharyngiomas (p < 0.017). Finally, an ovoid shape, a small tumour volume, cystic characteristics, and no or thin cyst wall enhancement were more common in Rathke cleft cysts (p < 0.017). The flowchart yielded diagnostic accuracies as follows: 92.1% in pituitary adenoma; 92.3% in craniopharyngioma; 92.3% in Rathke cleft cyst; and 92.2% overall. Conclusion: A combination of MRI findings is helpful in the differential diagnosis of the three tumours involving both intrasellar and suprasellar regions.

  2. Longitudinal Investigation of Adaptive Functioning Following Conformal Irradiation for Pediatric Craniopharyngioma and Low-Grade Glioma

    Energy Technology Data Exchange (ETDEWEB)

    Netson, Kelli L. [Department of Psychiatry and Behavioral Sciences, Kansas University School of Medicine—Wichita, Kansas (United States); Conklin, Heather M. [Department of Psychology, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Wu, Shengjie; Xiong, Xiaoping [Department of Biostatistics, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Merchant, Thomas E., E-mail: thomas.merchant@stjude.org [Division of Radiation Oncology, St Jude Children' s Research Hospital, Memphis, Tennessee (United States)

    2013-04-01

    Purpose: Children treated for brain tumors with conformal radiation therapy experience preserved cognitive outcomes. Early evidence suggests that adaptive functions or independent-living skills may be spared. This longitudinal investigation prospectively examined intellectual and adaptive functioning during the first 5 years following irradiation for childhood craniopharyngioma and low-grade glioma (LGG). The effect of visual impairment on adaptive outcomes was investigated. Methods and Materials: Children with craniopharyngioma (n=62) and LGG (n=77) were treated using conformal or intensity modulated radiation therapy. The median age was 8.05 years (3.21-17.64 years) and 8.09 years (2.20-19.27 years), respectively. Serial cognitive evaluations including measures of intelligence quotient (IQ) and the Vineland Adaptive Behavior Scales (VABS) were conducted at preirradiation baseline, 6 months after treatment, and annually through 5 years. Five hundred eighty-eight evaluations were completed during the follow-up period. Results: Baseline assessment revealed no deficits in IQ and VABS indices for children with craniopharyngioma, with significant (P<.05) longitudinal decline in VABS Communication and Socialization indices. Clinical factors associated with more rapid decline included females and preirradiation chemotherapy (interferon). The only change in VABS Daily Living Skills correlated with IQ change (r=0.34; P=.01) in children with craniopharyngioma. Children with LGG performed below population norms (P<.05) at baseline on VABS Communication, Daily Living Indices, and the Adaptive Behavior Composite, with significant (P<.05) longitudinal decline limited to VABS Communication. Older age at irradiation was a protective factor against longitudinal decline. Severe visual impairment did not independently correlate with poorer adaptive outcomes for either tumor group. Conclusions: There was relative sparing of postirradiation functional outcomes over time in this sample

  3. Assessment of visual function by optical coherence tomography and visual field for craniopharyngioma patients

    Directory of Open Access Journals (Sweden)

    Yang Tang

    2015-09-01

    Full Text Available AIM:To analyze the differences and correlations between ganglion cell complex(GCC, peripapillary retinal nerve fiber layer(pRNFLand mean defect(MD, mean sensitivity(MSof visual field(VFin craniopharyngioma patients, to evaluate the feasibility of optical coherence tomography(OCTin diagnosis of the visual pathway damage of craniopharyngioma patients.METHODS:Ninety-five craniopharyngioma patients treated in Beijing Tiantan Hospital, from September 2014 to April 2015 received the VF test by Octopus 900 automated perimeter with the central 30 degree program and the mean thickness measurements of GCC and pRNFL by RTVue OCT. Spearman rank correlation coefficient(rswas used to assess the correlation between GCC, pRNFL and MD, MS. The changes of VF and optic disc were analyzed. RESULTS: Abnormal pRNFL findings occurred in 53.1%(93/175, which included optic disk edema 3.4%(6/175, atrophic changes of optic nerve 47.4%(83/175and glaucoma-like optic neuropathy 7.4%(13/175. Various visual field defect was 71.4%(125/175. The average thickness of binocular pRNFL(rsOD=-0.411, rsOS=-0.354and GCC(rsOD=-0.400, rsOS=-0.314had correlation with MD(PrsOD=0.412, rsOS=0.342and GCC(rsOD=0.414, rsOS=0.299had correlation with MS(PCONCLUSION: The average thickness of pRNFL and GCC has correlation with VF damage, can evaluate the optic nerve damage of craniopharyngioma patients quantitatively. The thinner the thickness of pRNFL and GCC is, the serious damage of visual function is. During the clinical work, visual field test combined with OCT are helpful to find and assess the damage of visual pathway and prognosis.

  4. Longitudinal Investigation of Adaptive Functioning Following Conformal Irradiation for Pediatric Craniopharyngioma and Low-Grade Glioma

    International Nuclear Information System (INIS)

    Netson, Kelli L.; Conklin, Heather M.; Wu, Shengjie; Xiong, Xiaoping; Merchant, Thomas E.

    2013-01-01

    Purpose: Children treated for brain tumors with conformal radiation therapy experience preserved cognitive outcomes. Early evidence suggests that adaptive functions or independent-living skills may be spared. This longitudinal investigation prospectively examined intellectual and adaptive functioning during the first 5 years following irradiation for childhood craniopharyngioma and low-grade glioma (LGG). The effect of visual impairment on adaptive outcomes was investigated. Methods and Materials: Children with craniopharyngioma (n=62) and LGG (n=77) were treated using conformal or intensity modulated radiation therapy. The median age was 8.05 years (3.21-17.64 years) and 8.09 years (2.20-19.27 years), respectively. Serial cognitive evaluations including measures of intelligence quotient (IQ) and the Vineland Adaptive Behavior Scales (VABS) were conducted at preirradiation baseline, 6 months after treatment, and annually through 5 years. Five hundred eighty-eight evaluations were completed during the follow-up period. Results: Baseline assessment revealed no deficits in IQ and VABS indices for children with craniopharyngioma, with significant (P<.05) longitudinal decline in VABS Communication and Socialization indices. Clinical factors associated with more rapid decline included females and preirradiation chemotherapy (interferon). The only change in VABS Daily Living Skills correlated with IQ change (r=0.34; P=.01) in children with craniopharyngioma. Children with LGG performed below population norms (P<.05) at baseline on VABS Communication, Daily Living Indices, and the Adaptive Behavior Composite, with significant (P<.05) longitudinal decline limited to VABS Communication. Older age at irradiation was a protective factor against longitudinal decline. Severe visual impairment did not independently correlate with poorer adaptive outcomes for either tumor group. Conclusions: There was relative sparing of postirradiation functional outcomes over time in this sample

  5. [Craniopharyngioma and Klinefelter syndrome during the pubertal transition: A diagnostic challenge].

    Science.gov (United States)

    Mocarbel, Yamile; Arébalo de Cross, Graciela; Lebrethon, Marie C; Thiry, Albert; Beckersd, Albert; Valdes-Socin, Hernan

    2017-04-01

    Craniopharyngioma is the most common pituitary tumor in childhood. It can compromise the pubertal development because of its evolution or treatment. Syndrome of Klinefelter is the most common cause of hipergonadotrophic hypogonadism in males. The concomitant presentation of both entities is extremely low (1/109) and the pathophysiological association is questionned. We present the case of a 18-year-old Belgian patient. He had a diagnosis of craniopharyngioma in childhood and he presented with panhypopituitarism after radiotherapy and surgical treatment. At the age of 14, he started pubertal induction with gonadotropin therapy without clinical response. Asociación de craneofaringioma y síndrome de Klinefelter en la transición puberal: un desafío diagnóstico Craniopharyngioma and Klinefelter syndrome during the pubertal transition: A diagnostic challenge A genetic evaluation confirmed a homogeneous 47, XXY karyotype. Failure of exogenous gonadotropin therapy revealed the hidden association of primary and secondary hypogonadism, demonstrating the importance of the followup and a multidisciplinary approach in these patients. Sociedad Argentina de Pediatría.

  6. Craniopharyngioma and hypothalamic injury: latest insights into consequent eating disorders and obesity

    Science.gov (United States)

    Müller, Hermann L.

    2016-01-01

    Purpose of review Hypothalamic alterations, pathological or treatment induced, have major impact on prognosis in craniopharyngioma patients mainly because of consequent hypothalamic obesity. Recent insight in molecular genetics, treatment strategies, risk factors and outcomes associated with hypothalamic obesity provide novel therapeutic perspectives. This review includes relevant publications since 2013. Recent findings Recent findings confirm that alterations in posterior hypothalamic areas because of tumour location and/or treatment-related injuries are associated with severe hypothalamic obesity, reduced overall survival and impaired quality of life in long-term survivors of childhood-onset craniopharyngioma. However, eating disorders are observed because of hypothalamic obesity without clear disease-specific patterns. Treatment options for hypothalamic obesity are very limited. Treatment with invasive, nonreversible bariatric methods such as Roux-en-Y gastric bypass is most efficient in weight reduction, but controversial in the paediatric population because of medical, ethical, and legal considerations. Accordingly, treatment in craniopharyngioma should focus on prevention of (further) hypothalamic injury. Presurgical imaging for grading of hypothalamic involvement should be the basis for hypothalamus-sparing strategies conducted by experienced multidisciplinary teams. Summary Until a nonsurgical therapeutic option for hypothalamic obesity for paediatric patients is found, prevention of hypothalamic injury should be the preferred treatment strategy, conducted exclusively by experienced multidisciplinary teams. PMID:26574645

  7. The ophthalmic natural history of paediatric craniopharyngioma: a long-term review.

    Science.gov (United States)

    Drimtzias, Evangelos; Falzon, Kevin; Picton, Susan; Jeeva, Irfan; Guy, Danielle; Nelson, Olwyn; Simmons, Ian

    2014-12-01

    We present our experience over the long-term of monitoring of visual function in children with craniopharyngioma. Our study involves an analysis of all paediatric patients with craniopharyngioma younger than 16 at the time of diagnosis and represents a series of predominantly sub-totally resected tumours. Visual data, of multiple modality, of the paediatric patients was collected. Twenty patients were surveyed. Poor prognostic indicators of the visual outcome and rate of recurrence were assessed. Severe visual loss and papilledema at the time of diagnosis were more common in children under the age of 6. In our study visual signs, tumour calcification and optic disc atrophy at presentation are predictors of poor visual outcome with the first two applying only in children younger than 6. In contrast with previous reports, preoperative visual field (VF) defects and type of surgery were not documented as prognostic indicators of poor postoperative visual acuity (VA) and VF. Contrary to previous reports calcification at diagnosis, type of surgery and preoperative VF defects were not found to be associated with tumour recurrence. Local recurrence is common. Younger age at presentation is associated with a tendency to recur. Magnetic resonance imaging (MRI) remains the recommended means of follow-up in patients with craniopharyngioma.

  8. Trends in treatment and outcomes of pediatric craniopharyngioma, 1975-2011.

    Science.gov (United States)

    Cohen, Michal; Bartels, Ute; Branson, Helen; Kulkarni, Abhaya V; Hamilton, Jill

    2013-06-01

    Craniopharyngioma tumors and their treatment can lead to significant long-term morbidity due to their proximity to vital structures. The optimal treatment has been debated for many years. We aimed to review the long-term outcomes of children treated for craniopharyngioma in our institution over the past decade and describe trends in treatment and outcomes over the past 3 decades. Charts of children with craniopharyngioma treated and followed at The Hospital for Sick Children between 2001 and 2011 were reviewed. Data regarding findings at diagnosis, treatment, and long-term outcomes were analyzed. Comparison was made with previously published data from our institution. Data from 33 patients are included; mean age at treatment, 10.7 ± 4.8 years. In 18 children (55%), the initial surgical approach was tumor cyst decompression with or without adjuvant therapy, compared with only 0-2% in the preceding decades (P < .01). Diabetes insipidus occurred in 55% of children and panhypopituitarism in 58% compared with 88% (P < .01) and 86% (P < .01), respectively, in the previous 10 years. Overall, there was a 36% reduction in the number of children who developed severe obesity compared with the preceding decade. Body mass index at follow-up was associated with body mass index at diagnosis (P = .004) and tumor resection as an initial treatment approach (P = .028). A shift in surgical treatment approach away from gross total resection has led to improved endocrine outcomes. This may have beneficial implications for quality of life in survivors.

  9. ON THE BENEFITS AND RISKS OF PROTON THERAPY IN PEDIATRIC CRANIOPHARYNGIOMA

    Science.gov (United States)

    Beltran, Chris; Roca, Monica; Merchant, Thomas E.

    2013-01-01

    Purpose Craniopharyngioma is a pediatric brain tumor whose volume is prone to change during radiation therapy. We compared photon- and proton-based irradiation methods to determine the effect of tumor volume change on target coverage and normal tissue irradiation in these patients. Methods and Materials For this retrospective study, we acquired imaging and treatment-planning data from 14 children with craniopharyngioma (mean age, 5.1 years) irradiated with photons (54 Gy) and monitored by weekly magnetic resonance imaging (MRI) examinations during radiation therapy. Photon intensity-modulated radiation therapy (IMRT), double-scatter proton (DSP) therapy, and intensity-modulated proton therapy (IMPT) plans were created for each patient based on his or her pre-irradiation MRI. Target volumes were contoured on each weekly MRI scan for adaptive modeling. The measured differences in conformity index (CI) and normal tissue doses, including functional sub-volumes of the brain, were compared across the planning methods, as was target coverage based on changes in target volumes during treatment. Results CI and normal tissue dose values of IMPT plans were significantly better than those of the IMRT and DSP plans (p craniopharyngioma. IMPT is the most conformal method and spares the most normal tissue; however, it is highly sensitive to target volume changes, whereas the DSP method is not. PMID:21570209

  10. Childhood craniopharyngioma: greater hypothalamic involvement before surgery is associated with higher homeostasis model insulin resistance index

    Science.gov (United States)

    Trivin, Christine; Busiah, Kanetee; Mahlaoui, Nizar; Recasens, Christophe; Souberbielle, Jean-Claude; Zerah, Michel; Sainte-Rose, Christian; Brauner, Raja

    2009-01-01

    Background Obesity seems to be linked to the hypothalamic involvement in craniopharyngioma. We evaluated the pre-surgery relationship between the degree of this involvement on magnetic resonance imaging and insulin resistance, as evaluated by the homeostasis model insulin resistance index (HOMA). As insulin-like growth factor 1, leptin, soluble leptin receptor (sOB-R) and ghrelin may also be involved, we compared their plasma concentrations and their link to weight change. Methods 27 children with craniopharyngioma were classified as either grade 0 (n = 7, no hypothalamic involvement), grade 1 (n = 8, compression without involvement), or grade 2 (n = 12, severe involvement). Results Despite having similar body mass indexes (BMI), the grade 2 patients had higher glucose, insulin and HOMA before surgery than the grade 0 (P = 0.02, craniopharyngioma before surgery seems to determine the degree of insulin resistance, regardless of the BMI. The pre-surgery HOMA values were correlated with the post-surgery weight gain. This suggests that obesity should be prevented by reducing inn secretion in those cases with hypothalamic involvement. PMID:19341477

  11. Risk-adapted, long-term management in childhood-onset craniopharyngioma.

    Science.gov (United States)

    Müller, Hermann L

    2017-04-01

    This report is a review of findings on the diagnosis, treatment, clinical course, follow-up, and prognosis of craniopharyngioma patients with special regard to clinical trials and long-term management. Literature search on Pubmed for paper published after 1994. Craniopharyngiomas are rare, embryonic malformations of the sellar/parasellar region with low histological grade. Clinical manifestations are related to increased intracranial pressure, visual impairment, and hypothalamic/pituitary deficiencies. If the tumor is favorably localized, therapy of choice is complete resection, with care taken to preserve hypothalamic and optic functions. In patients with unfavorable tumor location (i.e. involvement of hypothalamic areas), recommended therapy is limited hypothalamus-sparing surgical strategy followed by irradiation. Irradiation has proven effective in treatment of recurrences and progression. Surgical lesions and/or anatomical involvement of posterior hypothalamic areas can result in serious sequelae, mainly hypothalamic syndrome. It is crucial that craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' by experienced multidisciplinary teams in the context of multicenter trials.

  12. The Posterior Transpetrosal Approach in a Case of Large Retrochiasmatic Craniopharyngioma: Operative Video and Technical Nuances.

    Science.gov (United States)

    Labidi, Moujahed; Watanabe, Kentaro; Loit, Marie-Pier; Hanakita, Shunya; Froelich, Sébastien

    2018-02-01

    Objectives  To discuss the use of the posterior petrosal approach for the resection of a retrochiasmatic craniopharyngioma. Design  Operative video. Results  In this case video, the authors discuss the surgical management of a large craniopharyngioma, presenting with mass effect on the third ventricle and optic apparatus. A first surgical stage, through an endoscopic endonasal transtubercular approach, allowed satisfactory decompression of the optic chiasma and nerves in preparation for adjuvant therapy. However, accelerated growth of the tumor, with renewed visual deficits and mass effect on the hypothalamus and third ventricle, warranted a supplementary resection. A posterior transpetrosal 1 2 (also called "retrolabyrinthine transtentorial") was performed to obtain a better exposure of the tumor and the surrounding anatomy (floor and walls of the third ventricle, perforating vessels, optic nerves, etc.) 3 . Nuances of technique and surgical pearls related to the posterior transpetrosal are discussed and illustrated in this operative video, including the posterior mobilization of the transverse-sigmoid sinuses junction, preservation of the venous anatomy during the tentorial incision, identification and preservation of the floor of the third ventricle during tumor resection, and a careful multilayer closure. Conclusion  Retrochiasmatic craniopharyngiomas are difficult to reach tumors that often require skull base approaches, either endoscopic endonasal or transcranial. The posterior transpetrosal approach is an important part of the surgical armamentarium to safely resect these complex tumors. The link to the video can be found at: https://youtu.be/2MyGLJ_v1kI .

  13. Radiosurgery of craniopharyngiomas. Results of long-term follow-up

    International Nuclear Information System (INIS)

    Kida, Yoshihisa; Hasegawa, Toshinori; Yoshimoto, Masayuki; Koike, Johzi; Kobayashi, Tatsuya

    2007-01-01

    Long-term follow-up results of craniopharyngiomas after radiosurgery are reported. Among 125 cases of craniopharyngioma, long-term follow-up more than 6 months is obtained in 108 cases. Majority of the cases have had surgical excision before radiosurgery. The tumors, 19 mm in mean diameter were treated with the mean maximum dose of 22.1 Gy and with the marginal dose of 11.6 Gy. The final radiological outcomes during 63 months of mean follow-up showed 9 complete responses (CRs), 61 partial responses (PRs), 1 minor response (MR), 19 no changes (NCs) and 18 PGs, indicating the response rate of 65% and the control rate of 83% respectively. Neurological and endocrinological signs were improved in 18%, unchanged in 53% and worsened in 15% of cases, meanwhile 8% of the patients were dead. Small and solid tumors were the best indication for radiosurgery due to excellent tumor control as well as no adverse effects. They may have a good chance for complete remission. In conclusion a sufficient tumor resection with microsurgery is required and subsequent radiosurgery is most adequate for treating craniopharyngiomas. (author)

  14. Craniopharyngiomas - the utility of contrast medium enhancement for MR imaging at 1.5 T

    International Nuclear Information System (INIS)

    Hald, J.K.; Eldevik, O.P.; Brunberg, J.A.; Chandler, W.F.

    1994-01-01

    To evaluate the efficacy of i.v. contrast medium administration in MR imaging at 1.5 T in patients with craniopharyngiomas, MR studies of 10 men and 6 women with pathologically proven craniopharyngiomas were made. The MR images were obtained as 3- to 5-mm-thick coronal (n=13) or axial (n=3) T1-weighted images (T1WI) prior to an following i.v. Gd-DTPA administration. Proton density-(PD) and T2-weighted images (T2WI) were also obtained. Conspicuity of tumor margins, cystic versus solid components, size, location and effect upon adjacent structures were separately characterized in all imaging sequences. In 6 patients contrast medium-enhanced T1WI, PD and T2WI demonstrated cystic tumor components not seen on unenhanced T1WI. There were significant differences (p<0.004) on 2-tailed Student's t-test comparing tumor conspicuity on contrast medium-enhanced T1WI with unenhanced T1WI, PD and T2WI. Optimal tumor delineation on MR imaging of patients with craniopharyngiomas justifies the use of i.v. contrast medium. (orig.)

  15. Recent advances in molecular pathology of craniopharyngioma [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Sarah Larkin

    2017-07-01

    Full Text Available Craniopharyngiomas are rare epithelial tumours arising along the path of the craniopharyngeal duct. Two major histological subtypes have been recognised, the papillary and the adamantinomatous. Craniopharyngiomas remain challenging tumours to manage and are associated with significant morbidities and mortality. Recent advances in the molecular pathology of these neoplasms have identified BRAF mutations in the papillary variant, offering promising options for targeted pharmacological treatment. The involvement of β-catenin and the Wnt pathway in the tumorigenesis of the adamantinomatous subtype has been previously established with the identification of stabilising mutations in exon 3 of CTNNB1. Further understanding of the pathogenesis of this subtype has been facilitated with the use of mouse models and xenograft experiments. It has been proposed that the clusters of cells with upregulated Wnt/β-catenin signalling induce tumour formation in a paracrine manner; the complex interactions occurring between different cell populations need to be further clarified for further expansion of this hypothesis. This review outlines recent key advances in our understanding of the molecular pathology of craniopharyngiomas and discusses some of the challenges that need to be overcome for the development of targeted therapies that will hopefully improve the management and the outcomes of these patients.

  16. Childhood craniopharyngioma: greater hypothalamic involvement before surgery is associated with higher homeostasis model insulin resistance index

    Directory of Open Access Journals (Sweden)

    Sainte-Rose Christian

    2009-04-01

    Full Text Available Abstract Background Obesity seems to be linked to the hypothalamic involvement in craniopharyngioma. We evaluated the pre-surgery relationship between the degree of this involvement on magnetic resonance imaging and insulin resistance, as evaluated by the homeostasis model insulin resistance index (HOMA. As insulin-like growth factor 1, leptin, soluble leptin receptor (sOB-R and ghrelin may also be involved, we compared their plasma concentrations and their link to weight change. Methods 27 children with craniopharyngioma were classified as either grade 0 (n = 7, no hypothalamic involvement, grade 1 (n = 8, compression without involvement, or grade 2 (n = 12, severe involvement. Results Despite having similar body mass indexes (BMI, the grade 2 patients had higher glucose, insulin and HOMA before surgery than the grade 0 (P = 0.02, The data for the whole population before and 6–18 months after surgery showed increases in BMI (P Conclusion The hypothalamic involvement by the craniopharyngioma before surgery seems to determine the degree of insulin resistance, regardless of the BMI. The pre-surgery HOMA values were correlated with the post-surgery weight gain. This suggests that obesity should be prevented by reducing inn secretion in those cases with hypothalamic involvement.

  17. Preliminary Results of Fractionated Stereotactic Radiotherapy After Cyst Drainage for Craniopharyngioma in Adults

    International Nuclear Information System (INIS)

    Kanesaka, Naoto; Mikami, Ryuji; Nakayama, Hidetsugu; Nogi, Sachika; Tajima, Yu; Nakajima, Nobuyuki; Wada, Jun; Miki, Tamotsu; Haraoka, Jou; Okubo, Mitsuru; Sugahara, Shinji; Tokuuye, Koichi

    2012-01-01

    Purpose: To retrospectively evaluate the effectiveness of fractionated stereotactic radiotherapy (FSRT) for craniopharyngioma. Methods and Materials: Between 1999 and 2005, 16 patients with craniopharyngioma were referred to Tokyo Medical University Hospital. They received FSRT alone after histologic confirmation by needle biopsy and underwent cyst drainage via endoscopy. The median prescription dose fraction was 30 Gy in six fractions. All patients except 1 were followed up until December 2009 or death. Results: The median follow-up period was 52 months (range, 4–117 months). Of the 17 patients, 3 experienced recurrence 4 to 71 months after FSRT. The 3-year local control rate was 82.4%. One patient died of thyroid cancer, and the 3-year survival rate was 94.1%. Eight patients had improved visual fields at a median of 2.5 months after FSRT, but hormonal functions did not improve in any patient. Conclusions: FSRT after cyst drainage seems to be safe and effective for patients with craniopharyngiomas, and it may be a safe alternative to surgery.

  18. Proton Therapy for Craniopharyngioma - An Early Report from a Single European Centre.

    Science.gov (United States)

    Ajithkumar, T; Mazhari, A-L; Stickan-Verfürth, M; Kramer, P-H; Fuentes, C-S; Lambert, J; Thomas, H; Müller, H; Fleischhack, G; Timmermann, B

    2018-05-01

    Proton beam therapy (PBT) is being increasingly used for craniopharyngioma. We describe our early outcome of patients treated with PBT. Between August 2013 and July 2016, 18 patients with craniopharyngiomas were treated with 54 Cobalt Gray Equivalent (CGE) in 30 fractions over 6 weeks at our centre. The early outcome of 16 patients included in a registry study was analysed. Radiological response was assessed by RECIST criteria and the disease- and treatment-related toxicities were scored according to the CTCAE 4.0. All patients are alive at a median follow-up of 32.6 months (range 9.2-70.6 months) from initial diagnosis. The median age at PBT was 10.2 years (range 5.4-46.9 years). One patient progressed 8.7 months after PBT and subsequently had complete resection of the tumour. At a median follow-up of 18.4 months after PBT, five patients remained in complete remission, four in partial remission and seven with stable disease. The most common adverse effects during PBT were grade 1 (cutaneous in seven patients and fatigue in six patients). There were no treatment-related grade 3 toxicities. Our early results are encouraging and comparable with the limited literature on PBT for craniopharyngioma. Copyright © 2018. Published by Elsevier Ltd.

  19. Preliminary Results of Fractionated Stereotactic Radiotherapy After Cyst Drainage for Craniopharyngioma in Adults

    Energy Technology Data Exchange (ETDEWEB)

    Kanesaka, Naoto, E-mail: kaneka@tokyo-med.ac.jp [Department of Radiology, Tokyo Medical University Hospital, Tokyo (Japan); Mikami, Ryuji; Nakayama, Hidetsugu; Nogi, Sachika; Tajima, Yu [Department of Radiology, Tokyo Medical University Hospital, Tokyo (Japan); Nakajima, Nobuyuki; Wada, Jun; Miki, Tamotsu; Haraoka, Jou [Department of Neurosurgery, Tokyo Medical University Hospital, Tokyo (Japan); Okubo, Mitsuru [Department of Radiology, Tokyo Medical University Hachioji Medical Center, Tokyo (Japan); Sugahara, Shinji [Department of Radiology, Tokyo Medical University Ibaraki Medical Center, Tokyo (Japan); Tokuuye, Koichi [Department of Radiology, Tokyo Medical University Hospital, Tokyo (Japan)

    2012-03-15

    Purpose: To retrospectively evaluate the effectiveness of fractionated stereotactic radiotherapy (FSRT) for craniopharyngioma. Methods and Materials: Between 1999 and 2005, 16 patients with craniopharyngioma were referred to Tokyo Medical University Hospital. They received FSRT alone after histologic confirmation by needle biopsy and underwent cyst drainage via endoscopy. The median prescription dose fraction was 30 Gy in six fractions. All patients except 1 were followed up until December 2009 or death. Results: The median follow-up period was 52 months (range, 4-117 months). Of the 17 patients, 3 experienced recurrence 4 to 71 months after FSRT. The 3-year local control rate was 82.4%. One patient died of thyroid cancer, and the 3-year survival rate was 94.1%. Eight patients had improved visual fields at a median of 2.5 months after FSRT, but hormonal functions did not improve in any patient. Conclusions: FSRT after cyst drainage seems to be safe and effective for patients with craniopharyngiomas, and it may be a safe alternative to surgery.

  20. The diagnostic value of preoperative inflammatory markers in craniopharyngioma: a multicenter cohort study.

    Science.gov (United States)

    Chen, Ming; Zheng, Shi-Hao; Yang, Min; Chen, Zhi-Hua; Li, Shi-Ting

    2018-05-01

    To compare the different levels of preoperative inflammatory markers in peripheral blood samples between craniopharyngioma (CP) and other sellar region tumors so as to explore their differential diagnostic value. The level of white blood cell (WBC), neutrophil, lymphocyte, monocyte, platelet, albumin, neutrophil lymphocyte ratio (NLR), derived NLR (dNLR), platelet lymphocyte ratio (PLR), monocyte lymphocyte ratio (MLR) and prognostic nutritional index (PNI) were compared between the CP and other sellar region tumors. A receiver operating characteristics (ROC) curve analysis was performed to evaluate the diagnostic significance of the peripheral blood inflammatory markers and their paired combinations for CP including its pathological types. Patients with CP had higher levels of pre-operative WBC, lymphocyte and PNI. The papillary craniopharyngioma (PCP) group had higher neutrophil count and NLR than the adamantinomatous craniopharyngioma (ACP) and healthy control groups whereas the ACP group had higher platelet count and PNI than the PCP and healthy control groups. There were not any significant differences in preoperative inflammatory markers between the primary and recurrent CP groups. The AUC values of WBC, neutrophil, NLR + PLR and dNLR + PLR in PCP were all higher than 0.7. Inflammation seems to be closely correlated with CP's development. The preoperative inflammatory markers including WBC, neutrophil, NLR + PLR and dNLR + PLR may differentially diagnose PCP, pituitary tumor (PT) and Rathke cleft cyst (RCC). In addition, some statistical results in this study indirectly proved previous experimental conclusions and strictly matched CP's biological features.

  1. Long term results after fractionated stereotactic radiotherapy (FSRT) in patients with craniopharyngioma: maximal tumor control with minimal side effects.

    Science.gov (United States)

    Harrabi, Semi B; Adeberg, Sebastian; Welzel, Thomas; Rieken, Stefan; Habermehl, Daniel; Debus, Jürgen; Combs, Stephanie E

    2014-09-16

    There are already numerous reports about high local control rates in patients with craniopharyngioma but there are only few studies with follow up times of more than 10 years. This study is an analysis of long term control, tumor response and side effects after fractionated stereotactic radiotherapy (FSRT) for patients with craniopharyngioma. 55 patients who were treated with FSRT for craniopharyngioma were analyzed. Median age was 37 years (range 6-70 years), among them eight children craniopharyngioma. Overall treatment was tolerated well with almost no severe acute or chronic side effects. One patient developed complete anosmia, another one's initially impaired vision deteriorated further. In 83.6% of the cases with radiological follow up a regression of irradiated tumor residues was monitored, in 7 cases complete response was achieved. 44 patients presented themselves initially with endocrinologic dysfunction none of them showed signs of further deterioration during follow up. No secondary malignancies were observed. Long term results for patients with craniopharyngioma after stereotactic radiotherapy are with respect to low treatment related side effects as well as to local control and overall survival excellent.

  2. Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma.

    Science.gov (United States)

    Hayashi, Yasuhiko; Kita, Daisuke; Fukui, Issei; Sasagawa, Yasuo; Oishi, Masahiro; Okajima, Michiko; Tachibana, Osamu; Nakada, Mitsutoshi

    2016-09-01

    Symptomatic Rathke cleft cysts (RCCs) are rarely detected in neuroradiological screening and are less commonly found in children than in adults. However, when RCCs are observed in children, it is important to carefully distinguish a RCC from a cystic craniopharyngioma (CP) even if surgically treated or conservatively followed up. We conducted a retrospective review of clinical data from 11 patients with symptomatic RCCs whose ages were under 18 years and compared the data with data from 15 age- and sex-matched patients with cystic CP who were treated at our institute. The mean age of the patients with RCCs was 12.2 years (range, 6-18). There were six males and five females. As initial symptoms, nine patients presented with headache, while two each had impaired visual function, diabetes insipidus, and activity loss. The 14 patients with CP suffered from impaired visual function. Magnetic resonance imaging (MRI) mainly showed hyperintensity on T1-weighted images (WIs) and hypointensity on T2-WI in patients with RCC. However, patients with CP had characteristic hyperintensity on T2-WI. The average maximum diameter of the RCCs was 19.0 mm on average (range, 8-33 mm). The RCCs were thus significantly smaller than CPs (34.9 mm; range, 21-54 mm). The RCCs were usually oval or dumbbell-shaped and regular in appearance, while the larger CPs were lobular and irregular. A preoperative endocrinological evaluation revealed insufficiencies in four axes in five patients with RCC. Postoperative endocrinological status improved in three patients, remained unchanged in three, and worsened in one. The gonadotropin axis was damaged in a majority (nine) of the patients with CP preoperatively. Postoperative evaluation revealed deficits in five axes in 14 patients with CP, which is a significantly different trend than observed in patients with RCC. Eight patients underwent surgical procedures (transsphenoidal surgery (TSS) in four, craniotomy in four). Two of these patients

  3. Physiologic Growth Hormone-Replacement Therapy and Craniopharyngioma Recurrence in Pediatric Patients: A Meta-Analysis.

    Science.gov (United States)

    Alotaibi, Nawaf M; Noormohamed, Nadia; Cote, David J; Alharthi, Salman; Doucette, Joanne; Zaidi, Hasan A; Mekary, Rania A; Smith, Timothy R

    2018-01-01

    A systematic review and meta-analysis were conducted to examine the effect of growth hormone-replacement therapy (GHRT) on the recurrence of craniopharyngioma in children. PubMed, Embase, and Cochrane databases were searched through April 2017 for studies that evaluated the effect of GHRT on the recurrence of pediatric craniopharyngioma. Pooled effect estimates were calculated with fixed- and random-effects models. Ten studies (n = 3487 patients) met all inclusion criteria, including 2 retrospective cohorts and 8 case series. Overall, 3436 pediatric patients were treated with GHRT after surgery and 51 were not. Using the fixed effect model, we found that the overall craniopharyngioma recurrence rate was lower among children who were treated by GHRT (10.9%; 95% confidence interval 9.80%-12.1%; I 2  = 89.1%; P for heterogeneity <0.01; n = 10 groups) compared with those who were not (35.2%; 95% confidence interval 23.1%-49.6%; I 2  = 61.7%; P for heterogeneity = 0.11; n = 3); the P value comparing the 2 groups was <0.01. Among patients who were treated with GHRT, subgroup analysis revealed that there was a greater prevalence of craniopharyngioma recurrence among studies conducted outside the United States (P < 0.01), single-center studies (P < 0.01), lower impact factor studies (P = 0.03), or studies with a lower quality rating (P = 0.01). Using the random-effects model, we found that the results were not materially different except for when stratifying by GHRT, impact factor, or study quality; this led to nonsignificant differences. Both Begg's rank correlation test (P = 0.7) and Egger's linear regression test (P = 0.06) indicated no publication bias. This meta-analysis demonstrated a lower recurrence rate of craniopharyngioma among children treated with GHRT than those who were not. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. [Influence of preventive use of vasopressin tannate on diabetes insipidus and serum sodium at the early postoperation of craniopharyngioma].

    Science.gov (United States)

    Xiong, Tao; Wanggou, Siyi; Li, Xuejun; Liu, Qing; Jiang, Xingjun; Peng, Zefeng; Yuan, Xianrui

    2016-10-28

    To explore the influence of preventive use of vasopressin tannate on diabetes insipidus and serum sodium at the early postoperation of craniopharyngioma.
 Methods: The data of 83 patients, who underwent unilateral sub-frontal approach resection of craniopharyngioma between 2010 and 2014 by the same senior neurosurgeon, were retrospectively analyzed. The patients were divided into a vasopressin tannate group (used group) and a control group. The diabetes insipidus and serum sodium changes were compared between the two groups.
 Results: Compared with the control group, the incidence of diabetes insipidus decreased at the early postoperation in the vasopressin tannate group (Pcraniopharyngioma.

  5. Scanning and transmission electron microscopy of a craniopharyngioma: x-ray microanalytical study of the intratumoral mineralized deposits

    Energy Technology Data Exchange (ETDEWEB)

    Vilches, J.; Lopez, A.; Martinez, M.C.; Gomez, J.; Barbera, J.

    This paper discusses the value of scanning electron microscopy (SEM) and x-ray microanalysis in the classification of craniopharyngiomas. This neoplasm shows epithelial nest, cords of cuboid cells, foci of squamous metaplasia, and microcystic degeneration. SEM reveals that the epithelial cysts are lined with elongated cells that possess numerous microvilli and blebs and that some cysts are lined with polyhedral cells. The microvilli are interpreted as characteristic of the fast growing craniopharyngiomas. A microanalytical study of the calcified areas reveals the presence of magnesium, phosphorus, and calcium.

  6. Recurrent craniopharyngioma after conformal radiation in children and the burden of treatment.

    Science.gov (United States)

    Klimo, Paul; Venable, Garrett T; Boop, Frederick A; Merchant, Thomas E

    2015-05-01

    In this paper the authors present their experience treating children with recurrent craniopharyngioma who were initially managed with surgery followed by conformal radiation therapy (CRT). A departmental oncology information system was queried to identify all children (craniopharyngioma between 1998 and 2010 (inclusive) and specifically those who experienced tumor progression. For each patient, the authors recorded the type of recurrence (solid, cystic, or both), the time interval to first progression and each subsequent progression, the associated treatment complications, and disease status at last follow-up evaluation. Among the 97 patients that met criteria for entry into this study, 18 (18.6%) experienced tumor progression (9 cystic, 3 solid, 6 cystic and solid). The median time to first recurrence was 4.62 years (range 1.81-9.11 years). The subgroup included 6 female and 12 male patients with a median age of 7.54 years (range 3.61-13.83 years). Ten patients experienced first progression within 5 years of CRT. The 5- and 10-year treatment-free survival rates for the entire cohort were 89.0% (95% confidence interval [CI] 80.5%-93.9%) and 76.2% (95% CI 64%-85%), respectively. Seven patients had a single episode of progression and 11 had more than 1. The time interval between each subsequent progression was progressively shorter. The 18 patients underwent 38 procedures. The median follow-up duration for this group was 9.32 years (range 4.04-19.0 years). Three patients died, including 1 from perioperative complications. Craniopharyngioma progression after prior irradiation is exceedingly difficult to treat and local control is challenging despite repeated surgical procedures. Given our results, gross-total resection may need to be the surgical goal at the time of first recurrence, if possible. Decompressing new cyst formation alone has a low rate of long-term success.

  7. Learning and Memory Following Conformal Radiation Therapy for Pediatric Craniopharyngioma and Low-Grade Glioma

    Science.gov (United States)

    Pinto, Marcos Di; Conklin, Heather M.; Li, Chenghong; Merchant, Thomas E.

    2012-01-01

    Purpose The primary objective of this study was to examine whether children with low-grade glioma (LGG) or craniopharyngioma had impaired learning and memory after conformal radiation therapy (CRT). A secondary objective was to determine whether children who received chemotherapy before CRT, a treatment often used to delay radiation therapy in younger children with LGG, received any protective benefit with respect to learning. Methods and Materials Learning and memory in 57 children with LGG and 44 children with craniopharyngioma were assessed with the California Verbal Learning Test–Children’s Version and the Visual-Auditory Learning tests. Learning measures were administered before CRT, 6 months later, and then yearly for a total of 5 years. Results No decline in learning scores after CRT was observed when patients were grouped by diagnosis. For children with LGG, chemotherapy before CRT did not provide a protective effect on learning. Multiple regression analyses, which accounted for age and tumor volume and location, found that children treated with chemotherapy before CRT were at greater risk of decline on learning measures than those treated with CRT alone. Variables predictive of learning and memory decline included hydrocephalus, shunt insertion, younger age at time of treatment, female gender, and pre-CRT chemotherapy. Conclusions This study did not reveal any impairment or decline in learning after CRT in over-all aggregate learning scores. However, several important variables were found to have a significant effect on neurocognitive outcome. Specifically, chemotherapy before CRT was predictive of worse outcome on verbal learning in LGG patients. In addition, hydrocephalus and shunt insertion in craniopharyngioma were found to be predictive of worse neurocognitive outcome, suggesting a more aggressive natural history for those patients. PMID:22867897

  8. Trends in treatment and outcomes of pediatric craniopharyngioma, 1975–2011

    Science.gov (United States)

    Cohen, Michal; Bartels, Ute; Branson, Helen; Kulkarni, Abhaya V.; Hamilton, Jill

    2013-01-01

    Background Craniopharyngioma tumors and their treatment can lead to significant long-term morbidity due to their proximity to vital structures. The optimal treatment has been debated for many years. We aimed to review the long-term outcomes of children treated for craniopharyngioma in our institution over the past decade and describe trends in treatment and outcomes over the past 3 decades. Methods Charts of children with craniopharyngioma treated and followed at The Hospital for Sick Children between 2001 and 2011 were reviewed. Data regarding findings at diagnosis, treatment, and long-term outcomes were analyzed. Comparison was made with previously published data from our institution. Results Data from 33 patients are included; mean age at treatment, 10.7 ± 4.8 years. In 18 children (55%), the initial surgical approach was tumor cyst decompression with or without adjuvant therapy, compared with only 0–2% in the preceding decades (P < .01). Diabetes insipidus occurred in 55% of children and panhypopituitarism in 58% compared with 88% (P < .01) and 86% (P < .01), respectively, in the previous 10 years. Overall, there was a 36% reduction in the number of children who developed severe obesity compared with the preceding decade. Body mass index at follow-up was associated with body mass index at diagnosis (P = .004) and tumor resection as an initial treatment approach (P = .028). Conclusions A shift in surgical treatment approach away from gross total resection has led to improved endocrine outcomes. This may have beneficial implications for quality of life in survivors. PMID:23486689

  9. Potential evolution of neurosurgical treatment paradigms for craniopharyngioma based on genomic and transcriptomic characteristics.

    Science.gov (United States)

    Robinson, Leslie C; Santagata, Sandro; Hankinson, Todd C

    2016-12-01

    The recent genomic and transcriptomic characterization of human craniopharyngiomas has provided important insights into the pathogenesis of these tumors and supports that these tumor types are distinct entities. Critically, the insights provided by these data offer the potential for the introduction of novel therapies and surgical treatment paradigms for these tumors, which are associated with high morbidity rates and morbid conditions. Mutations in the CTNNB1 gene are primary drivers of adamantinomatous craniopharyngioma (ACP) and lead to the accumulation of β-catenin protein in a subset of the nuclei within the neoplastic epithelium of these tumors. Dysregulation of epidermal growth factor receptor (EGFR) and of sonic hedgehog (SHH) signaling in ACP suggest that paracrine oncogenic mechanisms may underlie ACP growth and implicate these signaling pathways as potential targets for therapeutic intervention using directed therapies. Recent work shows that ACP cells have primary cilia, further supporting the potential importance of SHH signaling in the pathogenesis of these tumors. While further preclinical data are needed, directed therapies could defer, or replace, the need for radiation therapy and/or allow for less aggressive surgical interventions. Furthermore, the prospect for reliable control of cystic disease without the need for surgery now exists. Studies of papillary craniopharyngioma (PCP) are more clinically advanced than those for ACP. The vast majority of PCPs harbor the BRAF v600e mutation. There are now 2 reports of patients with PCP that had dramatic therapeutic responses to targeted agents. Ongoing clinical and research studies promise to not only advance our understanding of these challenging tumors but to offer new approaches for patient management.

  10. TREM-1 expression in craniopharyngioma and Rathke's cleft cyst: its possible implication for controversial pathology.

    Science.gov (United States)

    Liu, Yi; Wang, Chao-Hu; Li, Dan-Ling; Zhang, Shi-Chao; Peng, Yu-Ping; Peng, Jun-Xiang; Song, Ye; Qi, Song-Tao; Pan, Jun

    2016-08-02

    Whether a mixed type of craniopharyngioma (CP) exists and whether papillary craniopharyngioma (pCP) is on a histopathological continuum with Rathke's cleft cyst (RCC) remain controversial. Herein, we examined the expression and localization of β-catenin, BRAF p.V600E (V600E), and triggering receptor expressed on myeloid cells-1 (TREM-1) in 58 samples including 20 pCPs, 26 adamantinomatous craniopharyngiomas (aCP), and 12 RCCs. Five aCPs were diagnosed with mixed type CPs and the remaining 21 cases were pure aCPs. Four of the 12 RCCs presented with significant squamous epithelium (SE). V600E immunoreactivity was observed in all pCPs in the cytoplasm, but not in the nuclei. aCPs and RCCs, including mixed type CP, did not express V600E. Nuclear β-catenin translocation was detected exclusively in aCPs. TREM-1 was expressed in pCPs. Additionally, TREM-1 expression was detected in the SE of 5 "mixed type" CPs, while it was absent in pure aCPs. TREM-1 was expressed in 4 RCCs with SE, but not in the remaining 8 RCCs. TREM-1 mRNA levels were compared in cultured pCP and aCP cells. TREM-1 mRNA level was significantly (p < 0.001; up to 4.045 fold) higher in pCPs than in aCPs. Western blotting revealed a significantly (p < 0.001; up to 7.19 fold) lower level of TREM-1 expression in aCP cells compared to that in pCP cells. Our findings further supported that RCC and pCP may represent two ends of a morphological spectrum. A variant showing overlapping histological features of aCP and pCP should not be considered as a mixed type.

  11. Patterns of care and treatment outcomes of patients with Craniopharyngioma in the national cancer database.

    Science.gov (United States)

    Rao, Yuan J; Hassanzadeh, Comron; Fischer-Valuck, Benjamin; Chicoine, Michael R; Kim, Albert H; Perkins, Stephanie M; Huang, Jiayi

    2017-03-01

    To investigate the patterns of care and outcomes in patients with craniopharyngioma in the National Cancer Data Base (NCDB). This study included 697 patients (166 pediatric and 531 adult cases) treated for craniopharyngioma between 2004 and 2012 in the NCDB. Adjuvant radiotherapy (RT) was defined if within 6 months of surgery. Limited surgery (LS) was defined as biopsy or subtotal resection. Proportional-hazards models were used to evaluate associations between covariates and overall survival (OS). A time-dependent analysis of RT was performed to account for early deaths after surgery. Median follow-up was 46 months. Overall, 21% of patients received adjuvant RT. Of patients with known surgical extent (n = 195), 71% had LS. Utilization of adjuvant RT increased from 18% in 2004-2007 to 24% in 2008-2012. Patterns of care regarding adjuvant RT or LS were not significantly different between adult and pediatric patients. Tumor size, low comorbidity, and LS were associated with increased utilization of adjuvant RT. The 5-year OS among patients treated with LS, LS+RT, and gross total resection were 75, 85, and 82% (p = 0.02). On multivariate analysis of the 195 patients with known surgical extent, LS+RT was associated with improved OS compared to LS (HR 0.22, 95% CI 0.05-0.99, p = 0.04), but was not significant when early deaths (craniopharyngiomas. Immortal-time bias may confound assessment of OS for adjuvant RT. Prospective studies comparing adjuvant RT versus observation after LS are warranted.

  12. Learning and Memory Following Conformal Radiation Therapy for Pediatric Craniopharyngioma and Low-Grade Glioma

    Energy Technology Data Exchange (ETDEWEB)

    Di Pinto, Marcos [Department of Pediatric Psychology, Children' s Hospital of Orange County, Orange, California (United States); Conklin, Heather M. [Department of Psychology, St. Jude Children' s Research Hospital, Memphis, Tennessee (United States); Li, Chenghong [Department of Biostatistics, St. Jude Children' s Research Hospital, Memphis, Tennessee (United States); Merchant, Thomas E., E-mail: thomas.merchant@stjude.org [Division of Radiation Oncology, St. Jude Children' s Research Hospital, Memphis, Tennessee (United States)

    2012-11-01

    Purpose: The primary objective of this study was to examine whether children with low-grade glioma (LGG) or craniopharyngioma had impaired learning and memory after conformal radiation therapy (CRT). A secondary objective was to determine whether children who received chemotherapy before CRT, a treatment often used to delay radiation therapy in younger children with LGG, received any protective benefit with respect to learning. Methods and Materials: Learning and memory in 57 children with LGG and 44 children with craniopharyngioma were assessed with the California Verbal Learning Test-Children's Version and the Visual-Auditory Learning tests. Learning measures were administered before CRT, 6 months later, and then yearly for a total of 5 years. Results: No decline in learning scores after CRT was observed when patients were grouped by diagnosis. For children with LGG, chemotherapy before CRT did not provide a protective effect on learning. Multiple regression analyses, which accounted for age and tumor volume and location, found that children treated with chemotherapy before CRT were at greater risk of decline on learning measures than those treated with CRT alone. Variables predictive of learning and memory decline included hydrocephalus, shunt insertion, younger age at time of treatment, female gender, and pre-CRT chemotherapy. Conclusions: This study did not reveal any impairment or decline in learning after CRT in overall aggregate learning scores. However, several important variables were found to have a significant effect on neurocognitive outcome. Specifically, chemotherapy before CRT was predictive of worse outcome on verbal learning in LGG patients. In addition, hydrocephalus and shunt insertion in craniopharyngioma were found to be predictive of worse neurocognitive outcome, suggesting a more aggressive natural history for those patients.

  13. Learning and Memory Following Conformal Radiation Therapy for Pediatric Craniopharyngioma and Low-Grade Glioma

    International Nuclear Information System (INIS)

    Di Pinto, Marcos; Conklin, Heather M.; Li, Chenghong; Merchant, Thomas E.

    2012-01-01

    Purpose: The primary objective of this study was to examine whether children with low-grade glioma (LGG) or craniopharyngioma had impaired learning and memory after conformal radiation therapy (CRT). A secondary objective was to determine whether children who received chemotherapy before CRT, a treatment often used to delay radiation therapy in younger children with LGG, received any protective benefit with respect to learning. Methods and Materials: Learning and memory in 57 children with LGG and 44 children with craniopharyngioma were assessed with the California Verbal Learning Test–Children's Version and the Visual-Auditory Learning tests. Learning measures were administered before CRT, 6 months later, and then yearly for a total of 5 years. Results: No decline in learning scores after CRT was observed when patients were grouped by diagnosis. For children with LGG, chemotherapy before CRT did not provide a protective effect on learning. Multiple regression analyses, which accounted for age and tumor volume and location, found that children treated with chemotherapy before CRT were at greater risk of decline on learning measures than those treated with CRT alone. Variables predictive of learning and memory decline included hydrocephalus, shunt insertion, younger age at time of treatment, female gender, and pre-CRT chemotherapy. Conclusions: This study did not reveal any impairment or decline in learning after CRT in overall aggregate learning scores. However, several important variables were found to have a significant effect on neurocognitive outcome. Specifically, chemotherapy before CRT was predictive of worse outcome on verbal learning in LGG patients. In addition, hydrocephalus and shunt insertion in craniopharyngioma were found to be predictive of worse neurocognitive outcome, suggesting a more aggressive natural history for those patients.

  14. Dosimetric effect of target expansion and setup uncertainty during radiation therapy in pediatric craniopharyngioma

    International Nuclear Information System (INIS)

    Beltran, Chris; Naik, Mihir; Merchant, Thomas E.

    2010-01-01

    Purpose: Investigate the effect of tumor change and setup uncertainties on target coverage for pediatric craniopharyngioma during RT. Methods and materials: Fifteen pediatric patients with craniopharyngioma (mean 5.1 years) were included in this study. MRI was performed before and a median of six times during RT to monitor changes in the tumor volume. IMRT plans were created and compared to the CRT plan used for treatment. The role of adaptive therapy based on GTV changes was investigated. Dosimetric effects of interfraction and intrafraction motion were examined. Results: The mean of the maximal change in the GTV was 28.5% [-20.7% to 82.0%]. For the standard margin IMRT plans, the mean D 95 of the base plan on the base target was 53.6 Gy [53.1-54.1]. The mean D 95 of the base plans on the adaptive targets was 52.1 Gy [47.9-54.1]. The D 95 for the adaptive plan on the adaptive target was 53.8 Gy [53.4-54.3]. A linear regression equation of y=-0.12x , r 2 = 0.70, was found for the percent change in D 95 of the PTV (y) vs. the percent change in the GTV (x). Inter and intrafraction motion did not affect the target coverage for standard and reduced margin plans. Conclusions: The GTV of pediatric craniopharyngioma patients change size during therapy and adaptive planning is critical for conformal plans; therefore early and regular surveillance imaging is required.

  15. Multiplexed Immunofluorescence Reveals Potential PD-1/PD-L1 Pathway Vulnerabilities in Craniopharyngioma.

    Science.gov (United States)

    Coy, Shannon; Rashid, Rumana; Lin, Jia-Ren; Du, Ziming; Donson, Andrew M; Hankinson, Todd C; Foreman, Nicholas K; Manley, Peter E; Kieran, Mark W; Reardon, David A; Sorger, Peter K; Santagata, Sandro

    2018-03-02

    Craniopharyngiomas are neoplasms of the sellar/parasellar region that are classified into adamantinomatous (ACP) and papillary (PCP) subtypes. Surgical resection of craniopharyngiomas is challenging, and recurrence is common, frequently leading to profound morbidity. BRAF V600E mutations render PCP susceptible to BRAF/MEK inhibitors, but effective targeted therapies are needed for ACP. We explored the feasibility of targeting the PD-1/PD-L1 immune checkpoint pathway in ACP and PCP. We mapped and quantified PD-L1 and PD-1 expression in ACP and PCP resections using immunohistochemistry, immunofluorescence, and RNA in situ hybridization. We used tissue-based cyclic immunofluorescence (t-CyCIF) to map the spatial distribution of immune cells and characterize cell cycle and signaling pathways in ACP tumor cells which intrinsically express PD-1. All ACP (15±14% of cells, n=23, average±S.D.) and PCP (35±22% of cells, n=18) resections expressed PD-L1. In ACP, PD-L1 was predominantly expressed by tumor cells comprising the cyst-lining. In PCP, PD-L1 was highly-expressed by tumor cells surrounding the stromal fibrovascular cores. ACP also exhibited tumor cell-intrinsic PD-1 expression in whorled epithelial cells with nuclear-localized beta-catenin. These cells exhibited evidence of elevated mTOR and MAPK signaling. Profiling of immune populations in ACP and PCP showed a modest density of CD8+ T-cells. ACP exhibit PD-L1 expression in the tumor cyst-lining and intrinsic PD-1 expression in cells proposed to comprise an oncogenic stem-like population. In PCP, proliferative tumor cells express PD-L1 in a continuous band at the stromal-epithelial interface. Targeting PD-L1 and/or PD-1 in both subtypes of craniopharyngioma might therefore be an effective therapeutic strategy.

  16. Characterization of the murine orthotopic adamantinomatous craniopharyngioma PDX model by MRI in correlation with histology.

    Science.gov (United States)

    Hölsken, Annett; Schwarz, Marc; Gillmann, Clarissa; Pfister, Christina; Uder, Michael; Doerfler, Arnd; Buchfelder, Michael; Schlaffer, Sven; Fahlbusch, Rudolf; Buslei, Rolf; Bäuerle, Tobias

    2018-01-01

    Adamantinomatous craniopharyngiomas (ACP) as benign sellar brain tumors are challenging to treat. In order to develop robust in vivo drug testing methodology, the murine orthotopic craniopharyngioma model (PDX) was characterized by magnetic resonance imaging (MRI) and histology in xenografts from three patients (ACP1-3). In ACP PDX, multiparametric MRI was conducted to assess morphologic characteristics such as contrast-enhancing tumor volume (CETV) as well as functional parameters from dynamic contrast-enhanced MRI (DCE-MRI) and diffusion-weighted imaging (DWI) including area-under-the-curve (AUC), peak enhancement (PE), time-to-peak (TTP) and apparent diffusion coefficient (ADC). These MRI parameters evaluated in 27 ACP PDX were correlated to histological features and percentage of vital tumor cell content. Qualitative analysis of MRI and histology from PDX revealed a similar phenotype as seen in patients, although the MRI appearance in mice resulted in a more solid tumor growth than in humans. CETV were significantly higher in ACP2 xenografts relative to ACP1 and ACP3 which correspond to respective average vitality of 41%, <10% and 26% determined histologically. Importantly, CETV prove tumor growth of ACP2 PDX as it significantly increases in longitudinal follow-up of 110 days. Furthermore, xenografts from ACP2 revealed a significantly higher AUC, PE and TTP in comparison to ACP3, and significantly increased ADC relative to ACP1 and ACP3 respectively. Overall, DCE-MRI and DWI can be used to distinguish vital from non-vital grafts, when using a cut off value of 15% for vital tumor cell content. MRI enables the assessment of craniopharyngioma PDX vitality in vivo as validated histologically.

  17. Endoscopic Endonasal Transplanum Transtuberculum Approach for the Resection of a Large Suprasellar Craniopharyngioma.

    Science.gov (United States)

    Mangussi-Gomes, João; Vellutini, Eduardo A; Truong, Huy Q; Pahl, Felix H; Stamm, Aldo C

    2018-04-01

    Objectives  To demonstrate an endoscopic endonasal transplanum transtuberculum approach for the resection of a large suprasellar craniopharyngioma. Design  Single-case-based operative video. Setting  Tertiary center with dedicated skull base team. Participants  A 72-year-old male patient diagnosed with a suprasellar craniopharyngioma. Main Outcomes Measured  Surgical resection of the tumor and preservation of the normal surrounding neurovascular structures. Results  A 72-year-old male patient presented with a 1-year history of progressive bitemporal visual loss. He also referred symptoms suggestive of hypogonadism. Neurological examination was unremarkable and endocrine workup demonstrated mildly elevated prolactin levels. Magnetic resonance images demonstrated a large solid-cystic suprasellar lesion, consistent with the diagnosis of craniopharyngioma. The lesion was retrochiasmatic, compressed the optic chiasm, and extended into the interpeduncular cistern ( Fig. 1 ). Because of that, the patient underwent an endoscopic endonasal transplanum transtuberculum approach. 1 2 3 The nasal stage consisted of a transnasal transseptal approach, with complete preservation of the patient's left nasal cavity. 4 The cystic component of the tumor was decompressed and its solid part was resected. It was possible to preserve the surrounding normal neurovascular structures ( Fig. 2 ). Skull base reconstruction was performed with a dural substitute, a fascia lata graft, and a right nasoseptal flap ( Video 1 ). The patient did well after surgery and referred complete visual improvement. However, he also presented pan-hypopituitarism on long-term follow-up. Conclusions  The endoscopic endonasal route is a good alternative for the resection of suprasellar lesions. It permits tumor resection and preservation of the surrounding neurovascular structures while avoiding external incisions and brain retraction. The link to the video can be found at: https://youtu.be/zmgxQe8w-JQ .

  18. Effects of Surgery and Proton Therapy on Cerebral White Matter of Craniopharyngioma Patients

    Energy Technology Data Exchange (ETDEWEB)

    Uh, Jinsoo, E-mail: jinsoo.uh@stjude.org [Department of Radiological Sciences, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Merchant, Thomas E. [Department of Radiological Sciences, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Li, Yimei; Li, Xingyu [Department of Biostatistics, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Sabin, Noah D. [Department of Radiological Sciences, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Indelicato, Daniel J. [Department of Radiation Oncology, University of Florida, Jacksonville, Florida (United States); Ogg, Robert J. [Department of Radiological Sciences, St Jude Children' s Research Hospital, Memphis, Tennessee (United States); Boop, Frederick A. [Semmes-Murphey Neurologic and Spine Institute, Memphis, Tennessee (United States); Jane, John A. [Department of Neurosurgery, University of Virginia, Charlottesville, Virginia (United States); Hua, Chiaho [Department of Radiological Sciences, St Jude Children' s Research Hospital, Memphis, Tennessee (United States)

    2015-09-01

    Purpose: The purpose of this study was to determine radiation dose effect on the structural integrity of cerebral white matter in craniopharyngioma patients receiving surgery and proton therapy. Methods and Materials: Fifty-one patients (2.1-19.3 years of age) with craniopharyngioma underwent surgery and proton therapy in a prospective therapeutic trial. Anatomical magnetic resonance images acquired after surgery but before proton therapy were inspected to identify white matter structures intersected by surgical corridors and catheter tracks. Longitudinal diffusion tensor imaging (DTI) was performed to measure microstructural integrity changes in cerebral white matter. Fractional anisotropy (FA) derived from DTI was statistically analyzed for 51 atlas-based white matter structures of the brain to determine radiation dose effect. FA in surgery-affected regions in the corpus callosum was compared to that in its intact counterpart to determine whether surgical defects affect radiation dose effect. Results: Surgical defects were seen most frequently in the corpus callosum because of transcallosal resection of tumors and insertion of ventricular or cyst catheters. Longitudinal DTI data indicated reductions in FA 3 months after therapy, which was followed by a recovery in most white matter structures. A greater FA reduction was correlated with a higher radiation dose in 20 white matter structures, indicating a radiation dose effect. The average FA in the surgery-affected regions before proton therapy was smaller (P=.0001) than that in their non–surgery-affected counterparts with more intensified subsequent reduction of FA (P=.0083) after therapy, suggesting that surgery accentuated the radiation dose effect. Conclusions: DTI data suggest that mild radiation dose effects occur in patients with craniopharyngioma receiving surgery and proton therapy. Surgical defects present at the time of proton therapy appear to accentuate the radiation dose effect longitudinally

  19. Long-Term Outcomes and Complications in Patients With Craniopharyngioma: The British Columbia Cancer Agency Experience

    Energy Technology Data Exchange (ETDEWEB)

    Lo, Andrea C., E-mail: andrealo@gmail.com [Department of Radiation Oncology, British Columbia Cancer Agency Vancouver Centre, Vancouver, British Columbia (Canada); Department of Surgery, University of British Columbia, Vancouver, British Columbia (Canada); Howard, A. Fuchsia [School of Population and Public Health, University of British Columbia, Vancouver, British Columbia (Canada); Nichol, Alan; Sidhu, Keerat; Abdulsatar, Farah [Department of Radiation Oncology, British Columbia Cancer Agency Vancouver Centre, Vancouver, British Columbia (Canada); Department of Surgery, University of British Columbia, Vancouver, British Columbia (Canada); Hasan, Haroon [Department of Radiation Oncology, British Columbia Cancer Agency Vancouver Centre, Vancouver, British Columbia (Canada); Goddard, Karen [Department of Radiation Oncology, British Columbia Cancer Agency Vancouver Centre, Vancouver, British Columbia (Canada); Department of Surgery, University of British Columbia, Vancouver, British Columbia (Canada)

    2014-04-01

    Purpose: We report long-term outcomes and complications of craniopharyngioma patients referred to our institution. Methods and Materials: Between 1971 and 2010, 123 consecutive patients received primary treatment for craniopharyngioma in British Columbia and were referred to our institution. The median age was 30 years (range, 2-80 years). Thirty-nine percent of patients were treated primarily with subtotal resection (STR) and radiation therapy (RT), 28% with STR alone, 15% with gross total resection, 11% with cyst drainage (CD) alone, 5% with CD+RT, and 2% with RT alone. Eight percent of patients received intracystic bleomycin (ICB) therapy. Results: Median follow-up was 8.9 years, and study endpoints were reported at 10 years. Ten-year Kaplan-Meier progression-free survival (PFS) was 46%. Patients treated with STR+RT or CD+RT had the highest PFS (82% and 83%, respectively). There were no significant differences between PFS after adjuvant versus salvage RT (84% vs 74%, respectively; P=.6). Disease-specific survival (DSS) was 88%, and overall survival (OS) was 80%. Primary treatment modality did not affect DSS or OS, while older age was a negative prognostic factor for OS but not DSS. Kaplan-Meier rates for visual deterioration, anterior pituitary hormone deficiency, diabetes insipidus, seizure disorder, and cerebrovascular events (CVE) due to treatment, not tumor progression, were 27%, 76%, 45%, 16%, and 11%, respectively. The CVE rate was 29% in patients who received ICB compared to 10% in those who did not (P=.07). Conclusions: We report favorable PFS in patients with craniopharyngioma, especially in those who received RT after surgery. DSS and OS rates were excellent regardless of primary treatment modality. We observed a high incidence of hypopituitarism, visual deterioration, and seizure disorder. Eleven percent of patients experienced CVEs after treatment. There was a suggestion of increased CVE risk in patients treated with ICB.

  20. External beam irradiation of craniopharyngiomas: long-term analysis of tumor control and morbidity

    International Nuclear Information System (INIS)

    Varlotto, John M.; Flickinger, John C.; Kondziolka, Douglas; Lunsford, L.D.; Deutsch, Melvin

    2002-01-01

    Purpose: To delineate the long-term control and morbidity with external beam radiotherapy (EBRT) of craniopharyngiomas. Methods and Materials: Between 1971 and 1992, 24 craniopharyngioma patients underwent EBRT at the University of Pittsburgh. Most (19 of 24) were treated within 1-3 months after subtotal resection. The other prior surgical procedures were biopsy (n = 2) and gross total resection (n = 1); 2 patients did not undergo any surgical procedure. The median follow-up was 12.1 years. The median patient age was 29 years (range 5-69). The total radiation doses varied from 36 to 70 Gy (median 59.75). The normalized total dose (NTD, biologically equivalent dose given in 2 Gy/fraction [α/β ratio = 2]) varied from 28 to 83 Gy (median 55.35). Results: The actuarial survival rate at 10 and 20 years was 100% and 92.3%, respectively. The actuarial local control rate at 10 and 20 years was 89.1% and 54.0%, respectively. No local failures occurred with doses ≥60 Gy (n=12) or NTDs ≥55 Gy. The complication-free survival rate at 10 and 20 years was 80.1% and 72.1%, respectively. No complications were noted with an NTD of ≤55 Gy. The actuarial survival free from any adverse outcome (recurrence or complication) was 70.1% and 31.8% at 10 and 20 years, respectively. The adverse outcome-free survival appeared optimized (at 73%) with an NTD of 55-63 Gy. Multivariate analysis found that tumor control correlated significantly with the total dose (p=0.02), treatment complications with NTD (p=0.008), and adverse outcome with hypopituitarism on presentation (p=0.03). Conclusion: We recommend treating craniopharyngioma with 1.6-1.7-Gy dose fractions to 60 Gy to optimize outcome from EBRT

  1. Effects of Surgery and Proton Therapy on Cerebral White Matter of Craniopharyngioma Patients

    International Nuclear Information System (INIS)

    Uh, Jinsoo; Merchant, Thomas E.; Li, Yimei; Li, Xingyu; Sabin, Noah D.; Indelicato, Daniel J.; Ogg, Robert J.; Boop, Frederick A.; Jane, John A.; Hua, Chiaho

    2015-01-01

    Purpose: The purpose of this study was to determine radiation dose effect on the structural integrity of cerebral white matter in craniopharyngioma patients receiving surgery and proton therapy. Methods and Materials: Fifty-one patients (2.1-19.3 years of age) with craniopharyngioma underwent surgery and proton therapy in a prospective therapeutic trial. Anatomical magnetic resonance images acquired after surgery but before proton therapy were inspected to identify white matter structures intersected by surgical corridors and catheter tracks. Longitudinal diffusion tensor imaging (DTI) was performed to measure microstructural integrity changes in cerebral white matter. Fractional anisotropy (FA) derived from DTI was statistically analyzed for 51 atlas-based white matter structures of the brain to determine radiation dose effect. FA in surgery-affected regions in the corpus callosum was compared to that in its intact counterpart to determine whether surgical defects affect radiation dose effect. Results: Surgical defects were seen most frequently in the corpus callosum because of transcallosal resection of tumors and insertion of ventricular or cyst catheters. Longitudinal DTI data indicated reductions in FA 3 months after therapy, which was followed by a recovery in most white matter structures. A greater FA reduction was correlated with a higher radiation dose in 20 white matter structures, indicating a radiation dose effect. The average FA in the surgery-affected regions before proton therapy was smaller (P=.0001) than that in their non–surgery-affected counterparts with more intensified subsequent reduction of FA (P=.0083) after therapy, suggesting that surgery accentuated the radiation dose effect. Conclusions: DTI data suggest that mild radiation dose effects occur in patients with craniopharyngioma receiving surgery and proton therapy. Surgical defects present at the time of proton therapy appear to accentuate the radiation dose effect longitudinally

  2. On the Benefits and Risks of Proton Therapy in Pediatric Craniopharyngioma

    International Nuclear Information System (INIS)

    Beltran, Chris; Roca, Monica; Merchant, Thomas E.

    2012-01-01

    Purpose: Craniopharyngioma is a pediatric brain tumor whose volume is prone to change during radiation therapy. We compared photon- and proton-based irradiation methods to determine the effect of tumor volume change on target coverage and normal tissue irradiation in these patients. Methods and Materials: For this retrospective study, we acquired imaging and treatment-planning data from 14 children with craniopharyngioma (mean age, 5.1 years) irradiated with photons (54 Gy) and monitored by weekly magnetic resonance imaging (MRI) examinations during radiation therapy. Photon intensity-modulated radiation therapy (IMRT), double-scatter proton (DSP) therapy, and intensity-modulated proton therapy (IMPT) plans were created for each patient based on his or her pre-irradiation MRI. Target volumes were contoured on each weekly MRI scan for adaptive modeling. The measured differences in conformity index (CI) and normal tissue doses, including functional sub-volumes of the brain, were compared across the planning methods, as was target coverage based on changes in target volumes during treatment. Results: CI and normal tissue dose values of IMPT plans were significantly better than those of the IMRT and DSP plans (p 3 , and the mean increase in PTV was 11.3% over the course of treatment. The dose to 95% of the PTV was correlated with a change in the PTV; the R 2 values for all models, 0.73 (IMRT), 0.38 (DSP), and 0.62 (IMPT), were significant (p < 0.01). Conclusions: Compared with photon IMRT, proton therapy has the potential to significantly reduce whole-brain and -body irradiation in pediatric patients with craniopharyngioma. IMPT is the most conformal method and spares the most normal tissue; however, it is highly sensitive to target volume changes, whereas the DSP method is not.

  3. The supraorbital eyebrow approach for removal of craniopharyngioma in children: a case series.

    Science.gov (United States)

    de Oliveira, Ricardo Santos; Viana, Dinark Conceição; Augusto, Lucas Pires; Santos, Marcelo Volpon; Machado, Hélio Rubens

    2018-03-01

    Craniopharyngiomas can be a surgical challenge for the pediatric neurosurgeon. Ideally, total removal must be achieved. However, the need to reduce surgical morbidity and preserve quality of life has led to a number of neurosurgical approaches in order to attain this goal. The aim of this article is to present an alternative surgical approach to these lesions and to provide the rationale for this technique. Medical charts and operative records of eight pediatric patients harboring craniopharyngiomas who underwent surgical treatment using a supraorbital eyebrow approach (SOA) were reviewed from 2014 to 2016. Only patients younger than 18 years with a minimum follow-up of 12 months were included in this study. Using pre-operative magnetic resonance (MRI) scans, tumors were classified according to their degree of hypothalamic involvement. The surgical technique is also described in detail. The study group included six males and two females with a mean age of 10 years (range, 2-16 years). The SOA was used successfully in elective surgery of eight craniopharyngiomas. The hypothalamus was displaced by the tumor in three patients and severely involved in five patients. Subtotal resection was undertaken in six patients, whereas gross-total resection was achieved in two. Endoscopic assistance was used after standard microscopic visualization in two out of eight cases. Cosmetic outcomes were excellent, and the complication rate related to the surgical procedure was quite low, apart from diabetes insipidus (which occurred in three out of the eight patients). In one patient, a large subdural collection needed surgery for evacuation. Mean follow-up was 23.2 months (range, 12-36 months). Additionally, no CSF leak or wound infection was identified. The supraorbital eyebrow approach is an alternative route to operate on craniopharyngiomas in properly selected cases of all pediatric age ranges, from infants to teenagers. There is sufficient working space for the endoscope and

  4. [Rathke cysts, craniopharyngioma, and colloid cysts : What are the differences between these pathologies?

    Science.gov (United States)

    Eymann, R; Kiefer, M

    2018-05-17

    Headache is the most common symptom of colloid cysts, Rathke cysts, and craniopharyngioma due to their location in the midline, being extra-axial and typically presenting in the parasellar region. Although these tumors are generally considered benign, each has its typical characteristics defined by its location and histology. These individual characteristics define whether surgery is necessary at all and if so, the preferred surgical approach and resection's totality. The histopathological findings primarily indicate that embryonic malformations-at the first glance, ectodermal in nature-cause these tumors. Due to the fact that these disturbances occur at the boundary between ectodermal stomodeum and endodermal cephalogaster, however, does leave some doubts.

  5. [Effect of recombinant human growth hormone therapy on metabolic parameters in patients with craniopharyngioma].

    Science.gov (United States)

    Mao, J F; Wang, X; Xiong, S Y; Zheng, J J; Yu, B Q; Nie, M; Wu, X Y; Qi, S T

    2017-11-14

    Objective: To investigate the effects of recombinant human growth hormone (rhGH) on metabolic parameters in patients with craniopharyngioma surgeries. Methods: Totallys 30 patients with craniopharyngioma were included in this retrospective study. They were divided into growth hormone (GH) group and control group according to whether they received rhGH therapy or not. The following parameters, including body mass index (BMI), weight, waist circumstance, transaminase, fasting blood glucose, lipid profile and high-sensitivity C-reactive protein (hsCRP) were compared after rhGH therapy for 4-6 months. Results: In GH group, patients were 18-46 (30.0±8.8) years old. The duration after craniopharyngioma surgery was (12.9±5.4) years. Before rhGH therapy, they had got sufficient thyroid and glucocorticoid hormone replacement. After rhGH therapy, the body weight decreased from (92.3±20.1) to (87.6 ±14.6) kg ( P =0.190), with a reduction of BMI from (30.1±5.9) to (28.2±3.7) kg/m(2) ( P =0.120). The waist circumference decreased from (104.4±9.4) cm to (98.8±10.6) cm ( P =0.002). Alanine aminotransferase (ALT) decreased from (52±34) to (28±19) U/L ( P =0.029), with a reduction of aspartate transaminase (AST) from (46±21) to (33±18) U/L ( P =0.035) and γ-glutamyl transpeptadase (GGT) from (59±42) to (29±15) U/L ( P =0.02). hsCRP decreased from (5.3±4.9) to (2.3±2.8) mg/L ( P =0.006) and triglyceride (TG) decreased from (1.8±0.7) to (1.5±0.6) mmol/L ( P =0.028). Fasting blood glucose, low density lipoprotein cholesterol (LDL-C), high density lipoprotein cholesterol (HDL-C) and free fat acid (FFA) were not significantly changed(all P >0.05). In the control group, the above mentioned parameters did not changed significantly during 4-6 months of observational period(all P >0.05). Conclusion: rhGH therapy improves metabolic parameters in patients after craniopharyngioma surgery by decreasing body weight, waist circumstance and fat deposit in liver, as well as

  6. [A rare case of diencephalic cachexia in an adult female with cranio-pharyngioma].

    Science.gov (United States)

    Klochkova, I S; Astaf'eva, L I; Konovalov, A N; Kadashev, B A; Kalinin, P L; Sharipov, O I; Kutin, M A; Sidneva, Yu G; Shishkina, L V; Pronin, I N

    Diencephalic cachexia (DС) is progressive weight loss despite a normal caloric intake and a satisfactory state of health, which is caused by hypothalamic lesions. This is a rare (about 100 cases were reported) and potentially fatal disorder of unknown pathogenesis. At present, there is no effective pharmacological therapy for the disorder. Cachexia may regress only if the tumor reduces in size, therefore the timely diagnosis and treatment are of vital importance for the patient. DС is typical of early childhood, and only a few cases have been reported in adults. We present a rare case of DС in a 24-year-old female with papillary craniopharyngioma.

  7. Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma.

    Science.gov (United States)

    Greenfield, Brad J; Okcu, Mehmet F; Baxter, Patricia A; Chintagumpala, Murali; Teh, Bin S; Dauser, Robert C; Su, Jack; Desai, Snehal S; Paulino, Arnold C

    2015-02-01

    To report long-term progression-free survival (PFS) and late-toxicity outcomes in pediatric craniopharyngioma patients treated with IMRT. Twenty-four children were treated with IMRT to a median dose of 50.4Gy (range, 49.8-54Gy). The clinical target volume (CTV) was the gross tumor volume (GTV) with a 1cm margin. The planning target volume (PTV) was the CTV with a 3-5mm margin. Median follow-up was 107.3months. The 5- and 10-year PFS rates were 65.8% and 60.7%. The 5- and 10-year cystic PFS rates were 70.2% and 65.2% while the 5- and 10-year solid PFS were the same at 90.7%. Endocrinopathy was seen in 42% at initial diagnosis and in 74% after surgical intervention, prior to IMRT. Hypothalamic dysfunction and visual deficits were associated with increasing PTV and number of surgical interventions. IMRT is a viable treatment option for pediatric craniopharyngioma. Despite the use of IMRT, majority of the craniopharyngioma patients experienced long-term toxicity, many of which present prior to radiotherapy. Limitations of retrospective analyses on small patient cohort elicit the need for a prospective multi-institutional study to determine the absolute benefit of IMRT in pediatric craniopharyngioma. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  8. Eating behavior, weight problems and eating disorders in 101 long-term survivors of childhood-onset craniopharyngioma.

    Science.gov (United States)

    Hoffmann, Anika; Postma, Frank P; Sterkenburg, Anthe S; Gebhardt, Ursel; Müller, Hermann L

    2015-01-01

    As a result of hypothalamic involvement and/or treatment-related hypothalamic damage, up to 75% of childhood craniopharyngioma patients develop hypothalamic obesity. Eating behavior was analyzed in 101 survivors of childhood craniopharyngioma, recruited from 1980 to 2001 in the HIT-Endo multicenter study, and in 85 body mass index (BMI)-matched healthy controls using the Inventory for Eating Behavior and Weight Problems (IEG) and the Inventory for Eating Disorders (ESI). Severely obese patients (BMI>8 SD; n=9) presented with pathological eating behavior, more weight problems, and eating disorders, as compared to obese (BMI 3-8 SD; n=44) and normal or overweight patients (BMICraniopharyngioma patients with different degrees of obesity showed similar or even less pathological findings as compared to BMI-matched normal controls. Severe obesity is associated with pathological eating behavior/disorders in craniopharyngioma patients. As these disorders are not disease-specific, risk factors for hypothalamic obesity should be the focus of further craniopharyngioma research.

  9. Frontal burr hole approach for neuroendoscopic resection of craniopharyngioma with the NICO Myriad device: report of two cases.

    Science.gov (United States)

    Moore, Reilin J; Scherer, Andrea; Fulkerson, Daniel H

    2017-04-01

    Craniopharyngiomas are challenging tumors to resect due to their deep location and proximity to vital structures. The perceived benefit of gross total resection may be tempered by the possibility of permanent disability. Minimally invasive techniques may reduce surgical morbidity while still allowing effective resection. The authors describe their initial experience with a neuroendoscopic transcortical, transventricular approach to two craniopharyngiomas. The surgeries were performed through a right frontal burr hole using the NICO Myriad, a side-cutting, aspiration device that fits through the working channel of a standard neuroendoscope. The imaging and medical records of two children (a 5-year-old male and a 9-year-old female) undergoing endoscopic resection of a craniopharyngioma with this technique were reviewed. Outcomes, results, and complications were noted. A gross total resection was achieved in both patients. The operative time was 180 and 143 min, respectively. The estimated blood loss was 20 and 50 cm 3 , respectively. Both patients required a cerebrospinal fluid shunt. There were no surgical complications. The NICO Myriad is an effective tool that allows a safe minimally invasive endoscopic resection of craniopharyngiomas in patients with amenable anatomy. Surgeons with experience in neuroendoscopy may be able to achieve a gross total resection of these challenging tumors through a minimally invasive burr hole approach.

  10. Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma

    International Nuclear Information System (INIS)

    Greenfield, Brad J.; Okcu, Mehmet F.; Baxter, Patricia A.; Chintagumpala, Murali; Teh, Bin S.; Dauser, Robert C.; Su, Jack; Desai, Snehal S.; Paulino, Arnold C.

    2015-01-01

    Purpose: To report long-term progression-free survival (PFS) and late-toxicity outcomes in pediatric craniopharyngioma patients treated with IMRT. Patients and methods: Twenty-four children were treated with IMRT to a median dose of 50.4 Gy (range, 49.8–54 Gy). The clinical target volume (CTV) was the gross tumor volume (GTV) with a 1 cm margin. The planning target volume (PTV) was the CTV with a 3–5 mm margin. Median follow-up was 107.3 months. Results: The 5- and 10-year PFS rates were 65.8% and 60.7%. The 5- and 10-year cystic PFS rates were 70.2% and 65.2% while the 5- and 10-year solid PFS were the same at 90.7%. Endocrinopathy was seen in 42% at initial diagnosis and in 74% after surgical intervention, prior to IMRT. Hypothalamic dysfunction and visual deficits were associated with increasing PTV and number of surgical interventions. Conclusions: IMRT is a viable treatment option for pediatric craniopharyngioma. Despite the use of IMRT, majority of the craniopharyngioma patients experienced long-term toxicity, many of which present prior to radiotherapy. Limitations of retrospective analyses on small patient cohort elicit the need for a prospective multi-institutional study to determine the absolute benefit of IMRT in pediatric craniopharyngioma

  11. Effects of T3 treatment on brown adipose tissue and energy expenditure in a patient with craniopharyngioma and hypothalamic obesity

    NARCIS (Netherlands)

    van Santen, Hanneke M.; Schouten-Meeteren, Antoinette Y.; Serlie, Mireille; Meijneke, Ruud W. H.; van Trotsenburg, A. S.; Verberne, Hein; Holleman, Frits; Fliers, Eric

    2015-01-01

    Patients treated for childhood craniopharyngioma often develop hypothalamic obesity (HO), which has a huge impact on the physical condition and quality of life of these patients. Treatment for HO thus far has been disappointing, and although several different strategies have been attempted, all

  12. Frequent occurrence of the triphasic response (diabetes insipidus/hyponatremia/diabetes insipidus) after surgery for craniopharyngioma in childhood

    NARCIS (Netherlands)

    Finken, Martijn J. J.; Zwaveling-Soonawala, Nitash; Walenkamp, Marie J. E.; Vulsma, Thomas; van Trotsenburg, A. S. Paul; Rotteveel, Joost

    2011-01-01

    It is not exactly known how many children develop the triphasic response (diabetes insipidus (DI)/hyponatremia/DI) immediately after surgery for childhood craniopharyngioma; neither is it known which factors predict this. We studied the occurrence of the triphasic response after primary surgery for

  13. The development of hypothalamic obesity in craniopharyngioma patients: A risk factor analysis in a well-defined cohort

    NARCIS (Netherlands)

    van Iersel, Laura; Meijneke, Ruud W. H.; Schouten-van Meeteren, Antoinette Y. N.; Reneman, Liesbeth; de Win, Maartje M.; van Trotsenburg, A. S. Paul; Bisschop, Peter H.; Finken, Martijn J. J.; Vandertop, W. Peter; van Furth, Wouter R.; van Santen, Hanneke M.

    2018-01-01

    Hypothalamic obesity (HO) is a major concern in patients treated for craniopharyngioma (CP). The influence of degree of resection on development of HO, event-free survival (EFS), and neuroendocrine sequelae is an issue of debate. A retrospective cohort consisting of all CP patients treated between

  14. Systemic Effects of Hypothermia due to Hypothalamic Dysfunction after Resection of a Craniopharyngioma : Case Report and Review of Literature

    NARCIS (Netherlands)

    de Vetten, L.; Bocca, Gianni

    Objective With this case report, we aim to improve recognition of the systemic effects of hypothermia due to hypothalamic dysfunctioning. We present a patient who developed temperature dysregulation after surgery for craniopharyngioma. He suffered from several episodes of hypothermia associated with

  15. Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report

    Directory of Open Access Journals (Sweden)

    Dai Jung

    2018-03-01

    Full Text Available Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD and diabetes mellitus (DM. We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.

  16. Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report.

    Science.gov (United States)

    Jung, Dai; Seo, Go Hun; Kim, Yoon-Myung; Choi, Jin-Ho; Yoo, Han-Wook

    2018-03-01

    Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.

  17. Frequent Occurrence of the Triphasic Response (Diabetes Insipidus/Hyponatremia/Diabetes Insipidus) after Surgery for Craniopharyngioma in Childhood

    NARCIS (Netherlands)

    Finken, M.J.J.; Zwaveling-Soonawala, N.; Walenkamp, M.J.E.; Vulsma, T.; van Trotsenburg, A.S.P.; Rotteveel, J.

    2011-01-01

    Background/Aims: It is not exactly known how many children develop the triphasic response (diabetes insipidus (DI)/hyponatremia/DI) immediately after surgery for childhood craniopharyngioma; neither is it known which factors predict this. We studied the occurrence of the triphasic response after

  18. Long term results after fractionated stereotactic radiotherapy (FSRT) in patients with craniopharyngioma: maximal tumor control with minimal side effects

    International Nuclear Information System (INIS)

    Harrabi, Semi B; Adeberg, Sebastian; Welzel, Thomas; Rieken, Stefan; Habermehl, Daniel; Debus, Jürgen; Combs, Stephanie E

    2014-01-01

    There are already numerous reports about high local control rates in patients with craniopharyngioma but there are only few studies with follow up times of more than 10 years. This study is an analysis of long term control, tumor response and side effects after fractionated stereotactic radiotherapy (FSRT) for patients with craniopharyngioma. 55 patients who were treated with FSRT for craniopharyngioma were analyzed. Median age was 37 years (range 6–70 years), among them eight children < 18 years. Radiotherapy (RT) was indicated for progressive disease after neurosurgical resection or postoperatively after repeated resection or partial resection. A median dose of 52.2 Gy (50 – 57.6 Gy) was applied with typical dose per fraction of 1.8 Gy five times per week. The regular follow up examinations comprised in addition to contrast enhanced MRI scans thorough physical examinations and clinical evaluation. During median follow up of 128 months (2 – 276 months) local control rate was 95.3% after 5 years, 92.1% after 10 years and 88.1% after 20 years. Overall survival after 10 years was 83.3% and after 20 years 67.8% whereby none of the deaths were directly attributed to craniopharyngioma. Overall treatment was tolerated well with almost no severe acute or chronic side effects. One patient developed complete anosmia, another one’s initially impaired vision deteriorated further. In 83.6% of the cases with radiological follow up a regression of irradiated tumor residues was monitored, in 7 cases complete response was achieved. 44 patients presented themselves initially with endocrinologic dysfunction none of them showed signs of further deterioration during follow up. No secondary malignancies were observed. Long term results for patients with craniopharyngioma after stereotactic radiotherapy are with respect to low treatment related side effects as well as to local control and overall survival excellent

  19. Outcome of endoscopy-assisted microscopic extended transsphenoidal surgery for suprasellar craniopharyngiomas

    Directory of Open Access Journals (Sweden)

    Hidetoshi eIkeda

    2012-02-01

    Full Text Available Objectives. Craniopharyngiomas are difficult to treat. The extended transsphenoidal approach has recently been described in several small series. We describe the usefulness of microscopy-assisted angled endoscopy for visualizing vital structures such as tumor attachment or tumor invasion to the pituitary stalk to achieve confident radical tumor removal.Design and Participants. Between 2006 and 2010, 15 patients underwent the microscopy-assisted extended transsphenoidal approach for resection of entirely suprasellar craniopharyngiomas. 14 patients had the transinfundibular type, and one had the transinfundibular type with the extension to third ventricle. We observed color change within the pituitary stalk by endoscopy. The pituitary stalk was cut intentionally in ten patients, because of suspected tumor invasion surrounding the stalk. Main Outcome Measures and Results. Total removal was accomplished in nine patients. Pathological specimens from the pituitary stalk showed tumor invasion spreading over the surface of the pituitary stalk, shown by a discolored pituitary stalk, and this was essential for confident radical tumor removal. Even after stalk resection, postoperative DI was minimal when a bright signal on T1 in the posterior lobe was not confirmed on preoperative magnetic resonance imaging. Conclusion. Confident radical tumor removal is possible with the introduction of the endoscopy-assisted microscopic extended transsphenoidal approach.

  20. Periostin activates pathways involved in epithelial-mesenchymal transition in adamantinomatous craniopharyngioma.

    Science.gov (United States)

    Chen, Ming; Zheng, Shi-hao; Liu, Yi; Shi, Jin; Qi, Song-tao

    2016-01-15

    Periostin (POSTN) is an extracellular matrix protein (ECM) critical for epithelial-mesenchymal transitions (EMT) in several kinds of tumor cells. Previous studies have indicated that EMT exists in craniopharyngioma (CP), and expression of POSTN is a significant factor in the prognosis of CP. However, it has never been explored whether POSTN exists in CP, or how it activates CP's EMT. The expression of POSTN was examined in adamantinomatous craniopharyngioma (ACP) primary cells and tissues by immunohistochemistry, PCR and Western blot, respectively. The effects and mechanisms of POSTN on ACP cells' EMT were also analyzed. It was found that POSTN expression increased in ACP-associated fibroblasts. Overexpressed POSTN significantly elevated the EMT of ACP cells by regulating the expression of associated genes. More importantly, our further study revealed that the upregulated POSTN activated Akt signaling pathway to regulate the EMT. This study showed that POSTN is responsible for the EMT of ACP cells, and POSTN might be a potential molecular therapeutic target for ACP treatment in future. Copyright © 2015 Elsevier B.V. All rights reserved.

  1. Hypothalamic Obesity following Craniopharyngioma Surgery: Results of a Pilot Trial of Combined Diazoxide and Metformin Therapy

    Directory of Open Access Journals (Sweden)

    Ahmet Alexandra

    2011-03-01

    Full Text Available Objective. To assess the effect of combined diazoxide-metformin therapy in obese adolescents treated for craniopharyngioma. Design. A prospective open-label 6-month pilot treatment trial in 9 obese subjects with craniopharyngioma. Diazoxide (2 mg/kg divided b.i.d., maximum 200 mg/day and metformin (1000 mg b.i.d.. Whole body insulin sensitivity index (WBISI and area-under-the-curve insulin (AUCins were calculated. Results. Seven subjects completed: 4M/3F, mean ± SD age years, weight  kg, BMI  kg/m2, and BMI SDS . Two were withdrawn due to vomiting and peripheral edema. Of participants completing the study, the mean ± SD weight gain, BMI, and BMI SDS during the 6 months were reduced compared to the 6 months prestudy ( versus  kg, ; versus  kg/m2, ; versus , , resp.. AUCins correlated with weight loss (, and BMI decrease (, . Conclusion. Combined diazoxide-metformin therapy was associated with reduced weight gain in patients with hypothalamic obesity. AUCins at study commencement predicted effectiveness of the treatment.

  2. Simultaneous development of craniopharyngioma and choroid plexus carcinoma in the childhood -a clinical case

    International Nuclear Information System (INIS)

    Marinova, L.; Georgiev, R.; Mihaylova, I.

    2014-01-01

    We present a clinical case of 9 years old girl with concomitant brain tumors - choroid plexus carcinoma and craniopharyngioma diagnosed in 2009. After three operations, cranio-spinal irradiation with boost for the remaining tumor located in left ventricular trigonum to a total dose of 55 Gy and 7 courses chemotherapy, local tumor control was achieved for the choroid plexus carcinoma. Four years following the achievement of local tumor control of the choroid plexus carcinoma, an increase of the tumor formation located in the left side of the pituitary was reported. The diagnosis cystic craniopharyngeoma was found during the surgical operation. With this clinical case we would like to stress on the achieved local tumor control following the complex treatment of carcinoma of the choroid plexus, as well as on the slow growth of simultaneously diagnosed craniopharyngeoma. This case report raises the question of the genetic predisposition of the brain tumors in children, as well as possibility of malignant transformation of craniopharyngeoma following radiotherapy. The differential diagnosis of neuroectodermal brain tumors requires immunohistochemical analysis and if necessary genetic analysis. Key words: Complex treatment. Choroid plexus carcinoma. Craniopharyngioma. Radiotherapy. Malignant transformation. Simultaneity

  3. Megavoltage external beam irradiation of craniopharyngiomas: Analysis of tumor control and morbidity

    International Nuclear Information System (INIS)

    Flickinger, J.C.; Lunsford, L.D.; Singer, J.; Cano, E.R.; Deutsch, M.

    1990-01-01

    From 1971 to 1985, 21 patients received megavoltage external beam radiation therapy at the University of Pittsburgh for control of craniopharyngioma. Minimum tumor doses prescribed to the 95% isodose volume ranged between 51.3 to 70.0 Gy. Median total dose was 60.00 Gy and median dose per fraction was 1.83 Gy. Three deaths occurred from intercurrent disease and no deaths from tumor progression. Actuarial overall survival was 89% and 82% at 5 and 10 years. Actuarial local control was 95% at 5 and 10 years. Radiation related complications included one patient with optic neuropathy, one with brain necrosis, and one that developed optic neuropathy followed by brain necrosis. The high dose group of patients who received a NSD or Neuret equivalent of greater than 60 Gy at 1.8 Gy per fraction had a significantly greater risk of radiation complications (p = .024). The actuarial risk at 5 years for optic neuropathy was 30% and brain necrosis was 12.5% in the high dose group. Tumor control in the high dose group was not shown to be significantly better. Any possible benefit in tumor control in treating patients with craniopharyngioma with doses above 60 Gy at 1.8 Gy per fraction appears to be offset by the increased risk of radiation injury

  4. Endoscopic Endonasal Approach for Resection of a Pediatric Craniopharyngioma: Operative Video and Technical Nuances.

    Science.gov (United States)

    Liu, James K; Eloy, Jean Anderson

    2018-04-01

    We present a pediatric case of a retrochiasmatic craniopharyngioma in the suprasellar region with third ventricular extension that was resected through a purely endoscopic endonasal approach (EEA) via the transplanum transtuberculum corridor. The patient is a 12-year-old boy who presented with progressive visual loss and panhypopituitarism. The EEA allows direct visualization of the undersurface of the optic chiasm and hypothalamus so that safe and meticulous tumor dissection can be performed to preserve these critical neurovascular structures. This video atlas demonstrates the operative technique and surgical nuances of the endoscopic skull base approach, microdissection of the tumor from the critical neurovascular structures, and multilayered reconstruction of the skull base defect with a nasoseptal flap. A gross total resection was achieved, and the patient was neurologically intact with improved visual acuity and visual fields. In summary, the EEA via the transplanum transtuberculum corridor is an important strategy in the armamentarium for surgical management of pediatric craniopharyngiomas. The link to the video can be found at: https://youtu.be/bmgO_PMRHPk .

  5. Craniopharyngioma identification by CT and MR imaging at 1.5 T

    Energy Technology Data Exchange (ETDEWEB)

    Hald, J.K. [Dept. of Radiology, Rikshospitalet, Oslo Univ. (Norway)]|[Dept. of Radiology, Univ. of Michigan Hospitals, Ann Arbor, MI (United States); Eldevik, O.P. [Dept. of Radiology, Rikshospitalet, Oslo Univ. (Norway)]|[Dept. of Radiology, Univ. of Michigan Hospitals, Ann Arbor, MI (United States); Skalpe, I.O. [Dept. of Radiology, Rikshospitalet, Oslo Univ. (Norway)]|[Dept. of Radiology, Univ. of Michigan Hospitals, Ann Arbor, MI (United States)

    1995-03-01

    To compare the detectability of craniopharyngiomas by CT and MR imaging, preoperative CT and MR studies obtained within 16 days of each other were evaluated retrospectively in 9 patients. MR imaging demonstrated cystic and solid tumor components in all 9 tumors, and enhancement in the 7 tumors that were studied after contrast medium injection. MR imaging demonstrated a signal void consistent with calcification in 4 patients. Combining unenhanced and contrast medium-enhanced studies, CT also identified all the tumors. CT demonstrated cysts in 7 lesions, calcification in 7 and enhancement in 6 of the 7 lesions that received i.v. contrast medium. Calcification was better seen by CT than MR imaging, while MR imaging identified cystic tumor components not seen on CT. The contrast medium enhancement pattern was the same with the 2 modalities. MR imaging of the sellar region, including at least one contrast medium-enhanced sequence, should be sufficient in most instances to establish a preoperative diagnosis of craniopharyngioma. (orig.).

  6. Central pontine and extrapontine myelinolysis in an infant associated with the treatment of craniopharyngioma: case report.

    Science.gov (United States)

    Tsutsumi, Satoshi; Yasumoto, Yukimasa; Ito, Masanori

    2008-08-01

    A 3-year-old girl presented with osmotic demyelination syndrome after undergoing uneventful neuroendoscopic cystostomy for a growing cystic suprasellar craniopharyngioma following microscopic subtotal resection 1 year previously. Endocrinopathy had well been controlled by hormone replacement therapy and administration of 1-amino-8-d-arginine-vasopressin with serum sodium concentration within the normal range. She presented generalized seizure and fever on postoperative day 7, with hyponatremia beginning on postoperative day 4 and deteriorating despite frequent correction. The serum sodium concentration began to fluctuate on the same day, in the range 111-164 mEq/l, which lasted for 2 weeks, refractory for intense management. Her body temperature also fluctuated between hypo- and hyperthermia not correlated with serum inflammatory markers. Her conscious disturbance progressively deteriorated with spastic paraparesis. T(2)-weighted magnetic resonance (MR) imaging taken on postoperative day 19 revealed hyperintense areas in the pons, external capsule, bilateral thalami, and basal nuclei, which had not been recognized before, suggesting osmotic demyelination syndrome causing central pontine and extrapontine myelinolysis. MR imaging taken on postoperative days 230 and 360 showed some diminished lesions but others persisted and resulted in a cavity. The patient's depressed conscious level did not improve. Suprasellar craniopharyngioma with long-standing hypothalamic dysfunction may be associated with severe osmotic demyelination syndrome even after less invasive surgery, so serum sodium derangement after surgery should be promptly corrected even if only subtle signs are present.

  7. Mixed pituitary adenoma/craniopharyngioma: clinical, morphological, immunohistochemical and ultrastructural study of a case, review of the literature, and pathogenetic and nosological considerations.

    Science.gov (United States)

    Finzi, Giovanna; Cerati, Michele; Marando, Alessandro; Zoia, Cesare; Ferreli, Fabio; Tomei, Giustino; Castelnuovo, Paolo; La Rosa, Stefano; Capella, Carlo

    2014-02-01

    Mixed pituitary adenoma/craniopharyngiomas are very rare tumors. Their pathogenesis is still unclear and it is not known whether they are collision tumors derived from independent stem cells or whether they originate from a single stem cell undergoing divergent differentiation. The latter hypothesis is supported by the close commixture between the two tumor components with transition areas that has been previously described. However, "hybrid" cells with both pituitary adenoma and craniopharyngioma features have never been described. In this paper we report a case of mixed pituitary adenoma/craniopharyngioma observed in a 75-year-old woman presenting with diplopia and slight increase of serum prolactin, who underwent endoscopic endonasal trans-sphenoidal tumor resection. Histologically, the tumor was composed of a typical pituitary silent subtype 2 ACTH cell adenoma admixed with islands of adamantinomatous craniopharyngioma. Electron microscopy showed that, in addition to distinct silent subtype 2 ACTH and craniopharyngioma cells, there were "hybrid" cells, showing characteristics of both pituitary adenoma and craniopharyngioma, consisting of small dense secretory granules, bundles of cytoplasmic filaments, and desmosomes. This ultrastructural finding was also confirmed by the presence of cells showing nuclear p40 expression and chromogranin A immunoreactivity. The close commixture between the two components and the ultrastructural and immunohistochemical findings demonstrate a common histogenesis of the two components and support the classification of the neoplasm as a mixed tumor. The patient completely recovered and, 10 months after surgery, head MR confirmed the complete resection of the lesion.

  8. A quantitative analysis of craniopharyngioma cyst expansion during and after radiation therapy and surgical implications.

    Science.gov (United States)

    Lamiman, Kelly; Wong, Kenneth K; Tamrazi, Benita; Nosrati, Jason D; Olch, Arthur; Chang, Eric L; Kiehna, Erin N

    2016-12-01

    OBJECTIVE When complete resection of craniopharyngioma is not achievable or the sequelae are prohibitive, limited surgery and radiation therapy have demonstrated excellent local disease control while minimizing treatment-related sequelae. When residual tissue exists, there is a propensity for further cyst development and expansion during and after radiation therapy. This can result in obstructive hydrocephalus, visual changes, and/or clinical decline. The authors present a quantitative analysis of cyst expansion during and after radiotherapy and examine how it affected subsequent management. METHODS The authors performed an institutional review board-approved retrospective study of patients with histologically confirmed craniopharyngioma treated between 2000 and 2015 with surgery and intensity-modulated radiation therapy (IMRT) at a single institution. Volumetric measurements of cyst contours were generated by radiation oncology treatment planning software postoperatively, during IMRT, and up to 12 months after IMRT. Patient, tumor, and treatment-related variables were collected until the last known follow-up and were analyzed. RESULTS Twenty-seven patients underwent surgery and IMRT. The median total radiation dose was 54 Gy. Of the 27 patients, 11 patients (40.7%) demonstrated cyst expansions within 1 year of IMRT. Of note, all tumors with cyst expansion were radiographically Puget Grade 2. Maximal cyst expansion peaked at 4.27 months following radiation therapy, with a median volume growth of 4.1 cm 3 (mean 9.61 cm 3 ) above the postoperative cyst volume. Eight patients experienced spontaneous cyst regression without therapeutic intervention. Three patients experienced MRI-confirmed cyst enlargement during IMRT, all of whom required adaptive planning to ensure adequate coverage of the entire tumor volume. Two of these 3 patients required ventriculoperitoneal shunt placement and additional intervention. One underwent additional resection, and the other had

  9. On the Benefits and Risks of Proton Therapy in Pediatric Craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Beltran, Chris, E-mail: chris.beltran@stjude.org [Division of Radiation Oncology, St Jude Children' s Research Hospital, Memphis, TN (United States); Roca, Monica; Merchant, Thomas E. [Division of Radiation Oncology, St Jude Children' s Research Hospital, Memphis, TN (United States)

    2012-02-01

    Purpose: Craniopharyngioma is a pediatric brain tumor whose volume is prone to change during radiation therapy. We compared photon- and proton-based irradiation methods to determine the effect of tumor volume change on target coverage and normal tissue irradiation in these patients. Methods and Materials: For this retrospective study, we acquired imaging and treatment-planning data from 14 children with craniopharyngioma (mean age, 5.1 years) irradiated with photons (54 Gy) and monitored by weekly magnetic resonance imaging (MRI) examinations during radiation therapy. Photon intensity-modulated radiation therapy (IMRT), double-scatter proton (DSP) therapy, and intensity-modulated proton therapy (IMPT) plans were created for each patient based on his or her pre-irradiation MRI. Target volumes were contoured on each weekly MRI scan for adaptive modeling. The measured differences in conformity index (CI) and normal tissue doses, including functional sub-volumes of the brain, were compared across the planning methods, as was target coverage based on changes in target volumes during treatment. Results: CI and normal tissue dose values of IMPT plans were significantly better than those of the IMRT and DSP plans (p < 0.01). Although IMRT plans had a higher CI and lower optic nerve doses (p < 0.01) than did DSP plans, DSP plans had lower cochlear, optic chiasm, brain, and scanned body doses (p < 0.01). The mean planning target volume (PTV) at baseline was 54.8 cm{sup 3}, and the mean increase in PTV was 11.3% over the course of treatment. The dose to 95% of the PTV was correlated with a change in the PTV; the R{sup 2} values for all models, 0.73 (IMRT), 0.38 (DSP), and 0.62 (IMPT), were significant (p < 0.01). Conclusions: Compared with photon IMRT, proton therapy has the potential to significantly reduce whole-brain and -body irradiation in pediatric patients with craniopharyngioma. IMPT is the most conformal method and spares the most normal tissue; however, it is highly

  10. A reformed surgical treatment modality for children with giant cystic craniopharyngioma.

    Science.gov (United States)

    Zhu, Wanchun; Li, Xiang; He, Jintao; Sun, Tao; Li, Chunde; Gong, Jian

    2017-09-01

    Surgical removal plays an important role in treating children's craniopharyngioma. For a safe and minimally invasive craniotomy, a reformed surgical modality was proposed in this paper by combining the insertion of an Ommaya reservoir system (ORS) by stereotactic puncture, aspiration of cystic fluid in 2-day interval for consecutive 7-10 days, and the delayed tumor resection. Eleven patients (aged from 5 to 9 years old) with giant cystic craniopharyngiomas who had undergone the reformed surgical modality during November 2014 and December 2015 were collected as group A. In contrast, seven patients (aged from 5 to 11 years old) who had undergone the traditional directed operation without any prior management from January 2014 to October 2014 were collected into group B. A retrospective analysis was performed for both groups at one institution. The preoperative and postoperative clinical presentations, neuroimaging, early postoperative outcome, and the surgery-related complications of both groups were reviewed. For group A, the mean value of the maximum tumor diameters shank from 52.36 to 23.82 mm after implementing aspiration of the cystic fluid in 1-day interval for consecutive 8.23 days. Eight patients (72.73%) in group A underwent a gross total resection (GTR), while two (28.57%) patients underwent GTR in group B. The postoperative electrolyte disturbance rate and endocrine disorder rate of group B were significantly higher than those of group A (42.86 vs 36.36%; 71.43 vs 45.45%). Postoperative long-term diabetes insipidus only occurred in one patient of group B, and postoperative visual deterioration occurred in two patients of group B. Besides, one patient of group B died of severe postoperative hypothalamus dysfunction. Patients with residual tumors were applied with additional adjuvant radiotherapy, and no recurrence was observed in follow-up examinations. A favorable outcome can be achieved by combining the insertion of an ORS by stereotactic puncture

  11. Genetically engineered mouse models of craniopharyngioma: an opportunity for therapy development and understanding of tumor biology.

    Science.gov (United States)

    Apps, John Richard; Martinez-Barbera, Juan Pedro

    2017-05-01

    Adamantinomatous craniopharyngioma (ACP) is the commonest tumor of the sellar region in childhood. Two genetically engineered mouse models have been developed and are giving valuable insights into ACP biology. These models have identified novel pathways activated in tumors, revealed an important function of paracrine signalling and extended conventional theories about the role of organ-specific stem cells in tumorigenesis. In this review, we summarize these mouse models, what has been learnt, their limitations and open questions for future research. We then discussed how these mouse models may be used to test novel therapeutics against potentially targetable pathways recently identified in human ACP. © 2017 The Authors. Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology.

  12. Childhood craniopharyngioma: survival, local control, endocrine and neurologic function following radiotherapy

    International Nuclear Information System (INIS)

    Danoff, B.F.; Cowchock, F.S.; Kramer, S.

    1983-01-01

    Between 1961 and 1978, 19 patients with a diagnosis of childhood or teenage craniopharyngioma received supervoltage radiotherapy. All patients had previously undergone either partial surgical resection (10 patients), total gross resection (3 patients), or aspiration and biopsy (6 patients). Fourteen patients were treated primarily and five were treated for recurrence. The five-year survival was 73% with a 10-year survival of 64%. Sixteen percent developed a recurrence following radiotherapy. Long term effects were assesed in terms of neurologic, intellectual, psychological and endocrine function. Seventy-nine percent had none or minimal neurologic disability. The mean full scale IQ for the group was 90. There were no additional endocrine deficiencies that could be directly attributed to radiation. Behavioral disorders occurred in 50%. These results are at least comparable, if not superior, to those of surgery

  13. Radiotherapeutic results in the management of craniopharyngioma. Strahlentherapeutische Ergebnisse bei der Behandlung von Kraniopharyngeomen

    Energy Technology Data Exchange (ETDEWEB)

    Latz, D.; Schraube, P. (Radiologische Universitaetsklinik, Heidelberg (Germany)); Mittermaier, G. (Paediatrische Universitaetsklinik, Heidelberg (Germany))

    1992-02-01

    From 1974 to 1985, twelve patients (seven children and five adults) with craniopharyngioma received megavoltage external beam radiation therapy at the University of Heidelberg. All patients had biopsy proven diagnosis. Nine patients received irradiation after incomplete surgical removal. Three patients were irradiated primary because of inoperability. In all cases irradiation was applied by a rotation technique with 42 MeV photons. A mean dose of 52.2 Gy (ranged from 46 yo 60 Gy) was administered at 2.0 Gy per fraction. In one case radiotherapy was broken off on account of the development of a hyperglycemic coma of unknown genesis. The mean follow-up period amounts nine years up to now. Local tumor control was achieved in nine patients. Two deaths occurred from tumor progression, after temporary improvement. In all cases no radiation related neurological, ophthalmological or endocrine side ffects were observed. (orig.).

  14. Recurrent papillary craniopharyngioma with BRAF V600E mutation treated with dabrafenib: case report.

    Science.gov (United States)

    Himes, Benjamin T; Ruff, Michael W; Van Gompel, Jaimie J; Park, Sean S; Galanis, Evanthia; Kaufmann, Timothy J; Uhm, Joon H

    2018-04-27

    The authors present the case of a man with a papillary craniopharyngioma, first diagnosed at 47 years of age, who experienced multiple recurrences. Review of the pathologic specimen from his first resection demonstrated the BRAF V600E mutation. With his most recent recurrence following previous surgery and radiotherapy, at 52 years of age, the decision was made to initiate treatment with the BRAF V600E inhibitor dabrafenib. Imaging following initiation of dabrafenib demonstrated reduction in tumor size. He remained on dabrafenib therapy for approximately 1 year and continued to demonstrate a good clinical result. At that time the decision was made to discontinue dabrafenib therapy and follow up with serial imaging. After more than 1 year of follow-up since stopping dabrafenib, the patient has continued to do well with no radiographic evidence of tumor progression and continues to be monitored with frequent interval imaging.

  15. Dosimetry of {sup 32}P radiocolloid for treatment of cystic craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Sadeghi, Mahdi [Engineering Faculty, Science and Research Campus, Islamic Azad University, P.O. Box 14155-775, Tehran (Iran, Islamic Republic of); Radioisotope Production Department, Atomic Energy Organization, Tehran (Iran, Islamic Republic of); E-mail: msadeghi@nrcam.org; Moradi, Somayeh [Engineering Faculty, Science and Research Campus, Islamic Azad University, P.O. Box 14155-775, Tehran (Iran, Islamic Republic of); Shahzadi, Sohrab [Radiosurgery Department, Tajrish Shohada Hospital, Shahid Beheshti University, Tehran (Iran, Islamic Republic of); Pourbeigi, Hossien [Radioisotope Production Department, Atomic Energy Organization, Tehran (Iran, Islamic Republic of)

    2007-05-15

    In Tajrish Shohada Hospital, patients with either cystic craniopharyngiomas or cystic astrocytomas have been treated with {sup 32}P radiocolloid by stereotactic procedure. The total activity was prescribed for delivery dose between 200 and 300 Gy to the cyst wall thicknesses of 1-3 mm. In this project, MD-55-2 radiochromic film was utilized to determine the dose distribution around the source. The film dosimetry data were compared with Monte Carlo simulated values calculated with MCNP4C code. In addition, the clinical and dosimetric factors such as the cyst volume, and method of radiocolloid injection were evaluated. The required activity and distribution of radial dose in and out of cyst wall have been investigated.

  16. Hypothalamic obesity in patients with craniopharyngioma: Profound changes of several weight regulatory circuits

    Directory of Open Access Journals (Sweden)

    Christian eRoth

    2011-10-01

    Full Text Available One of the most striking examples of dysfunctional hypothalamic signaling of energy homeostasis is observed in patients with hypothalamic lesions leading to hypothalamic obesity (HO. This drastic condition is frequently seen in patients with craniopharyngioma (CP, an embryological tumor located in the hypothalamic and/or pituitary region, frequently causing not only hypopituitarism, but also leading to damage of medial hypothalamic nuclei due to the tumor and its treatment. HO syndrome in CP patients is characterized by fatigue, decreased physical activity, uncontrolled appetite, and morbid obesity, and is associated with insulin and leptin resistance. Mechanisms leading to the profoundly disturbed energy homeostasis are complex. This review summarizes different aspects of important clinical studies as well as data obtained in rodent studies. In addition a model is provided describing how medial hypothalamic lesion can interact simultaneously with several weight regulating circuitries.

  17. A case of paraventricular anaplastic astrocytoma following radiation therapy for craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Shimizu, Hiroaki; Fujiwara, Kazunori; Kobayashi, Shin-ichi; Kitahara, Masakazu (Ishinomaki Red Cross Hospital, Miyagi (Japan))

    1994-04-01

    A 20-year-old man received 60 Gy of radiation therapy after partial removal of craniopharyngioma. The patient had been well and follow-up CT scans did not show any aggravation for 16 years. Since his activity gradually diminished, he underwent an MRI at the age of 36 which revealed and abnormal mass on the corpus callosum. The mass lesion progressively enlarged thereafter, and was diagnosed as anaplastic astrocytoma by a stereotactic biopsy. He was treated with interferon, however, died at the age of 37. Review of the literature disclosed 19 other cases of glioma following radiation therapy for sellar/parasellar tumors. Characteristic features of these cases included (1) lowness of age compared to common glioma cases, (2) tendency to be malignant, (3) tendency to occur in areas where significant doses of radiation had been received previously. (author).

  18. A juvenile case of radiation-induced meningioma two years after radiation for craniopharyngioma

    International Nuclear Information System (INIS)

    Kano, Tomoaki; Zama, Akira; Ono, Nobuo; Nakamura, Tadashi; Tamura, Masaru; Ohe, Tihiro; Nakazato, Yoichi

    1994-01-01

    The patient was a 7-years-old boy who received radiation therapy of 50 Gy after total gross removal of a craniopharyngioma. After two years a follow up CT scan showed a new enhanced lesion in the right temporal tip within the previous irradiation field. Total removal of the tumor was performed and its histological examination showed it to be an atypical meningioma. This atypical meningioma satisfied Cahan's criteria. So we diagnosed this atypical meningioma as radiation-induced meningioma. Immunohistochemically this meningioma stained for Vimentin. An electron microscopical examination showed neither desmosome nor interdigitation. The score of Ki-67 and BrdU-L. I was very small. Compared with previously reported juvenile radiation-induced meningioma, the latency was very short. The patient received growth hormone (GH) replacement therapy. We suspected relation between GH replacement therapy and short latency. He was discharged without any new neurological deficits and we haven't detected tumor recurrence for two years. (author)

  19. Evaluation of Macular Ganglion Cell Complex and Peripapillary Retinal Nerve Fiber Layer in Primary Craniopharyngioma by Fourier-Domain Optical Coherence Tomography.

    Science.gov (United States)

    Yang, Liu; Qu, Yuanzhen; Lu, Wen; Liu, Fengjun

    2016-07-03

    BACKGROUND The aim of this study was to compare the differences in macular ganglion cell complex (GCC) and peripapillary retinal nerve fiber layer (pRNFL) in child and adult patients with primary craniopharyngioma by Fourier-domain optical coherence tomography (FD-OCT) and to evaluate their significance in the diagnosis of primary craniopharyngioma. MATERIAL AND METHODS Ninety-six participants were divided into 3 groups: 32 in the child craniopharyngioma group (CCG) and 32 in the adult craniopharyngioma group (ACG) who were treated in Beijing Tiantan Hospital between November 2013 and October 2014, and 32 in the normal group (NG). All subjects were scanned by FD-OCT to map GCC and pRNFL thicknesses. Spearman correlation coefficient was used to assess the correlation between GCC and pRNFL thickness, and pRNFL thickness and optic nerve head (ONH) parameters, including horizontal cup-disc ratio (HCDR), vertical cup-disc ratio (VCDR), optic disc area (ODA), and cup area (CA), respectively. RESULTS The correlation between GCC and pRNFL thickness in the CCG was slightly stronger compared with the ACG. A significant difference in GCC thickness was observed among the CCG, ACG, and NG. Although the pRNFL thickness in both the CCG and ACG was significantly higher than that in NG, no significant difference in pRNFL thickness was detected between the 2 craniopharyngioma groups. The average, superior, and inferior pRNFL thicknesses were negatively correlated with VCDR in the CCG (in double eyes) and ACG (only in left eyes). CONCLUSIONS GCC was more sensitive than pRNFL in detecting optic nerve damage in the eyes of craniopharyngioma patients. A thinner pRNFL was especially correlated with VCDR in child craniopharyngioma patients.

  20. Comparison of energy expenditure, body composition, metabolic disorders, and energy intake between obese children with a history of craniopharyngioma and children with multifactorial obesity.

    Science.gov (United States)

    Bomer, Ilanit; Saure, Carola; Caminiti, Carolina; Ramos, Javier Gonzales; Zuccaro, Graciela; Brea, Mercedes; Bravo, Mónica; Maza, Carmen

    2015-11-01

    Craniopharyngioma is a histologically benign brain malformation with a fundamental role in satiety modulation, causing obesity in up to 52% of patients. To evaluate cardiovascular risk factors, body composition, resting energy expenditure (REE), and energy intake in craniopharyngioma patients and to compare the data with those from children with multifactorial obesity. All obese children and adolescents who underwent craniopharyngioma resection and a control group of children with multifactorial obesity in follow-up between May 2012 and April 2013. Anthropometric measurements, bioelectrical impedance, indirect calorimetry, energy intake, homeostatic model assessment insulin resistance (HOMA-IR), and dyslipidemia were evaluated. Twenty-three patients with craniopharyngioma and 43 controls were included. Children with craniopharyngioma-related obesity had a lower fat-free mass percentage (62.4 vs. 67.5; p=0.01) and a higher fat mass percentage (37.5 vs. 32.5; p=0.01) compared to those with multifactorial obesity. A positive association was found between %REE and %fat-free mass in subjects with multifactorial obesity (68±1% in normal REE vs. 62.6±1% in low REE; p=0.04), but not in craniopharyngioma patients (62±2.7 in normal REE vs. 61.2±1.8% in low REE; p=0.8). No differences were found in metabolic involvement or energy intake. REE was lower in craniopharyngioma patients compared to children with multifactorial obesity regardless of the amount of fat-free mass, suggesting that other factors may be responsible for the lower REE.

  1. Endoscopic Endonasal Resection of Retrochiasmatic Craniopharyngioma with Fascia Lata Button and Nasoseptal Flap Reconstruction.

    Science.gov (United States)

    Kshettry, Varun R; Nyquist, Gurston; Evans, James J

    2018-02-01

    Surgery for craniopharyngiomas can be challenging due to the involvement of multiple critical neurovascular structures. The expanded endoscopic endonasal approach can provide superior access to suprasellar craniopharyngiomas, particularly with retrochiasmatic extension and significant hypothalamic involvement. We describe the surgical technique used to treat a 30-year-old patient who presented with 4 weeks of worsening vision, fatigue, and memory loss. His vision was counting fingers at 1 feet on the right and 20/800 on the left with a temporal hemianopsia. Laboratory evaluation demonstrated central hypoadrenalism, hypothyroidism, and hypogonadism. Imaging showed a large solid and cystic suprasellar mass. The transtubercular approach with removal of the lateral tubercular strut can provide wide bilateral access to the opticocarotid region. The superior intercavernous sinus must be coagulated and ligated. Initial arachnoid dissection is centered at the midline, mobilizing the superior hypophyseal branches to the optic apparatus laterally. The cyst capsule is opened and care is taken to minimize spillage of cyst fluid into the subarachnoid space. Central debulking and then extracapsular dissection is performed under direct visualization using sharp dissection. Reconstruction of the dura is performed with an inlay/onlay fascia lata button that is held together with four sutures that hold the graft edges against the native dural edges. This is followed by vascularized nasoseptal flap reconstruction. No lumbar drain or nonabsorbable packing is required. The patient's vision had dramatic improvement and by 1 week postoperatively was 20/20 with full visual fields. Postoperative diabetes insipidus was managed with nasal desmopressin. Postoperative MRI demonstrated complete removal. The link to the video can be found at: https://youtu.be/QQxCNUcq1qg .

  2. A review of Craniopharyngioma and the retrospective study on 100 patients

    Directory of Open Access Journals (Sweden)

    Larijani B

    1995-07-01

    Full Text Available This study was performed in order to improve the knowledge about Craniopharyngioma tumors, and tried to present extensive datas about the signs and symptoms, the clinical process and the treatments and their complications of the patients with Craniopharyngioma, admitted in the hospitals of Tehran university during 15 years (1355-70. Ultimately, these datas have been compared with those in the reputable books and new medical papers; And the results are present at the end of the discussion. Undoubtedly, this study is not free of the limitations of retrospective studies. Our patients-contrary to the textbooks are predominantly males; And also their most common chief complaints are neurological manifestations, and headache (82% is the most common one. There is only a little difference in the prevalence of some of the endocrinologic and ophthalmic manifestations between our datas and what in the texts, while, in some others, we can see a great diversity between the 2. For instance, decreased libido in men and amenorrhea in women, among our patients are prominently less than what we study in the books; And blood pressure disturbances, sensory and motor symptoms and urinary incontinence have not been seen in our patients. In most cases, CT scan leads to diagnosis and its diagnostic accuracy in 2 times higher than that of the simple radiography and it can show the sella enlargement obviously. In a majority of cases (More than 95%, excision a part of the tumor (From just limited to the biopsy area to 90% of tumor mass was the only therapeutic measure. In only a few patients, tumor excision was followed by radiotherapy, that showed the better results. The most common delayed complication was the recurrence of the disease.

  3. Long-term effects of gamma knife radiosurgery for pediatric craniopharyngioma

    International Nuclear Information System (INIS)

    Osuka, Koji; Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki; Yoshida, Kazuo; Hasegawa, Toshinori

    2001-01-01

    Twenty-one pediatric patients with craniopharyngioma have undergone gamma knife radiosurgery at our facility since May 1991, and we have followed up 10 of these patients for at least 24 months (mean 44.5 months). The patients' ages ranged from 6 to 12 years (mean 8.6 years). Initial presentations were headache in 7 patients, decreased visual acuity in 8, visual field deficit in 5, hypopituitarism in 7, and diabetes insipidus in 3. Prior to radiosurgery, surgical procedures had been performed in 8 patients, conventional radiotherapy in 1, and chemotherapy in 1. Mean tumor diameter was 17.4 mm, and treatment was by gamma knife, with mean maximum and marginal doses of 25.4 Gy and 13.3 Gy, respectively. Follow-up MRI revealed good tumor control (partial response+complete response) in 7 of the 10 patients, with neither further neurological nor endocrinological deterioration. Two patients showed tumor shrinkage for 60 months and 46 months, respectively, after radiosurgery, while, thereafter, tumor gradually recurred at the side of the optic nerve. In one patient, rapid growth of the cyst was observed 6 months after radiosurgery. Gamma knife radiosurgery for pediatric craniopharyngioma is considered to be a safe and effective treatment after microsurgical tumor removal. In cases in which total removal is impossible, it is important to detach the tumor from the optic nerve and to reduce the tumor volume for gamma knife radiosurgery after microsurgery. If the tumor has a cystic component, this should be removed at the time of surgery. (author)

  4. Long-term effects of gamma knife radiosurgery for pediatric craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Osuka, Koji; Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki; Yoshida, Kazuo; Hasegawa, Toshinori [Komaki City Hospital, Aichi (Japan)

    2001-02-01

    Twenty-one pediatric patients with craniopharyngioma have undergone gamma knife radiosurgery at our facility since May 1991, and we have followed up 10 of these patients for at least 24 months (mean 44.5 months). The patients' ages ranged from 6 to 12 years (mean 8.6 years). Initial presentations were headache in 7 patients, decreased visual acuity in 8, visual field deficit in 5, hypopituitarism in 7, and diabetes insipidus in 3. Prior to radiosurgery, surgical procedures had been performed in 8 patients, conventional radiotherapy in 1, and chemotherapy in 1. Mean tumor diameter was 17.4 mm, and treatment was by gamma knife, with mean maximum and marginal doses of 25.4 Gy and 13.3 Gy, respectively. Follow-up MRI revealed good tumor control (partial response+complete response) in 7 of the 10 patients, with neither further neurological nor endocrinological deterioration. Two patients showed tumor shrinkage for 60 months and 46 months, respectively, after radiosurgery, while, thereafter, tumor gradually recurred at the side of the optic nerve. In one patient, rapid growth of the cyst was observed 6 months after radiosurgery. Gamma knife radiosurgery for pediatric craniopharyngioma is considered to be a safe and effective treatment after microsurgical tumor removal. In cases in which total removal is impossible, it is important to detach the tumor from the optic nerve and to reduce the tumor volume for gamma knife radiosurgery after microsurgery. If the tumor has a cystic component, this should be removed at the time of surgery. (author)

  5. Craniopharyngioma: the St. Jude Children's Research Hospital experience 1984-2001

    International Nuclear Information System (INIS)

    Merchant, Thomas E.; Kiehna, Erin N.; Sanford, Robert A.; Mulhern, Raymond K.; Thompson, Stephen J.; Wilson, Matthew W.; Lustig, Robert H.; Kun, Larry E.

    2002-01-01

    Purpose: To review our institution's experience in the treatment of craniopharyngioma and assess the merits of initial therapy with limited surgery and irradiation. Methods and Materials: The data of 30 patients (median age 8.6 years) with a diagnosis of craniopharyngioma between April 1984 and September 1997 were reviewed. Their course of treatment, neurologic, endocrine, and cognitive function, and quality of life at last follow-up were compared. Results: Fifteen patients were initially treated with surgery (8 required irradiation after relapse) and 15 with limited surgery and irradiation (2 required additional treatment for tumor progression). Only 1 patient died of tumor progression. The surgery group lost a mean of 9.8 points in full-scale IQ, and the combined-modality group lost only 1.25 points (p<0.063). Patients in the surgery group who had relapses (n=9) lost a mean of 13.1 points (p<0.067). A loss of 10 points was considered clinically significant. The surgery group also had more frequent neurologic, ophthalmic, and endocrine complications. The mean Health Utility Index (a functional quality-of-life index) was higher for the combined-modality group (0.85) than for the surgery group (0.71; p<0.063, one-sided t test). Conclusions: The acute neurologic, cognitive, and endocrine effects of surgery often affect long-term function and quality of life. Our experience suggests that limited surgery and radiotherapy cause lesser or comparable sequelae. Diabetes insipidus was the only endocrine deficiency that differed substantially in frequency between the two groups. Newer radiation planning and delivery techniques may make a combined-modality approach a good initial option for most patients

  6. Endocavitary treatment of craniopharyngioma cysts by 186-rhenium. Traitement endocavitaire par le rhenium 186 des kystes de craniopharyngiomes

    Energy Technology Data Exchange (ETDEWEB)

    Berenger, N.; Lebtahi, R.; Piketty, M.L.; Merienne, L.; Turak, B.; Bok, R.; Askienazy, S. (Hopital Sainte-Anne, 75 - Paris (France)); Munari, C. (Centre Hospitalier Universitaire, 38 - Grenoble (France))

    1993-01-01

    Forty-three patients with craniopharyngioma cysts were treated by intracystic injection of 186-rhenium. Leakage of colloid isotope into the CSF spaces during the ''test'' or ''therapeutic'' injection was detected by scintigraphic follow-up (15 cases/58 intracystic injections). In fact the physical characteristics of [sup 186]Re are well adapted to the requirements of treatment and, with the gamma emission, also allows early detection of leakage, avoiding irradiation of neighbouring structures. Follow-up studies revealed that craniopharyngioma cysts were effectively treated, with cessation of fluid formation, progressive shrinkage of the cysts leading to total disappearance in 14 cases (10-156 months, mean 52.5) and a considerable decrease in 13 cases (5-53 months, mean 23).

  7. Child cranio-pharyngioma: the benefit of proton-therapy; Craniopharyngiome de l'enfant: interet de la protontherapie

    Energy Technology Data Exchange (ETDEWEB)

    Alapetite, C.; Bolle, S. [Institut Curie, 75 - Paris (France); Alapetite, C.; Habrand, J.L.; Bolle, S.; Datchary, J.; Nauraye, C.; De Marzy, L. [Institut Curie Centre de protontherapie d' Orsay, 91 - Orsay (France); Puget, S.; Sainte Rose, C. [Hopital Necker-Enfants Malades, 75 - Paris (France); Habrand, J.L.; Datchary, J. [Institut Gustave-Roussy, 94 - Villejuif (France); Noel, G. [Centre Paul-Strauss, 67 - Strasbourg (France); Laffond, C. [Hopital national Saint-Maurice, Saint-Maurice (France)

    2010-10-15

    Based on a survey concerning 33 children suffering from cranio-pharyngiomas, the authors report a comparison of the dosimetry of different techniques (three-dimensional conformational radiotherapy, intensity-modulated conformation radiotherapy or IMRT, and proton therapy). They show that the ballistic peculiarities of proton beams result in a better protection of proximity critical structures and a reduction of the parenchyma total dose, and therefore in morbidity reduction without altering the local control rate. Short communication

  8. Frontal dermoid cyst coexisting with suprasellar craniopharyngioma: a spectrum of ectodermally derived epithelial-lined cystic lesions?

    Science.gov (United States)

    Abou-Al-Shaar, Hussam; Abd-El-Barr, Muhammad M; Zaidi, Hasan A; Russell-Goldman, Eleanor; Folkerth, Rebecca D; Laws, Edward R; Chiocca, E Antonio

    2016-12-01

    There is a wide group of lesions that may exist in the sellar and suprasellar regions. Embryologically, there is varying evidence that many of these entities may in fact represent a continuum of pathology deriving from a common ectodermal origin. The authors report a case of a concomitant suprasellar craniopharyngioma invading the third ventricle with a concurrent frontal lobe cystic dermoid tumor. A 21-year-old man presented to the authors' service with a 3-day history of worsening headache, nausea, vomiting, and blurry vision. Magnetic resonance imaging depicted a right frontal lobe lesion associated with a separate suprasellar cystic lesion invading the third ventricle. The patient underwent a right pterional craniotomy for resection of both lesions. Gross-total resection of the right frontal lesion was achieved, and subtotal resection of the suprasellar lesion was accomplished with some residual tumor adherent to the walls of the third ventricle. Histopathological examination of the resected right frontal lesion documented a diagnosis of dermoid cyst and, for the suprasellar lesion, a diagnosis of adamantinomatous craniopharyngioma. The occurrence of craniopharyngioma with dermoid cyst has not been reported in the literature before. Such an association might indeed suggest the previously reported hypothesis that these lesions represent a spectrum of ectodermally derived epithelial-lined cystic lesions.

  9. A case study of the neuropsychological outcomes following microsurgery, conventional radiotherapy and stereotactic radiotherapy for an adult's recurrent craniopharyngioma.

    Science.gov (United States)

    Preece, David; Allan, Alfred; Becerra, Rodrigo

    2016-01-01

    To examine the neuropsychological outcomes for an adult patient, 2 years after receiving microsurgery and conventional radiotherapy for a recurrent craniopharyngioma; and the impact of a further intervention, stereotactic radiotherapy, on this level of neuropsychological functioning. JD, a 30 year old male whose recurrent craniopharyngioma had 2 years earlier been treated with two operations and conventional radiotherapy. JD was assessed (using standardized clinical tests) before and after a course of stereotactic radiotherapy. Prior to stereotactic radiotherapy (and 2 years after microsurgery and conventional radiotherapy) JD's IQ was intact, but considerable impairments were present in executive functioning, memory, theory of mind and processing speed. Fifteen months after stereotactic radiotherapy, all neuropsychological domains remained largely static or improved, supporting the utility of this treatment option in the neuropsychological domain. However, deficits in executive functioning, memory and processing speed remained. These findings suggest that, even after multiple treatments, substantial cognitive impairments can be present in an adult patient with a recurrent craniopharyngioma. This profile of deficits underlines the inadequacy of relying purely on IQ as a marker for cognitive health in this population and emphasizes the need to include neuropsychological impairments as a focus of rehabilitation with these patients.

  10. Bypass surgery to treat symptomatic fusiform dilation of the internal carotid artery following craniopharyngioma resection: report of 2 cases.

    Science.gov (United States)

    Wang, Long; Shi, Xiang'en; Liu, Fangjun; Qian, Hai

    2016-12-01

    Fusiform dilation of the internal carotid artery (FDICA) is an infrequent vascular complication following resection of suprasellar lesions in the pediatric population, and its course appears to be benign without apparent clinical symptoms. However, data correlating symptomatic FDICA with bypass surgery are scarce. The authors here report 2 symptomatic cases that were treated using internal maxillary artery bypass more than 5 years after total removal of a craniopharyngioma at an outside institution. Both cases of FDICA were resected to relieve the mass effect and to expose the craniopharyngioma. The postoperative course was uneventful, and radiological imaging revealed graft conduit patency. To the authors' knowledge, this is the first reported use of extracranial to intracranial bypass to treat FDICA following removal of a suprasellar lesion. Their findings suggest that bypass surgery is a useful therapeutic approach for symptomatic cases of FDICA and total removal of recurrent craniopharyngioma. Moreover, the indications for surgical intervention and treatment modalities are discussed in the context of previous relevant cases.

  11. Sleeve gastrectomy leads to easy management of hormone replacement therapy and good weight loss in patients treated for craniopharyngioma.

    Science.gov (United States)

    Trotta, Manuela; Da Broi, Joël; Salerno, Angelo; Testa, Rosa M; Marinari, Giuseppe M

    2017-03-01

    The aim of this study is to investigate the effects of sleeve gastrectomy on hormone replacement therapy and on hypothalamic obesity in patients affected by craniopharyngioma with post-surgical pan-hypopituitarism. A retrospective review of three patients, treated for hypothalamic obesity with laparoscopic sleeve gastrectomy, who have previously undergone surgery for craniopharyngioma in their childhood, was done. Patients' mean age and BMI were 22.3 years (range 21-24) and 49.2 kg/m 2 (range 41.6-58.1), respectively. The mean time of delay between neurosurgery and bariatric surgery was 12.3 years (range 6-16). There were no major complications or deaths. At 24 months follow-up, the mean BMI was 35.3 kg/m 2 (range 31.2-40.6). No hydrocortisone and sex steroids dose changes were observed, while levothyroxine was decreased in two patients. Growth hormone replacement therapy was increased in two patients, whereas it was started in one patient. Desmopressin was significantly decreased in all of them. Patients with surgically induced pan-hypopituitarism after craniopharyngioma who become obese, can expect good results from sleeve gastrectomy: this procedure does not have significant negative effects on hormone substitution and leads to a good stabilization of body weight in a mid-term follow-up.

  12. Parent observed neuro-behavioral and pro-social improvements with oxytocin following surgical resection of craniopharyngioma.

    Science.gov (United States)

    Cook, Naomi; Miller, Jennifer; Hart, John

    2016-08-01

    Social and emotional impairment, school dysfunction, and neurobehavioral impairment are highly prevalent in survivors of childhood craniopharyngioma and negatively affect quality of life. As surgical resection of craniopharyngioma typically impairs hypothalamic/pituitary function, it has been postulated that perhaps post-operative deficiency of the hormone oxytocin may be the etiology of social/emotional impairment. Research on the benefits of oxytocin treatment as a hormone facilitating social interaction is well established. However, no research has yet been conducted on patients with known pituitary/hypothalamic dysfunction due to structural lesions or surgery. This case report investigates the effects of oxytocin therapy on a youngster with pituitary/hypothalamic dysfunction after craniopharyngioma removal. In this individual, treatment with low dose intranasal oxytocin resulted in increased desire for socialization and improvement in affection towards family. In light of these findings, the authors believe that further research into the potential benefits of intranasal oxytocin therapy for patients with panhypopituitarism is necessary to determine whether a broader population may also benefit from intranasal oxytocin therapy.

  13. Increased Daytime Sleepiness in Patients with Childhood Craniopharyngioma and Hypothalamic Tumor Involvement: Review of the Literature and Perspectives

    Directory of Open Access Journals (Sweden)

    Hermann L. Müller

    2010-01-01

    Full Text Available Childhood craniopharyngiomas are rare embryogenic malformations of the sellar region, presumably derived from Rathke cleft epithelium. The overall survival rates after neurosurgical intervention and/or irradiation are high (92%. However, the quality of survival is frequently impaired due to endocrine deficiencies, sleep disturbances, daytime sleepiness, and severe obesity caused by hypothalamic lesions. Based on self-assessment using nutritional diaries, caloric intake was similar in patients and BMI-matched controls. Analyses of physical activity by accelerometric measurements showed a markedly lower level of physical activity. Significant daytime sleepiness and disturbances of circadian rhythms have been demonstrated in obese childhood craniopharyngioma patients. Daytime sleepiness and obesity in these patients were both correlated with low nocturnal and early morning melatonin levels. Polysomnographic studies in patients with severe daytime sleepiness revealed sleeping patterns typical for secondary narcolepsy. Reports on a beneficial effect of treatment with central stimulating agents supported the hypothesis that secondary narcolepsy should be considered as a rare cause for severe daytime sleepiness in patients with childhood craniopharyngioma.

  14. Comparing survival outcomes of gross total resection and subtotal resection with radiotherapy for craniopharyngioma: a meta-analysis.

    Science.gov (United States)

    Wang, Guoqing; Zhang, Xiaoyang; Feng, Mengzhao; Guo, Fuyou

    2018-06-01

    Recent studies suggest that subtotal resection (STR) followed by radiation therapy (RT) is an appealing alternative to gross total resection (GTR) for craniopharyngioma, but it remains controversial. We conducted a meta-analysis to determine whether GTR is superior to STR with RT for craniopharyngioma. A systematic search was performed for articles published until October 2017 in the PubMed, Embase, and Cochrane Central databases. The endpoints of interest are overall survival and progression-free survival. Pooled hazard ratios (HRs) and corresponding 95% confidence intervals (CIs) were calculated using a fixed or random-effects model. The data were analyzed using Review Manager 5.3 software. A total of 744 patients (seven cohort studies) were enrolled for analyses. There were no significant differences between the GTR and STR with RT groups when the authors compared the pooled HRs at the end of the follow-up period. Overall survival (pooled HR = 0.76, 95% CI: 0.46-1.25, P = 0.28) and progression-free survival (pooled HR = 1.52, 95% CI: 0.42-5.44, P = 0.52) were similar between the two groups. The current meta-analysis suggests that GTR and STR with RT have the similar survival outcomes for craniopharyngioma. Copyright © 2018 Elsevier Inc. All rights reserved.

  15. Efficacy and safety of bariatric surgery for craniopharyngioma-related hypothalamic obesity: a matched case-control study with 2 years of follow-up.

    Science.gov (United States)

    Wijnen, M; Olsson, D S; van den Heuvel-Eibrink, M M; Wallenius, V; Janssen, J A M J L; Delhanty, P J D; van der Lely, A J; Johannsson, G; Neggers, S J C M M

    2017-02-01

    Hypothalamic obesity is a devastating consequence of craniopharyngioma. Bariatric surgery could be a promising therapeutic option. However, its efficacy and safety in patients with craniopharyngioma-related hypothalamic obesity remain largely unknown. We investigated the efficacy of bariatric surgery for inducing weight loss in patients with craniopharyngioma-related hypothalamic obesity. In addition, we studied the safety of bariatric surgery regarding its effects on hormone replacement therapy for pituitary insufficiency. In this retrospective matched case-control study, we compared weight loss after bariatric surgery (that is, Roux-en-Y gastric bypass and sleeve gastrectomy) between eight patients with craniopharyngioma-related hypothalamic obesity and 75 controls with 'common' obesity during 2 years of follow-up. We validated our results at 1 year of follow-up in a meta-analysis. In addition, we studied alterations in hormone replacement therapy after bariatric surgery in patients with craniopharyngioma. Mean weight loss after bariatric surgery was 19% vs 25% (difference -6%, 95% confidence of interval (CI) -14.1 to 4.6; P=0.091) at 2 years of follow-up in patients with craniopharyngioma-related hypothalamic obesity compared with control subjects with 'common' obesity. Mean weight loss was 25% vs 29% (difference -4%, 95% CI -11.6 to 8.1; P=0.419) after Roux-en-Y gastric bypass and 10% vs 20% (difference -10%, 95% CI -14.1 to -6.2; P=0.003) after sleeve gastrectomy at 2 years of follow-up in patients with craniopharyngioma-related hypothalamic obesity vs control subjects with 'common' obesity. Our meta-analysis demonstrated significant weight loss 1 year after Roux-en-Y gastric bypass, but not after sleeve gastrectomy. Seven patients with craniopharyngioma suffered from pituitary insufficiency; three of them required minor adjustments in hormone replacement therapy after bariatric surgery. Weight loss after Roux-en-Y gastric bypass, but not sleeve gastrectomy

  16. Urgent Optic Nerve Decompression via an Endoscopic Endonasal Transsphenoidal Approach for Craniopharyngioma in a 12-Month-Old Infant: A Case Report.

    Science.gov (United States)

    Shibata, Teishiki; Tanikawa, Motoki; Sakata, Tomohiro; Mase, Mitsuhito

    2018-01-01

    Craniopharyngiomas are benign tumors and account for approximately 5.6-13% of all intracranial tumors in children. Diagnosis of pediatric craniopharyngioma is often delayed until the tumor becomes relatively large and manifests severe visual and/or endocrine disturbance. Endoscopic endonasal approaches have recently been introduced to surgery for craniopharyngioma. These techniques, however, have rarely been utilized in patients affected with craniopharyngioma as young as 1 year old. This report documents a 12-month-old male infant with sellar craniopharyngioma who presented with acute total vision loss. To increase the chances of visual recovery, an endoscopic endonasal optic nerve decompression was performed as an urgent procedure. After decompression, which resulted in improvement of his visual disturbance, gross total resection of the tumor was undertaken through an anterior interhemispheric approach at a later date. Tumor mass reduction through an endoscopic endonasal transsphenoidal approach followed by secondary radical total resection under craniotomy was considered to be useful in cases such as this when urgent optic nerve decompression is required. © 2018 S. Karger AG, Basel.

  17. Efficacy of phosphorus-32 brachytherapy without external-beam radiation for long-term tumor control in patients with craniopharyngioma.

    Science.gov (United States)

    Ansari, Shaheryar F; Moore, Reilin J; Boaz, Joel C; Fulkerson, Daniel H

    2016-04-01

    OBJECT Radioactive phosphorus-32 (P32) has been used as brachytherapy for craniopharyngiomas with the hope of providing local control of enlarging tumor cysts. Brachytherapy has commonly been used as an adjunct to the standard treatment of surgery and external-beam radiation (EBR). Historically, multimodal treatment, including EBR, has shown tumor control rates as high as 70% at 10 years after treatment. However, EBR is associated with significant long-term risks, including visual deficits, endocrine dysfunction, and cognitive decline. Theoretically, brachytherapy may provide focused local radiation that controls or shrinks a symptomatic cyst without exposing the patient to the risks of EBR. For this study, the authors reviewed their experiences with craniopharyngioma patients treated with P32 brachytherapy as the primary treatment without EBR. The authors reviewed these patients' records to evaluate whether this strategy effectively controls tumor growth, thus avoiding the need for further surgery or EBR. METHODS The authors performed a retrospective review of pediatric patients treated for craniopharyngioma between 1997 and 2004. This was the time period during which the authors' institution had a relatively high use of P32 for treatment of cystic craniopharyngioma. All patients who had surgery and injection of P32 without EBR were identified. The patient records were analyzed for complications, cyst control, need for further surgery, and need for future EBR. RESULTS Thirty-eight patients were treated for craniopharyngioma during the study period. Nine patients (23.7%) were identified who had surgery (resection or biopsy) with P32 brachytherapy but without initial EBR. These 9 patients represented the study group. For 1 patient (11.1%), there was a complication with the brachytherapy procedure. Five patients (55.5%) required subsequent surgery. Seven patients (77.7%) required subsequent EBR for tumor growth. The mean time between the injection of P32 and

  18. Therapeutic outcomes of transsphenoidal surgery in pediatric patients with craniopharyngiomas: a single-center study.

    Science.gov (United States)

    Yamada, Shozo; Fukuhara, Noriaki; Yamaguchi-Okada, Mitsuo; Nishioka, Hiroshi; Takeshita, Akira; Takeuchi, Yasuhiro; Inoshita, Naoko; Ito, Junko

    2018-03-30

    OBJECTIVE The aim of this study was to analyze the outcomes of transsphenoidal surgery (TSS) in a single-center clinical series of pediatric craniopharyngioma patients treated with gross-total resection (GTR). METHODS The authors retrospectively reviewed the surgical outcomes for 65 consecutive patients with childhood craniopharyngiomas (28 girls and 37 boys, mean age 9.6 years) treated with TSS (45 primary and 20 repeat surgeries) between 1990 and 2015. Tumors were classified as subdiaphragmatic or supradiaphragmatic. Demographic and clinical characteristics, including extent of resection, complications, incidence of recurrence, pre- and postoperative visual disturbance, pituitary function, and incidence of diabetes insipidus (DI), as well as new-onset obesity, were analyzed and compared between the primary surgery and repeat surgery groups. RESULTS Of the 45 patients in the primary surgery group, 26 (58%) had subdiaphragmatic tumors and 19 had supradiaphragmatic tumors. Of the 20 patients in the repeat surgery group, 9 (45%) had subdiaphragmatic tumors and 11 had supradiaphragmatic tumors. The only statistically significant difference between the 2 surgical groups was in tumor size; tumors were larger (mean maximum diameter 30 mm) in the primary surgery group than in the repeat surgery group (25 mm) (p = 0.008). GTR was accomplished in 59 (91%) of the 65 cases; the GTR rate was higher in the primary surgery group than in the repeat surgery group (98% vs 75%, p = 0.009). Among the patients who underwent GTR, 12% experienced tumor recurrence, with a median follow-up of 7.8 years, and recurrence tended to occur less frequently in primary than in repeat surgery patients (7% vs 27%, p = 0.06). Of the 45 primary surgery patients, 80% had deteriorated pituitary function and 83% developed DI, whereas 100% of the repeat surgery patients developed these conditions. Among patients with preoperative visual disturbance, vision improved in 62% but worsened in 11%. Visual

  19. Endoscopic endonasal approach for craniopharyngioma: the importance of the relationship between pituitary stalk and tumor.

    Science.gov (United States)

    Dho, Yun-Sik; Kim, Yong Hwy; Se, Young-Bem; Han, Doo Hee; Kim, Jung Hee; Park, Chul-Kee; Wang, Kyu-Chang; Kim, Dong Gyu

    2017-09-29

    OBJECTIVE The endoscopic endonasal approach (EEA) is commonly used for the treatment of craniopharyngioma; therefore, it is essential to analyze outcomes in order to understand the benefits and drawbacks. The goal of this paper was to evaluate the clinical features and outcomes associated with this treatment approach. METHODS From July 2010 to March 2016, 82 adult craniopharyngioma patients underwent an EEA at the authors' institution. Of these cases, intraoperative records and immediate postoperative MR images were available for 68 patients. The patients underwent systemized endocrinological evaluation. Eighteen of 68 patients who underwent EEA for recurrence or regrowth of residual lesions after previous surgical management were excluded in the analysis of the anatomical tumor classification. The authors retrospectively analyzed preoperative clinical features and previous anatomical classifications, focusing on the relationship of the pituitary stalk and tumor, to determine predictive factors for the clinical outcome, such as the extent of resection, visual function, endocrinological function, recurrence rate, and complications. RESULTS The mean tumor size was 2.5 cm (3.1 cm for primary tumors and 1.9 cm for recurrent lesions). Gross-total resection (GTR) was achieved in 62 (91.1%) patients (48 [96.0%] patients with primary tumors and 14 [77.8%] patients with recurrent tumors). The rate of GTR was higher in the primary group than in the group with recurrence (p = 0.038). The overall pre- and postoperative visual impairment scale (VIS) scores were 40.8 and 22.1, respectively (50.9 and 14.3 in the primary group and 30.7 and 29.9 in patients with recurrence, respectively). The improvement rate in VIS score was higher in the primary group than in the recurrent group (p = 0.001). Endocrinological function was improved in 4 patients (5.9%) and deteriorated in 32 of 68 patients (47.1%). Tumor invasion into the center of the pituitary stalk affected the postoperative

  20. Neurocognitive functioning in pediatric craniopharyngioma: performance before treatment with proton therapy.

    Science.gov (United States)

    Fournier-Goodnight, Ashley S; Ashford, Jason M; Merchant, Thomas E; Boop, Frederick A; Indelicato, Daniel J; Wang, Lei; Zhang, Hui; Conklin, Heather M

    2017-08-01

    The goal of this study was to investigate the impact of patient-, disease-, and treatment-related variables upon neurocognitive outcomes in pediatric patients with craniopharyngioma prior to treatment with proton therapy or observation after radical resection. For all participants (N = 104), relevant clinical and demographic variables were attained and neurocognitive evaluations completed prior to irradiation or planned observation. One-sample t-tests were conducted to compare performance to published normative data. Linear models were used to investigate predictors of performance on measures where performance was below normative expectations. Participants showed poorer performance in comparison to the normative group across neurocognitive domains including executive functions (e.g., working memory; Wechsler Digit Span Backward p = 0.03), learning and memory (e.g., California Verbal Learning Test [CVLT] Total T p = 0.00), and fine-motor coordination (e.g., Grooved Pegboard Dominant Hand p = 0.00). Poor performance across areas was predicted by presurgical hypothalamic involvement (e.g., Behavior Rating Inventory of Executive Function Working Memory Index Grade 2 β = -7.68, p = 0.03; CVLT Total T Grade 2 β = 7.94, p = 0.04; Grade 3 β = -9.80, p = 0.00), extent of surgery (e.g., CVLT Total T Resection β = -7.77, p = 0.04; Grooved Pegboard Dominant Hand β = -1.58, p = 0.04), and vision status (e.g., CVLT Total T Reduced vision without impairment β = -10.01, p = 0.02; Grooved Pegboard Dominant Hand Bilateral field defect β = -1.45, p = 0.01; Reduced vision without impairment β = -2.30, p = 0.00). This study demonstrated that patients with craniopharyngioma show weaker neurocognitive performance in comparison to the normative population resulting from tumor, events leading to diagnosis, and early surgical intervention. Systematic investigation of neurocognitive performance before

  1. Molecular Analyses Reveal Inflammatory Mediators in the Solid Component and Cyst Fluid of Human Adamantinomatous Craniopharyngioma.

    Science.gov (United States)

    Donson, Andrew M; Apps, John; Griesinger, Andrea M; Amani, Vladimir; Witt, Davis A; Anderson, Richard C E; Niazi, Toba N; Grant, Gerald; Souweidane, Mark; Johnston, James M; Jackson, Eric M; Kleinschmidt-DeMasters, Bette K; Handler, Michael H; Tan, Aik-Choon; Gore, Lia; Virasami, Alex; Gonzalez-Meljem, Jose Mario; Jacques, Thomas S; Martinez-Barbera, Juan Pedro; Foreman, Nicholas K; Hankinson, Todd C

    2017-09-01

    Pediatric adamantinomatous craniopharyngioma (ACP) is a highly solid and cystic tumor, often causing substantial damage to critical neuroendocrine structures such as the hypothalamus, pituitary gland, and optic apparatus. Paracrine signaling mechanisms driving tumor behavior have been hypothesized, with IL-6R overexpression identified as a potential therapeutic target. To identify potential novel therapies, we characterized inflammatory and immunomodulatory factors in ACP cyst fluid and solid tumor components. Cytometric bead analysis revealed a highly pro-inflammatory cytokine pattern in fluid from ACP compared to fluids from another cystic pediatric brain tumor, pilocytic astrocytoma. Cytokines and chemokines with particularly elevated concentrations in ACPs were IL-6, CXCL1 (GRO), CXCL8 (IL-8) and the immunosuppressive cytokine IL-10. These data were concordant with solid tumor compartment transcriptomic data from a larger cohort of ACPs, other pediatric brain tumors and normal brain. The majority of receptors for these cytokines and chemokines were also over-expressed in ACPs. In addition to IL-10, the established immunosuppressive factor IDO-1 was overexpressed by ACPs at the mRNA and protein levels. These data indicate that ACP cyst fluids and solid tumor components are characterized by an inflammatory cytokine and chemokine expression pattern. Further study regarding selective cytokine blockade may inform novel therapeutic interventions. © 2017 American Association of Neuropathologists, Inc. All rights reserved.

  2. Usefulness and problems of radiotherapy for craniopharyngioma in <10 years old patients

    Energy Technology Data Exchange (ETDEWEB)

    Ono, Nobuo; Fujimaki, Hiroya; Koga, Hideaki; Inoue, Hiroshi [Gunma Univ., Maebashi (Japan). School of Medicine

    1997-01-01

    There are many problems in radiotherapy for pediatric patients with craniopharyngioma since it injures immature brain. The usefulness and problems in the therapy were evaluated. Subjects were 13 patients (8 males and 5 females) aged 2-9 y (mean 5.4 y) who received partial extraction of the tumor and then radiotherapy for residual tumor. A-group (8 cases) received linac irradiation at 2 Gy in total dose of 50-60 (mean 53) Gy and B-group received gamma knife therapy with the central and marginal dose of 20-34 (mean 27) Gy and of 10 Gy, respectively. The mean follow-up period was 9.6 years (A-group 13.7 y and B-group 2.4 y) with observations on imaging, clinical change, sight and visual field, and endocrinological tests. In A- and B-group, recurrence based on imaging was seen in 5/8 cases and in 3/5 cases, respectively. Statistical significance was observed in lowering IQ in A-group and in improvement of Karnofsky score in B-group. Endocrinological improvement was marked in A-group. Radiosurgery was thus useful. (K.H.)

  3. Oxytocin and Naltrexone Successfully Treat Hypothalamic Obesity in a Boy Post-Craniopharyngioma Resection.

    Science.gov (United States)

    Hsu, Eugenie A; Miller, Jennifer L; Perez, Francisco A; Roth, Christian L

    2018-02-01

    Hypothalamic obesity, a treatment-resistant condition common to survivors of craniopharyngioma (CP), is strongly associated with a poor quality of life in this population. Oxytocin (OT), a hypothalamic neuropeptide, has been shown to play a role in the regulation of energy balance and to have anorexigenic effects in animal studies. Naltrexone (NAL), an opiate antagonist, has been shown to deter hedonic eating and to potentiate OT's effects. In this parent-observed study, we tested the administration of intranasal OT for 10 weeks (phase 1), followed by a combination of intranasal OT and NAL for 38 weeks (phase 2) in a 13-year-old male with confirmed hypothalamic obesity and hyperphagia post-CP resection. Treatment resulted in 1) reduction in body mass index (BMI) z score from 1.77 to 1.49 over 10 weeks during phase 1; 2) reduction in BMI z score from 1.49 to 0.82 over 38 weeks during phase 2; 3) reduced hyperphagia during phases 1 and 2; 4) continued hedonic high-carbohydrate food-seeking in the absence of hunger during phases 1 and 2; and 5) sustained weight reduction during decreased parental monitoring and free access to unlocked food in the home during the last 10 weeks of phase 2. This successful intervention of CP-related hypothalamic obesity and hyperphagia by OT alone and in combination with NAL is promising for conducting future studies of this treatment-recalcitrant form of obesity. Copyright © 2017 Endocrine Society

  4. A juvenile case of radiation-induced meningioma two years after radiation for craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Kano, Tomoaki; Zama, Akira; Ono, Nobuo; Nakamura, Tadashi; Tamura, Masaru; Ohe, Tihiro; Nakazato, Yoichi (Gunma Univ., Maebashi (Japan). School of Medicine)

    1994-04-01

    The patient was a 7-years-old boy who received radiation therapy of 50 Gy after total gross removal of a craniopharyngioma. After two years a follow up CT scan showed a new enhanced lesion in the right temporal tip within the previous irradiation field. Total removal of the tumor was performed and its histological examination showed it to be an atypical meningioma. This atypical meningioma satisfied Cahan's criteria. So we diagnosed this atypical meningioma as radiation-induced meningioma. Immunohistochemically this meningioma stained for Vimentin. An electron microscopical examination showed neither desmosome nor interdigitation. The score of Ki-67 and BrdU-L. I was very small. Compared with previously reported juvenile radiation-induced meningioma, the latency was very short. The patient received growth hormone (GH) replacement therapy. We suspected relation between GH replacement therapy and short latency. He was discharged without any new neurological deficits and we haven't detected tumor recurrence for two years. (author).

  5. Clinical analysis of treatment and long-term outcome in 56 craniopharyngiomas

    International Nuclear Information System (INIS)

    Tanaka, Yuichiro; Takemae, Toshiki; Kobayashi, Shigeaki

    2007-01-01

    In order to improve quality of life (QOL) in patients with craniopharyngioma, retrospective analysis was performed to discover factors for influencing functional outcome and the best treatment strategy. 56 patients were treated between 1978 and 2005. They were initially treated with surgery and surgical cure was obtained in 29 patients (Group A). Subtotal tumor resection with prophylactic radiation was performed in 8 patients (Group D). Tumor recurrence was noted in 19 patients and stereotactic radiation or 2nd operation was performed in 11 patients (Group B). 2nd operation and fractionated radiation were undertaken in 8 patients (Group C). Endocrine, vision and recognition were scored from 2 to 0, respectively; 2 indicates normal, 1 partially disturbed, 0 fully disturbed. The mean score before treatment was 4.7 and the final score was 3.9. Factors leading to poor outcome included extrasellar origin, solid tumor, bad score before treatment, 2nd surgery for recurrence. The change of scores after the treatment was -0.1 in Group A, -0.7 in Group B, -0.9 in Group C, and 0.3 in Group D. Maximum, tumor removal should be attempted with functional preservation. Subtotal removal with prophylactic radiation is recommended if the patient has normal hypothalamic function. (author)

  6. Dosimetry for the treatment of cystic craniopharyngioma through radiocolloids with 186 Re

    International Nuclear Information System (INIS)

    Rojas, E.L.; Al-dweri, F.M.O.; Lallena, A.M.; Bodineau, C.; Galan, P.

    2003-01-01

    The cystic craniopharyngioma (CQ) are histologically benign tumors that can affect important organs, as the hypothalamus or the optic nerve. They are treated introducing, inside the cyst, a radioactive colloid. The wall constitutes the white volume and the dosimetry is usually carried out starting from analytic formulas that only are valid in the case of an homogeneous media, uniform and infinite. In this work it is studied the dosimetry of the CQ by means of Monte Carlo simulation (MC), taking in account the different materials and interfaces that conform them. We present the obtained results for the radionuclide 186 Re. We have used the Monte Carlo code Penelope and we follow 5x10 6 histories in each simulation. We supposed a size of CQ of 1.75 cm of radio and a wall thickness of 1 mm and we have varied the constituent materials of the interior of the cyst and of the wall. The analytic calculations that we carry out show an excellent agreement with the MC results for an unique media (water), as much for the beta radiation as for those originated of the Rhenium disintegration. However, when it takes in account the gel that is introduced in the tumor for the treatment and we vary the constituent material of the wall, we find important differences. Of the analysis of our results we can conclude that the dosimetry for the treatment of CQ based in the usual analytic formulas overestimates the doses really deposited in the wall of the CQ. (Author)

  7. Hypothalamic obesity after treatment for craniopharyngioma: the importance of the home environment.

    Science.gov (United States)

    Meijneke, Ruud W H; Schouten-van Meeteren, Antoinette Y N; de Boer, Nienke Y; van Zundert, Suzanne; van Trotsenburg, Paul A S; Stoelinga, Femke; van Santen, Hanneke M

    2015-01-01

    Hypothalamic obesity after treatment for craniopharyngioma is a well-recognized, severe problem. Treatment of hypothalamic obesity is difficult and often frustrating for the patient, the parents and the professional care-giver. Because hypothalamic obesity is caused by an underlying medical disorder, it is often assumed that regular diet and exercise are not beneficial to reduce the extraordinarily high body mass index, and in fact, lifestyle interventions have been shown to be insufficient in case of extreme hypothalamic obesity. Nevertheless, it is important to realize that also in this situation, informal care delivered by the family and appropriate parenting styles are required to minimize the obesity problem. We present a case in which weight gain in the home situation was considered unstoppable, and a very early mortality due to complications of the severe increasing obesity was considered inevitable. A permissive approach toward food intake became leading with rapid weight increase since a restrictive lifestyle was considered a senseless burden for the child. By admission to our hospital for a longer period of time, weight reduction was realized, and the merely permissive approach could be changed into active purposeful care by adequate information, instruction, guidance and encouragement of the affected child and her parents. This case illustrates that, although this type of obesity has a pathological origin, parental and environmental influences remain of extreme importance.

  8. A Cross-Sectional Cohort Study of Cerebrovascular Disease and Late Effects After Radiation Therapy for Craniopharyngioma.

    Science.gov (United States)

    Lo, Andrea C; Howard, A Fuchsia; Nichol, Alan; Hasan, Haroon; Martin, Monty; Heran, Manraj; Goddard, Karen

    2016-05-01

    The study objective was to describe radiation-induced vascular abnormalities, stroke prevalence, and stroke risk factors in survivors of childhood craniopharyngioma. Twenty survivors of childhood craniopharyngioma who received radiotherapy (RT) were included in the study. A clinical history, quality of life assessment, cognitive functioning assessment, magnetic resonance angiogram or computed tomography angiogram, fasting lipid profile, and fasting glucose or hemoglobin A1c test were obtained. Median age at diagnosis was 10.3 years and median age at time of study was 29.0 years. Vascular abnormalities were detected in six (32%) of 19 patients' angiograms (vascular stenosis, decreased artery size, aneurysm, cavernoma, and small vessel disease). Five (25%) of 20 patients experienced a stroke after RT. Median time since RT was 27.8 versus 9.1 years in patients with versus without vascular abnormalities (P = 0.02). A low level of high-density lipoproteiin (HDL) was present in 100% (5/5) of patients who had a post-RT stroke as compared with 13% (2/15) of patients who did not have any post-RT stroke (P = 0.02). Previous stroke had occurred in 0% (0/5) of patients receiving growth hormone (GH) replacement at the time of study, compared to 40% (6/15) of patients who were not receiving GH replacement (P = 0.09). Patients with craniopharyngioma treated with RT have a high prevalence of stroke and vascular abnormalities, particularly those with low HDL and longer duration of time since RT. There is a trend to suggest that continual GH replacement may reduce the risk of stroke. These patients should undergo careful monitoring and aggressive modification of stroke risk factors. © 2016 Wiley Periodicals, Inc.

  9. Nutritional assessment of a population with a history of childhood craniopharyngioma seen at Hospital "Prof. Dr. Juan P. Garrahan".

    Science.gov (United States)

    Caminiti, Carolina; Saure, Carola; Bomer, Ilanit; Brea, Mercedes; González Ramos, Javier

    2017-02-01

    Craniopharyngiomas are histologically benign malformations located between the pituitary and hypothalamus that may affect key hormone secretion for endocrine regulation and satiety modulation. Although this is a relatively benign disease, the combination of severe hypothalamic obesity and associated comorbidities results in a reduced quality of life. To assess the nutritional status of patients after craniopharyngioma surgery. Patients younger than 21 years old at the time of the study who required craniopharyngioma surgery at Hospital de Pediatr.a Garrahan and who signed an informed consent. Anthropometric characteristics, body composition by impedance analysis, energy expenditure by indirect calorimetry and energy intake were assessed. Insulin resistance and dyslipemia were estimated. A total of 39 patients were included; 41% had a normal weight and 59% were obese. Overall, 68% of patients had a central fat distribution; 40% had insulin resistance; and 32%, dyslipemia. No significant differences were observed in terms of insulin resistance, dyslipemia, energy expenditure at rest, or energy intake between normal weight and obese patients. Among obese patients, 77% had a low energy expenditure, regardless of their percentage of lean body mass (62 Å} 2.7% versus 61.2 Å} 1.8% of normal versus low energy expenditure at rest; p = 0.8). A total of 59% of the studied population was obese. No significant differences were observed in terms of metabolic complications between normal weight and obese patients. A lower energy expenditure was observed, regardless of the lean body mass percentage and a similar energy intake. Sociedad Argentina de Pediatría

  10. Superficial Siderosis of the Central Nervous System Caused by Hemorrhagic Intraventricular Craniopharyngioma: Case Report and Literature Review

    Science.gov (United States)

    TOSAKA, Masahiko; SATO, Koji; AMANUMA, Makoto; HIGUCHI, Tetsuya; ARAI, Motohiro; AISHIMA, Kaoru; SHIMIZU, Tatsuya; HORIGUCHI, Keishi; SUGAWARA, Kenichi; YOSHIMOTO, Yuhei

    2015-01-01

    Superficial siderosis is a rare condition caused by hemosiderin deposits in the central nervous system (CNS) due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). CNS tumor could be one of the sources of bleeding, both pre- and postoperatively. We report an extremely rare case of superficial siderosis associated with purely third ventricle craniopharyngioma, and review previously reported cases of superficial siderosis associated with CNS tumor. A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. Brain magnetic resonance (MR) imaging with gadolinium revealed a well enhanced, intraventricular mass in the anterior part of the third ventricle. T2*-weighted gradient echo (GE) MR imaging revealed a hypointense rim around the brain particularly marked within the depth of the sulci. Superficial siderosis was diagnosed based on these findings. The tumor was diffusely hypointense on T2*-weighted GE imaging, indicating intratumoral hemorrhage. The lateral ventricles were dilated, suggesting hydrocephalus. [18F]fluorodeoxyglucose positron emission tomography revealed increased uptake in the tumor. The whole brain surface appeared dark ocher at surgery. Histological examination showed the hemorrhagic tumor was papillary craniopharyngioma. His hearing loss progressed after removal of the tumor. T2*-weighted GE MR imaging demonstrated not only superficial siderosis but also diffuse intratumoral hemorrhage in the tumor. Superficial siderosis and its related symptoms, including hearing loss, should be considered in patients with hemorrhagic tumor related to the CSF space. Purely third ventricle craniopharyngioma rarely has hemorrhagic character, which could cause superficial siderosis and progressive hearing loss. PMID:24670310

  11. Effects of T3 treatment on brown adipose tissue and energy expenditure in a patient with craniopharyngioma and hypothalamic obesity.

    Science.gov (United States)

    van Santen, Hanneke M; Schouten-Meeteren, Antoinette Y; Serlie, Mireille; Meijneke, Ruud W H; van Trotsenburg, A S; Verberne, Hein; Holleman, Frits; Fliers, Eric

    2015-01-01

    Patients treated for childhood craniopharyngioma often develop hypothalamic obesity (HO), which has a huge impact on the physical condition and quality of life of these patients. Treatment for HO thus far has been disappointing, and although several different strategies have been attempted, all interventions had only transient effects. Since thyroid hormones increase energy expenditure metabolism (thyroid hormone induced thermogenesis), it was speculated that treatment with tri-iodothyronine (T3) may be beneficial. In 2002, a case report was published on reduction of body weight after T3 treatment for HO. No studies have been reported since. Recent experimental studies in rodents showed that T3 increases brown adipose tissue (BAT) activity via (pre)sympathetic pathways between the hypothalamus and BAT. Our aim was to investigate whether T3 treatment increases BAT activity in a patient with HO resulting from (treatment of) childhood craniopharyngioma. Thyroxine treatment for central hypothyroidism was switched to T3 monotherapy. Serum T3 and free thyroxine (FT4) concentrations were measured twice weekly for 2 months. ¹²³I-MIBG and ¹⁸F-FDG-PET after induction of non-shivering thermogenesis for the assessment of sympathetic and metabolic activity of BAT as well as indirect calorimetry for assessment of resting energy expenditure were performed before and during T3 treatment. No change in sympathetic and metabolic BAT activity, energy expenditure, or BMI was seen during T3 treatment despite the expected changes in thyroid hormone plasma concentrations. We conclude that T3 monotherapy does not seem to be effective in decreasing HO in childhood craniopharyngioma.

  12. Prevalence of Neurobehavioral, Social, and Emotional Dysfunction in Patients Treated for Childhood Craniopharyngioma: A Systematic Literature Review

    Science.gov (United States)

    Zada, Gabriel; Kintz, Natalie; Pulido, Mario; Amezcua, Lilyana

    2013-01-01

    Background Craniopharyngiomas (CP) are locally invasive and frequently recurring neoplasms often resulting in neurological and endocrinological dysfunction in children. In addition, social-behavioral impairment is commonly reported following treatment for childhood CP, yet remains to be fully understood. The authors aimed to further characterize the prevalence of neurobehavioral, social, and emotional dysfunction in survivors of childhood craniopharyngiomas. Materials and Methods A systematic literature review was conducted in PubMed to identify studies formally assessing neurobehavioral, social, and emotional outcomes in patients treated for CP prior to 18 years of age. Studies published between the years 1990-2012 that reported the primary outcome (prevalence of neurobehavioral, social, emotional/affective dysfunction, and/or impaired quality of life (QoL)) in ≥10 patients were included. Results Of the 471 studies screened, 11 met inclusion criteria. Overall neurobehavioral dysfunction was reported in 51 of 90 patients (57%) with available data. Social impairment (i.e. withdrawal, internalizing behavior) was reported in 91 of 222 cases (41%). School dysfunction was reported in 48 of 136 patients (35%). Emotional/affective dysfunction was reported in 58 of 146 patients (40%), primarily consisting of depressive symptoms. Health related quality of life was affected in 49 of 95 patients (52%). Common descriptors of behavior in affected children included irritability, impulsivity, aggressiveness, and emotional outbursts. Conclusions Neurobehavioral, social, and emotional impairment is highly prevalent in survivors of childhood craniopharyngioma, and often affects quality of life. Thorough neurobehavioral/emotional screening and appropriate counseling is recommended in this population. Additional research is warranted to identify risk factors and treatment strategies for these disorders. PMID:24223703

  13. Review of management and morbidity of pediatric craniopharyngioma patients in a low-middle-income country: a 12-year experience.

    Science.gov (United States)

    Amayiri, Nisreen; Swaidan, Maisa; Yousef, Yocoub; Halalsheh, Hadeel; Abu-Hijlih, Ramiz; Kalaldeh, Sima; Barbar, Maha; Elayyan, Maher; Faqih, Nesreen; Al-Hussaini, Maysa; Mehyar, Mustafa; Bartels, Ute; Drake, James; Musharbash, Awni; Bouffet, Eric

    2017-06-01

    Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few. The study provides a retrospective chart review of pediatric patients craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan. Twenty-four patients (12 males) were identified. Median age at diagnosis was 7.4 years (0.9-16.4 years). Commonest symptoms were visual impairment and headache (71%). Review of seventeen preoperative MRIs showed hypothalamic involvement in 88% and hydrocephalus in 76%. Thirteen patients (54%) had multiple surgical interventions. Five patients (21%) had initial gross total resection. Eleven patients (46%) received radiotherapy and six (25%) intra-cystic interferon. Five years' survival was 87 ± 7% with a median follow-up of 4.5 years (0.3-12.3 years). Four patients (17%) died; one after post-operative cerebral infarction and three secondary to hypothalamic damage. At their last evaluation, all but one patient required multiple hormonal supplements. Ten patients (42%) had best eye visual acuity (VA) >20/40, and four (16%) were legally blind. Eleven patients (46%) were overweight/obese; one had gastric bypass surgery. Seven patients had hyperlipidemia, and eight developed fatty liver infiltration. Eleven patients (65%) were attending schools and one at college. Nine of the living patients (53%) expressed difficulty to engage in the community. Management of pediatric craniopharyngioma is particularly complex and demanding in LMIC. Multidisciplinary care is integral to optimize the care and minimize the morbidities. A management outline for LMIC is proposed.

  14. SU-E-J-122: Detecting Treatment-Induced Metabolic Abnormalities in Craniopharyngioma Patients Undergoing Surgery and Proton Therapy

    Energy Technology Data Exchange (ETDEWEB)

    Hua, C; Shulkin, B; Li, Y; LI, X; Merchant, T [St. Jude Children' s Research Hospital, Memphis, TN (United States); Indelicato, D [University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Boop, F [Semmes-Murphey Neurologic and Spine Institute, Memphis, TN (United States)

    2014-06-01

    Purpose: To identify treatment-induced defects in the brain of children with craniopharyngioma receiving surgery and proton therapy using fluorodeoxyglucose positron emission tomography (FDG PET). Methods: Forty seven patients were enrolled on a clinical trial for craniopharyngioma with serial imaging and functional evaluations. Proton therapy was delivered using the double-scattered beams with a prescribed dose of 54 Cobalt Gray Equivalent. FDG tracer uptake in each of 63 anatomical regions was computed after warping PET images to a 3D reference template in Talairach coordinates. Regional uptake was deemed significantly low or high if exceeding two standard deviations of normal population from the mean. For establishing the normal ranges, 132 children aged 1–20 years with noncentral nervous system related diseases and normal-appearing cerebral PET scans were analyzed. Age- and gender-dependent regional uptake models were developed by linear regression and confidence intervals were calculated. Results: Most common PET abnormality before proton therapy was significantly low uptake in the frontal lobe, the occipital lobe (particularly in cuneus), the medial and ventral temporal lobe, cingulate gyrus, caudate nuclei, and thalamus. They were related to injury from surgical corridors, tumor mass effect, insertion of a ventricular catheter, and the placement of an Ommaya reservoir. Surprisingly a significantly high uptake was observed in temporal gyri and the parietal lobe. In 13 patients who already completed 18-month PET scans, metabolic abnormalities improved in 11 patients from baseline. One patient had persistent abnormalities. Only one revealed new uptake abnormalities in thalamus, brainstem, cerebellum, and insula. Conclusion: Postoperative FDG PET of craniopharyngioma patients revealed metabolic abnormalities in specific regions of the brain. Proton therapy did not appear to exacerbate these surgery- and tumor-induced defects. In patients with persistent and

  15. Prevalence of neurobehavioral, social, and emotional dysfunction in patients treated for childhood craniopharyngioma: a systematic literature review.

    Directory of Open Access Journals (Sweden)

    Gabriel Zada

    Full Text Available Craniopharyngiomas (CP are locally invasive and frequently recurring neoplasms often resulting in neurological and endocrinological dysfunction in children. In addition, social-behavioral impairment is commonly reported following treatment for childhood CP, yet remains to be fully understood. The authors aimed to further characterize the prevalence of neurobehavioral, social, and emotional dysfunction in survivors of childhood craniopharyngiomas.A systematic literature review was conducted in PubMed to identify studies formally assessing neurobehavioral, social, and emotional outcomes in patients treated for CP prior to 18 years of age. Studies published between the years 1990-2012 that reported the primary outcome (prevalence of neurobehavioral, social, emotional/affective dysfunction, and/or impaired quality of life (QoL in ≥ 10 patients were included.Of the 471 studies screened, 11 met inclusion criteria. Overall neurobehavioral dysfunction was reported in 51 of 90 patients (57% with available data. Social impairment (i.e. withdrawal, internalizing behavior was reported in 91 of 222 cases (41%. School dysfunction was reported in 48 of 136 patients (35%. Emotional/affective dysfunction was reported in 58 of 146 patients (40%, primarily consisting of depressive symptoms. Health related quality of life was affected in 49 of 95 patients (52%. Common descriptors of behavior in affected children included irritability, impulsivity, aggressiveness, and emotional outbursts.Neurobehavioral, social, and emotional impairment is highly prevalent in survivors of childhood craniopharyngioma, and often affects quality of life. Thorough neurobehavioral/emotional screening and appropriate counseling is recommended in this population. Additional research is warranted to identify risk factors and treatment strategies for these disorders.

  16. SU-E-J-122: Detecting Treatment-Induced Metabolic Abnormalities in Craniopharyngioma Patients Undergoing Surgery and Proton Therapy

    International Nuclear Information System (INIS)

    Hua, C; Shulkin, B; Li, Y; LI, X; Merchant, T; Indelicato, D; Boop, F

    2014-01-01

    Purpose: To identify treatment-induced defects in the brain of children with craniopharyngioma receiving surgery and proton therapy using fluorodeoxyglucose positron emission tomography (FDG PET). Methods: Forty seven patients were enrolled on a clinical trial for craniopharyngioma with serial imaging and functional evaluations. Proton therapy was delivered using the double-scattered beams with a prescribed dose of 54 Cobalt Gray Equivalent. FDG tracer uptake in each of 63 anatomical regions was computed after warping PET images to a 3D reference template in Talairach coordinates. Regional uptake was deemed significantly low or high if exceeding two standard deviations of normal population from the mean. For establishing the normal ranges, 132 children aged 1–20 years with noncentral nervous system related diseases and normal-appearing cerebral PET scans were analyzed. Age- and gender-dependent regional uptake models were developed by linear regression and confidence intervals were calculated. Results: Most common PET abnormality before proton therapy was significantly low uptake in the frontal lobe, the occipital lobe (particularly in cuneus), the medial and ventral temporal lobe, cingulate gyrus, caudate nuclei, and thalamus. They were related to injury from surgical corridors, tumor mass effect, insertion of a ventricular catheter, and the placement of an Ommaya reservoir. Surprisingly a significantly high uptake was observed in temporal gyri and the parietal lobe. In 13 patients who already completed 18-month PET scans, metabolic abnormalities improved in 11 patients from baseline. One patient had persistent abnormalities. Only one revealed new uptake abnormalities in thalamus, brainstem, cerebellum, and insula. Conclusion: Postoperative FDG PET of craniopharyngioma patients revealed metabolic abnormalities in specific regions of the brain. Proton therapy did not appear to exacerbate these surgery- and tumor-induced defects. In patients with persistent and

  17. Comparative immunohistochemical expression of β-catenin, EGFR, ErbB2, and p63 in adamantinomatous and papillary craniopharyngiomas

    International Nuclear Information System (INIS)

    Eshebaa, Gh.E.; Hassan, A.A.

    2015-01-01

    Craniopharyngiomas (CPs) are rare epithelial tumors located mainly in the sellar/parasellar region. CPs have been classified into histopathologically, genetically, clinically and prognostically two distinctive subtypes: adamantinomatous and papillary variants. Aim To determine the immunohistochemical expression of β-catenin, EGFR, ErbB2, and p63 in adamantinomatous and papillary CPs. Materials and methods β-Catenin, EGFR, ErbB2, and p63 immunostaining was performed on paraffin embedded tissue sections of 25 CPs including 18 adamantinomatous craniopharyngioma (ACP) and 7 cases of papillary craniopharyngiomas (PCPs). Results 17 cases (94%) of ACP exhibited strong nuclear/cytoplasmic expression of β-catenin. On the contrary, all cases of PCP showed exclusively membranous expression (ρ value <0.0001). Regarding EGFR, 15 (83%) and 5 cases (71%) of APC and PCP respectively were positive. On the other hand, only 3 cases (17%) of APC and none of PCP exhibited positivity for ErbB2. p63 over-expression was observed in 16 cases of ACP (89%) and 6 cases of PCP (86%). However, the distribution of p63 staining was diffuse in ACP, while in PCP; the staining was mainly restricted to the basal cell layer. Conclusion Nuclear accumulation of β-catenin is a diagnostic hallmark of the ACP and is very helpful in the differential diagnosis between both ACP and PCP in the setting of small biopsies. Moreover, the restricted nuclear β-catenin accumulation in the cohesive cell clusters within the whorl-like areas supports that aberrant β-catenin expression may play a role in the morphogenesis of ACP.

  18. Intracystic interferon-alpha in pediatric craniopharyngioma patients: an international multicenter assessment on behalf of SIOPE and ISPN.

    Science.gov (United States)

    Kilday, John-Paul; Caldarelli, Massimo; Massimi, Luca; Chen, Robert Hsin-Hung; Lee, Yi Yen; Liang, Muh-Lii; Parkes, Jeanette; Naiker, Thuran; van Veelen, Marie-Lise; Michiels, Erna; Mallucci, Conor; Pettorini, Benedetta; Meijer, Lisethe; Dorfer, Christian; Czech, Thomas; Diezi, Manuel; Schouten-van Meeteren, Antoinette Y N; Holm, Stefan; Gustavsson, Bengt; Benesch, Martin; Müller, Hermann L; Hoffmann, Anika; Rutkowski, Stefan; Flitsch, Joerg; Escherich, Gabriele; Grotzer, Michael; Spoudeas, Helen A; Azquikina, Kristian; Capra, Michael; Jiménez-Guerra, Rolando; MacDonald, Patrick; Johnston, Donna L; Dvir, Rina; Constantini, Shlomi; Kuo, Meng-Fai; Yang, Shih-Hung; Bartels, Ute

    2017-10-01

    Craniopharyngiomas are frequent hypothalamo-pituitary tumors in children, presenting predominantly as cystic lesions. Morbidity from conventional treatment has focused attention on intracystic drug delivery, hypothesized to cause fewer clinical consequences. However, the efficacy of intracystic therapy remains unclear. We report the retrospective experiences of several global centers using intracystic interferon-alpha. European Société Internationale d'Oncologie Pédiatrique and International Society for Pediatric Neurosurgery centers were contacted to submit a datasheet capturing pediatric patients with cystic craniopharyngiomas who had received intracystic interferon-alpha. Patient demographics, administration schedules, adverse events, and outcomes were obtained. Progression was clinical or radiological (cyst reaccumulation, novel cysts, or solid growth). Fifty-six children (median age, 6.3 y) from 21 international centers were identified. Median follow-up from diagnosis was 5.1 years (0.3-17.7 y). Lesions were cystic (n = 22; 39%) or cystic/solid (n = 34; 61%). Previous progression was treated in 43 (77%) patients before interferon use. In such cases, further progression was delayed by intracystic interferon compared with the preceding therapy for cystic lesions (P = 0.0005). Few significant attributable side effects were reported. Progression post interferon occurred in 42 patients (median 14 mo; 0-8 y), while the estimated median time to definitive therapy post interferon was 5.8 (1.8-9.7) years. Intracystic interferon-alpha can delay disease progression and potentially offer a protracted time to definitive surgery or radiotherapy in pediatric cystic craniopharyngioma, yet demonstrates a favorable toxicity profile compared with other therapeutic modalities-important factors for this developing age group. A prospective, randomized international clinical trial assessment is warranted. © The Author(s) 2017. Published by Oxford University Press on behalf of

  19. Spectroscopic techniques (Moessbauer spectrometry, NMR, ESR...) as tools to resolve doubtful NMR images: Study of the craniopharyngioma tumor

    International Nuclear Information System (INIS)

    Rimbert, J.N.; Dumas, F.; Lafargue, C.; Kellershohn, C.; Brunelle, F.; Lallemand, D.

    1990-01-01

    Craniopharyngioma, an intracranial tumor, exhibits hyperintensity in the Spin-Echo-T 2 -NMR image and a hyposignal in the SE-T 1 -image. However, in some cases (15-20% cases), hypersignals are seen in both SE-T 1 and T 2 -MRI. Using spectroscopic techniques, Moessbauer spectrometry in particular, we have demonstrated that the T 1 hypersignal is due to ferritin, dissolved in the cystic liquid, after tumor cell lysis, in the course of time. Other possible reasons inducing a shortening of the T 1 relaxation time (presence of lipids, intratumoral hemorrhage) have been rejected. (orig.)

  20. Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome.

    Science.gov (United States)

    Hoffmann, Anika; Warmuth-Metz, Monika; Lohle, Kristin; Reichel, Julia; Daubenbüchel, Anna M M; Sterkenburg, Anthe S; Müller, Hermann L

    2016-08-01

    Fusiform dilatations of the internal carotid artery (FDCA) represent a vascular complication following surgery for suprasellar tumors in children. Incidence rate and long-term prognosis of FDCA in terms of survival rates, vascular complications, and quality of survival are unknown for patients with childhood-onset craniopharyngioma. Magnetic resonance imaging (MRI) results of 583 patients with childhood-onset craniopharyngioma, recruited from 2001 to 2015 in the German Childhood Craniopharyngioma Registry, were reviewed for FDCA. Risk factors for FDCA and long-term outcome after FDCA were analyzed. Fourteen of 583 patients (2.4 %) developed FDCA based on reference assessment of MRI. FDCA occurred ipsilateral to the surgical approach and was not related to degree of resection, hypothalamic involvement, or irradiation. The median time interval between first detection of FDCA and initial surgery was 0.79 years (range 0.01-5.56 years). During a median follow-up of 6.47 years (range 1.2-21.9 years) after first detection of FDCA, no bleeding or cerebrovascular events were observed in any patient. Irradiation was not related to FDCA. Survival rates and functional capacity were similar in patients with and without FDCA. Clinically the FDCA was unapparent in all cases and not treated. FDCA is a rare complication related to surgical treatment of childhood-onset craniopharyngioma without major impact on prognosis and clinical course of the disease. KRANIOPHARYNGEOM 2000-NCT00258453; KRANIOPHARYNGEOM 2007-NCT01272622.

  1. Effect of Preserving the Pituitary Stalk During Resection of Craniopharyngioma in Children on the Diabetes Insipidus and Relapse Rates and Long-Term Outcomes.

    Science.gov (United States)

    Cheng, Jing; Fan, Yanqin; Cen, Bo

    2017-09-01

    The objective of this study was to investigate the effect of preserving an infiltrated pituitary stalk during the resection of craniopharyngioma of pituitary stalk origin on postoperative outcomes and thus provide a theoretical basis for microsurgical treatment and prognosis. We screened the clinical data of all 103 pediatric patients with craniopharyngioma undergoing surgical treatment at our department between January 2006 and January 2013 and conducted a retrospective analysis of 82 patients with craniopharyngioma originating in the pituitary stalk. The patients were followed up from 12 months to 8 years. We analyzed the effect of preserving the pituitary stalk on the early and persistent diabetes insipidus rates, postoperative relapse rate, and mortality. In the total resection group (n = 67), the early and persistent diabetes insipidus rates were significantly lower in the 46 patients (68.7%) with a pituitary stalk than in those whose pituitary stalk was removed (P  0.05). In the subtotal resection group (n = 15), a significant difference was observed in the early and persistent diabetes insipidus rates (P  0.05). For children with craniopharyngioma of pituitary stalk origin, preserving the pituitary stalk has a significant effect on the early and persistent diabetes insipidus rates. When intraoperative exploration showed excessive adhesion between the tumor and pituitary stalk, we opted to preserve the pituitary stalk, which significantly reduced the early and persistent postoperative diabetes insipidus rates, without significantly increasing the relapse or mortality rate.

  2. Fusiform dilatation of the internal carotid artery following childhood craniopharyngioma resection treated by endovascular flow diversion-A case report and literature review.

    Science.gov (United States)

    Reynolds, Matthew R; Heiferman, Daniel M; Boucher, Andrew B; Serrone, Joseph C; Barrow, Daniel L; Dion, Jacques E

    2018-05-24

    Fusiform dilatation of the internal carotid artery (FDICA) is a well-described radiographic finding following resection of childhood craniopharyngioma (CP). A 39-year-old woman with right-sided FDICA was successfully treated for lesion enlargement with endovascular flow diversion, which has not been described in the literature. Published by Elsevier Ltd.

  3. Patients cured from craniopharyngioma or nonfunctioning pituitary macroadenoma (NFMA) suffer similarly from increased daytime somnolence despite normal sleep patterns compared to healthy controls

    NARCIS (Netherlands)

    van der Klaauw, Agatha A.; Biermasz, Nienke R.; Pereira, Alberto M.; van Kralingen, Klaas W.; Dekkers, Olaf M.; Rabe, Klaus F.; Smit, Johannes W. A.; Romijn, Johannes A.

    2008-01-01

    Adults patients previously treated for craniopharyngioma have increased general and physical fatigue compared to healthy controls. This could be related to disturbed sleep patterns. The aim of this study was to compare sleepiness and sleep patterns in those patients to healthy controls and to

  4. Investigating the Role of Hypothalamic Tumor Involvement in Sleep and Cognitive Outcomes Among Children Treated for Craniopharyngioma.

    Science.gov (United States)

    Jacola, Lisa M; Conklin, Heather M; Scoggins, Matthew A; Ashford, Jason M; Merchant, Thomas E; Mandrell, Belinda N; Ogg, Robert J; Curtis, Elizabeth; Wise, Merrill S; Indelicato, Daniel J; Crabtree, Valerie M

    2016-07-01

    Despite excellent survival prognosis, children treated for craniopharyngioma experience significant morbidity. We examined the role of hypothalamic involvement (HI) in excessive daytime sleepiness (EDS) and attention regulation in children enrolled on a Phase II trial of limited surgery and proton therapy. Participants completed a sleep evaluation (N = 62) and a continuous performance test (CPT) during functional magnetic resonance imaging (fMRI; n = 29) prior to proton therapy. EDS was identified in 76% of the patients and was significantly related to increased HI extent (p = .04). There was no relationship between CPT performance during fMRI and HI or EDS. Visual examination of group composite fMRI images revealed greater spatial extent of activation in frontal cortical regions in patients with EDS, consistent with a compensatory activation hypothesis. Routine screening for sleep problems during therapy is indicated for children with craniopharyngioma, to optimize the timing of interventions and reduce long-term morbidity. © The Author 2016. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  5. Autonomic nervous system balance in children and adolescents with craniopharyngioma and hypothalamic obesity.

    Science.gov (United States)

    Cohen, Michal; Syme, Catriona; McCrindle, Brian W; Hamilton, Jill

    2013-06-01

    Dysregulation of the autonomic nervous system is thought to be involved in craniopharyngioma-related hypothalamic obesity (CRHO). Increased parasympathetic activity and decreased sympathetic activity have been suggested. We aimed to study autonomic activity using heart rate variability (HRV) and biochemical measures in youth with CRHO compared with controls and to explore relationships between obesity and autonomic indices. A cross-sectional study of 16 youth with CRHO and 16 controls matched for sex, age, and BMI. Anthropometrics, fasting blood-work, resting energy expenditure (REE), 24-h HRV, and 24-h urine catecholamines were assessed. Quality of life, sleepiness, and autonomic symptoms were evaluated. Power spectral analysis of the HRV was performed. HRV power spectral analysis parameters of both parasympathetic activity (mean high frequency (HF (ms(2))) 611±504 vs 459±336, P=0.325) and sympathetic activity (median low frequency/HF 1.62 (1.37, 2.41) vs 1.89 (1.44, 2.99), P=0.650) did not differ between the groups. Parasympathetic activity negatively correlated with central adiposity in both groups (r=-0.53, P=0.034 and r=-0.54, P=0.029) and sympathetic activity positively correlated with central adiposity in CRHO (r=0.51, P=0.043). Youth with CRHO had significantly lower REE; lower health and activity scores in the quality of life questionnaires, and higher sleepiness scores. Autonomic activity was similar in CRHO and control subjects. The degree of central adiposity correlated negatively with parasympathetic activity and positively with sympathetic activity in children with CRHO. These results provide a new perspective regarding autonomic balance in this unique patient population.

  6. Clinical and prognostic role of annexin A2 in adamantinomatous craniopharyngioma.

    Science.gov (United States)

    Wang, Yuelong; Deng, Jiaojiao; Guo, Gang; Tong, Aiping; Peng, Xirui; Chen, Haifeng; Xu, Jianguo; Liu, Yi; You, Chao; Zhou, Liangxue

    2017-01-01

    Annexin A2 (AnxA2) is a highly conserved Ca2 + -regulated membrane binding protein, which affects cell mobility and tumor progression. Adamantinomatous craniopharyngioma (AdaCP) are a kind of epithelial tumors of the sellar region with high tendency to recur. Robust biomarkers are required to predict tumor behavior and to establish follow-up individualized treatment approaches. In this study, we firstly compared four surgical AdaCP samples with normal brain by two-dimensional gel electrophoresis (2DE) proteomic analysis. Potential prognostic biomarkers were further validated in a large cohort of 65 AdaCPs by immunohistochemistry. The effects of AnxA2 on AdaCP cells proliferation and migration were analyzed in vitro with isolated primary AdaCP cells as well as SV40T-immortalized cells. Finally, the gefitinib sensitivity of AdaCPs with differentially expressed AnxA2 and the potential molecular mechanisms were examined by flow cytometric analysis, Real-time PCR and immunoblot assays. Proteomic analysis indicated that AnxA2 was the protein spot with the most elevated expression in AdaCP samples. Immunohistochemistry assays indicated the expression level of AnxA2 was significantly higher in recurrent AdaCPs compared with primary ones. Moreover, AnxA2 + AdaCP cells exhibited enhanced proliferation and migration ability compared with AnxA2 - AdaCP cells in vitro. Further, we show that AnxA2 + AdaCP cells exhibited elevated expression of EGFR and downstream p-AKT (S308) and p-AKT (S473), and were more sensitive to tyrosine kinase inhibitor gefitinib. Our data suggest that AnxA2 may serve as a promising biomarker for AdaCP progression, recurrence and drug susceptibility. Our data support potential clinical implications for the follow-up treatment of AdaCP patients with high AnxA2 expression.

  7. Microstructural white matter alterations and hippocampal volumes are associated with cognitive deficits in craniopharyngioma.

    Science.gov (United States)

    Fjalldal, S; Follin, C; Svärd, D; Rylander, L; Gabery, S; Petersén, Å; van Westen, D; Sundgren, P C; Björkman-Burtscher, I M; Lätt, J; Ekman, B; Johanson, A; Erfurth, E M

    2018-06-01

    Patients with craniopharyngioma (CP) and hypothalamic lesions (HL) have cognitive deficits. Which neural pathways are affected is unknown. To determine whether there is a relationship between microstructural white matter (WM) alterations detected with diffusion tensor imaging (DTI) and cognition in adults with childhood-onset CP. A cross-sectional study with a median follow-up time of 22 (6-49) years after operation. The South Medical Region of Sweden (2.5 million inhabitants). Included were 41 patients (24 women, ≥17 years) surgically treated for childhood-onset CP between 1958-2010 and 32 controls with similar age and gender distributions. HL was found in 23 patients. Subjects performed cognitive tests and magnetic resonance imaging, and images were analyzed using DTI of uncinate fasciculus, fornix, cingulum, hippocampus and hypothalamus as well as hippocampal volumetry. Right uncinate fasciculus was significantly altered ( P  ≤ 0.01). Microstructural WM alterations in left ventral cingulum were significantly associated with worse performance in visual episodic memory, explaining approximately 50% of the variation. Alterations in dorsal cingulum were associated with worse performance in immediate, delayed recall and recognition, explaining 26-38% of the variation, and with visuospatial ability and executive function, explaining 19-29%. Patients who had smaller hippocampal volume had worse general knowledge ( P  = 0.028), and microstructural WM alterations in hippocampus were associated with a decline in general knowledge and episodic visual memory. A structure to function relationship is suggested between microstructural WM alterations in cingulum and in hippocampus with cognitive deficits in CP. © 2018 The authors.

  8. Gene expression profiles reveal key genes for early diagnosis and treatment of adamantinomatous craniopharyngioma.

    Science.gov (United States)

    Yang, Jun; Hou, Ziming; Wang, Changjiang; Wang, Hao; Zhang, Hongbing

    2018-04-23

    Adamantinomatous craniopharyngioma (ACP) is an aggressive brain tumor that occurs predominantly in the pediatric population. Conventional diagnosis method and standard therapy cannot treat ACPs effectively. In this paper, we aimed to identify key genes for ACP early diagnosis and treatment. Datasets GSE94349 and GSE68015 were obtained from Gene Expression Omnibus database. Consensus clustering was applied to discover the gene clusters in the expression data of GSE94349 and functional enrichment analysis was performed on gene set in each cluster. The protein-protein interaction (PPI) network was built by the Search Tool for the Retrieval of Interacting Genes, and hubs were selected. Support vector machine (SVM) model was built based on the signature genes identified from enrichment analysis and PPI network. Dataset GSE94349 was used for training and testing, and GSE68015 was used for validation. Besides, RT-qPCR analysis was performed to analyze the expression of signature genes in ACP samples compared with normal controls. Seven gene clusters were discovered in the differentially expressed genes identified from GSE94349 dataset. Enrichment analysis of each cluster identified 25 pathways that highly associated with ACP. PPI network was built and 46 hubs were determined. Twenty-five pathway-related genes that overlapped with the hubs in PPI network were used as signatures to establish the SVM diagnosis model for ACP. The prediction accuracy of SVM model for training, testing, and validation data were 94, 85, and 74%, respectively. The expression of CDH1, CCL2, ITGA2, COL8A1, COL6A2, and COL6A3 were significantly upregulated in ACP tumor samples, while CAMK2A, RIMS1, NEFL, SYT1, and STX1A were significantly downregulated, which were consistent with the differentially expressed gene analysis. SVM model is a promising classification tool for screening and early diagnosis of ACP. The ACP-related pathways and signature genes will advance our knowledge of ACP pathogenesis

  9. HORMONE SUBSTITUTION AFTER GASTRIC BYPASS SURGERY IN PATIENTS WITH HYPOPITUITARISM SECONDARY TO CRANIOPHARYNGIOMA.

    Science.gov (United States)

    Wolf, Peter; Winhofer, Yvonne; Smajis, Sabina; Kruschitz, Renate; Schindler, Karin; Gessl, Alois; Riedl, Michaela; Vila, Greisa; Raber, Wolfgang; Langer, Felix; Prager, Gerhard; Ludvik, Bernhard; Luger, Anton; Krebs, Michael

    2016-05-01

    Craniopharyngiomas (CPs) are benign brain tumors presenting frequently in childhood and are treated by surgery with or without radiotherapy. About 50% of cured patients suffer from eating disorders and obesity due to hypothalamic damage, as well as hypopituitarism, necessitating subsequent hormone substitution therapy. Gastric bypass surgery has been reported to be an efficient treatment strategy for morbid hypothalamic obesity. However, so far it is unknown whether oral hormone substitution is affected by impaired intestinal drug absorption, potentially leading to severe hypopituitarism or pituitary crisis. Four morbidly obese CP patients with panhypopituitarism treated by gastric bypass surgery were included in this retrospective analysis. Dosages of hormone substitution therapy, blood concentrations of hormones, potential complications of impaired drug absorption, and anthropometric characteristics were investigated pre- and postoperatively after 6 to 14 months and 13 to 65 months. In all CP patients (3 female/1 male; baseline body mass index, 49 ± 7 kg/m(2)), gastric bypass resulted in distinct weight loss (-35 ± 27 kg). In follow-up examinations, mean daily dosage of thyroid hormone (levothyroxinebaseline 156 ± 44 μg/day versus levothyroxinefollow-up 150 ± 30 μg/day), hydrocortisone (hydrocortisonebaseline 29 ± 12 mg/day versus hydrocortisonefollow-up 26 ± 2 mg/day), growth-hormone (somatotropinbaseline 0.9 ± 0.5 mg/day versus somatotropinfollow-up 1.0 ± 0.4 mg/day), and desmopressin (desmopressinbaseline 222 ± 96 μg/day versus desmopressinfollow-up 222 ± 96 μg/day) substitution was unchanged. No patient developed adrenal insufficiency. Oral thyroid/hydrocortisone absorption testing performed in 1 patient indicated sufficient gastrointestinal drug absorption after bariatric surgery. Our preliminary results suggest that oral hormone substitution therapy is not impaired following gastric bypass operation in CP patients with morbid obesity

  10. Slow growing desmoid-type fibromatosis of the breast: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ah Reum; Kim, Ji Young; Kim, Hyun Jung; KIm, Jae Hyung; Jeong, Myeong Ja; Kim, Soung Hee; Kim, Soo Hyun; Kang, Mi Jin; Lee, Ji Hae; Bae, Kyung Eun [Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of)

    2017-04-15

    Fibromatosis or desmoid tumor of the breast is a rare benign entity that has no metastatic potential but has significant risk of local recurrence. Its association with previous surgical or accidental trauma and Gardner's syndrome has been reported. Awareness of this lesion is important as the diagnosis is often confused with breast carcinoma. We report a case of a 44-year-old woman who presented with a palpable mass in her left breast, close to the axilla since a few months ago. She had undergone excisional biopsy for her left breast mass 15 months ago, and the diagnosis was confirmed as intraductal papilloma with atypical ductal hyperplasia. Subsequent ultrasound and core needle biopsy revealed stromal fibrosis. After 9 months, the mass showed an increase in its size and the anteroposterior to width ratio on ultrasonography compared to the previous examination, and final excisional biopsy confirmed the diagnosis of desmoid-type fibromatosis.

  11. An inducible expression system for high-level expression of recombinant proteins in slow growing mycobacteria.

    Science.gov (United States)

    Leotta, Lisa; Spratt, Joanne M; Kong, Carlyn U; Triccas, James A

    2015-09-01

    A novel protein expression vector utilising the inducible hspX promoter of Mycobacterium tuberculosis was constructed and evaluated in this study. High-level induction of three mycobacterial antigens, comprising up to 9% of bacterial sonicate, was demonstrated in recombinant Mycobacterium bovis BCG when grown under low-oxygen tension, which serves to enhance hspX promoter activity. Recombinant proteins were efficiently purified from bacterial lysates in a soluble form by virtue of a C-terminal 6-histidine tag. Purification of the immunodominant M. tuberculosis Ag85B antigen using this system resulted in a recombinant protein that stimulated significant IFN-γ release from Ag85B-reactive T cells generated after vaccination of mice with an Ag85B-expressing vaccine. Further, the M. tuberculosis L-alanine dehydrogenase (Ald) protein purified from recombinant BCG displayed strong enzymatic activity in recombinant form. This study demonstrated that high levels of native-like recombinant mycobacterial proteins can be produced in mycobacterial hosts, and this may aid the analysis of mycobacterial protein function and the development of new treatments. Copyright © 2015 Elsevier B.V. All rights reserved.

  12. Prececal amino acid digestibility of soybean cake in fast- and slow-growing broiler chickens.

    Science.gov (United States)

    Ganzer, C; Siegert, W; Kluth, H; Bennewitz, J; Rodehutscord, M

    2017-08-01

    The objective of the present study was to determine whether there are differences in prececal amino acid digestibility between commonly used slow- and fast-growing broiler strains when the regression approach is applied. ISA J-275 and Ross 308 were selected as common representatives of slow- and fast-growing broiler strains, respectively. The experimental diets with soybean cake at levels of 0, 100, and 200 g/kg were offered for ad libitum consumption between 22 and 29 d post-hatch. Titanium dioxide was used as an indigestible marker. Each treatment was tested with six pens comprising 10 birds each. Digesta samples were collected on a pen basis from the distal two-thirds of the intestine section between Meckel's diverticulum and 2 cm anterior to the ileocecal-colonic junction. The prececal amino acid digestibility of soybean cake was calculated by linear regression simultaneously for both strains. There was no significant interaction between broiler strain and inclusion level of soybean cake with respect to the prececal CP and amino acid digestibility of complete diets; there was a significant strain effect for 5 out of the 16 measured amino acids. The prececal CP and amino acid digestibility of soybean cake did not differ significantly between strains and was numerically almost identical. The results of the present study provide evidence of the transferability between broiler strains of prececal amino acid digestibility data, determined using the regression approach, thus improving the accuracy of diet formulation without drawbacks. © 2017 Poultry Science Association Inc.

  13. The Slow-Growing Orange, A Demographer’s Look at Future Los Angeles,

    Science.gov (United States)

    1984-04-01

    and even some downtown Los Angeles neighborhoods, through " gentrification "--will grow rapidly at the expense of others. A second dramatic aspect of...shores. Modern developments in commuaications and transportation, which have enabled us to expand trade with Asia and Latin America, have also

  14. Slow-growing labyrinthine masses: contribution of MRI to diagnosis, follow-up and treatment

    International Nuclear Information System (INIS)

    Deux, J.F.; Marsot-Dupuch, K.; Tubiana, J.M.; Tran Ba Huy, P.; Sterkers, J.M.

    1998-01-01

    We report the use of MRI in the diagnosis, follow-up and therapeutic management of three cases of intralabyrinthine Schwannoma. The diagnosis was based on the history and initial and follow-up MRI findings. The main feature suggesting the diagnosis was a nodular intralabyrinthine mass of low signal intensity on T2-weighted images, and high or isointense signal on T1-weighted images (relative to cerebrospinal fluid), which showed contrast enhancement. Follow-up imaging showed growth of the tumour in one patient. One patient underwent surgery for severe tinnitus. To detect these lesions, MRI should be focussed on the inner ear, using thin-section T2-weighted and T1-weighted images before and after contrast medium. MRI allowed informed surgical planning. (orig.) (orig.)

  15. Slow-growing labyrinthine masses: contribution of MRI to diagnosis, follow-up and treatment

    Energy Technology Data Exchange (ETDEWEB)

    Deux, J F; Marsot-Dupuch, K; Tubiana, J M [Hopital Saint-Antoine, Paris (France). Service de Radiology; Ouayoun, M; Meyer, B [Service d` ORL, Hopital Saint-Antoine, 184 rue du Faubourg Saint-Antoine, F-75012 Paris (France); Tran Ba Huy, P [Service d` ORL, Hopital Lariboisiere, 2 rue Ambroise Pare, F-75010 Paris (France); Sterkers, J M

    1998-10-01

    We report the use of MRI in the diagnosis, follow-up and therapeutic management of three cases of intralabyrinthine Schwannoma. The diagnosis was based on the history and initial and follow-up MRI findings. The main feature suggesting the diagnosis was a nodular intralabyrinthine mass of low signal intensity on T2-weighted images, and high or isointense signal on T1-weighted images (relative to cerebrospinal fluid), which showed contrast enhancement. Follow-up imaging showed growth of the tumour in one patient. One patient underwent surgery for severe tinnitus. To detect these lesions, MRI should be focussed on the inner ear, using thin-section T2-weighted and T1-weighted images before and after contrast medium. MRI allowed informed surgical planning. (orig.) (orig.) With 3 figs., 1 tab., 21 refs.

  16. Behaviour of fast- and slow growing broilers to 12 weeks of age and the physical consequences

    NARCIS (Netherlands)

    Bokkers, E.A.M.; Koene, P.

    2003-01-01

    Behaviour of broilers up to 6 weeks of age has been studied extensively, but little is known what happens after 6 weeks. Insight in the behavioural abilities after 6 weeks may also yield insight in the period before 6 weeks as the disbalance between motivation and physical abilities is more clearly

  17. Geochemistry of Slow-Growing Corals: Reconstructing Sea Surface Temperature, Salinity and the North Atlantic Oscillation

    Science.gov (United States)

    2007-06-01

    Number 8, October 1989) The island of Bermuda (64°W, 32°N) (Fig. 1.3) located in the western sub- tropical Atlantic is an excellent location for examining...lobata at Clipperton Atoll, Coral Reefv, 18, 13-27, 1999. Lough, J. M., A strategy to improve the contribution of coral data to high-resolution...to evaluate winter Sr/Ca. The island of Bermuda (64°W, 32°N) is located in the Sargasso Sea in the sub-tropical North Atlantic. This site is

  18. Long-term follow-up of patients after intracavitary irradiation of cystic craniopharyngiomas with Y-90 colloid

    International Nuclear Information System (INIS)

    Vizda, J.; Urbanova, E.

    2007-01-01

    Full text: Craniopharyngiomas are cystic para-hypophyseal tumors. These tumors are benign but difficulties in curing can lead to serious morbidity or death. No primary medical therapy exists. The possible modes of treatment are surgical extirpation, evacuation of the cyst by craniotomy and marsupialisation into the subarachnoidal space or ventricular system, aspiration through an Ommaya reservoir, shunting of the ventricular system, external beam radiotherapy, stereotactic irradiation with the Leksell gamma knife, intracystic chemotherapy or intracystic installation of beta-emitting colloid radiopharmaceuticals. Aim of this study was long-term follow-up of the patients after intracystic administration of beta-emitting radiopharmaceutical. Patients and methods: 18 patients suffering from cystic craniopharyngioma (in age 2 67 years) were analyzed five years after intracystic irradiation. 90-yttrium silicate colloid was instilled stereotactically (CT guided) directly to the cyst or through Ommaya drainage system. Administered activity was in range 40-222 MBq, depending on the cyst volume. The cyst volume was determined by CT or MRI. Calculated cumulative dose to the inner surface of the cyst wall was 250 Gy. Modification of Backlund's formula for dosimetry was used. Control scintigraphy was performed 2-3 hours after instillation of radiopharmaceutical to detect possible leakage. None of our patients showed evidence of this. Subsequently visual function, neurological condition and cyst volume were checked. Results: Reduction of the initial cyst volume was observed in 50% patients during four month after therapy. One year after therapy, shrinkage of the initial volume was confirmed in 78% of the cysts, in five of these cases cysts disappeared nearly totally. In four patients the treatment was without evident success. In none of the patients worsening of visual function or evidence of hypothalamic dysfunction was observed after radiocolloid instillation. Five years after

  19. Drug priming enhances radiosensitivity of adamantinomatous craniopharyngioma via downregulation of survivin.

    Science.gov (United States)

    Stache, Christina; Bils, Christiane; Fahlbusch, Rudolf; Flitsch, Jörg; Buchfelder, Michael; Stefanits, Harald; Czech, Thomas; Gaipl, Udo; Frey, Benjamin; Buslei, Rolf; Hölsken, Annett

    2016-12-01

    OBJECTIVE In this study, the authors investigated the underlying mechanisms responsible for high tumor recurrence rates of adamantinomatous craniopharyngioma (ACP) after radiotherapy and developed new targeted treatment protocols to minimize recurrence. ACPs are characterized by the activation of the receptor tyrosine kinase epidermal growth factor receptor (EGFR), known to mediate radioresistance in various tumor entities. The impact of tyrosine kinase inhibitors (TKIs) gefitinib or CUDC-101 on radiation-induced cell death and associated regulation of survivin gene expression was evaluated. METHODS The hypothesis that activated EGFR promotes radioresistance in ACP was investigated in vitro using human primary cell cultures of ACP (n = 10). The effects of radiation (12 Gy) and combined radiochemotherapy on radiosensitivity were assessed via cell death analysis using flow cytometry. Changes in target gene expression were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Survivin, identified in qRT-PCR to be involved in radioresistance of ACP, was manipulated by small interfering RNA (siRNA), followed by proliferation and vitality assays to further clarify its role in ACP biology. Immunohistochemically, survivin expression was assessed in patient tumors used for primary cell cultures. RESULTS In primary human ACP cultures, activation of EGFR resulted in significantly reduced cell death levels after radiotherapy. Treatment with TKIs alone and in combination with radiotherapy increased cell death response remarkably, assessed by flow cytometry. CUDC-101 was significantly more effective than gefitinib. The authors identified regulation of survivin expression after therapeutic intervention as the underlying molecular mechanism of radioresistance in ACP. EGFR activation promoting ACP cell survival and proliferation in vitro is consistent with enhanced survivin gene expression shown by qRT-PCR. TKI treatment, as well as the combination with

  20. Nuchal Skinfold Thickness: A Novel Parameter for Assessment of Body Composition in Childhood Craniopharyngioma

    Science.gov (United States)

    Sterkenburg, Anthe S.; Hoffmann, Anika; Reichel, Julia; Lohle, Kristin; Eveslage, Maria; Warmuth-Metz, Monika

    2016-01-01

    Context: Hypothalamic obesity, cardiovascular disease (CVD), and relapse/progression have a major impact on prognosis in childhood-onset craniopharyngioma (CP). We analyzed nuchal skinfold thickness (NST) on magnetic resonance imaging performed for follow-up monitoring as a novel parameter for body composition (BC) and CVD in CP. Objective: The objective of the study was to identify the association of NST with body mass index (BMI), waist to height ratio (WHtR), functional capacity, and blood pressure (BP) in CP and controls. Design: This was a cross-sectional and longitudinal prospective study in CP patients. Setting: The study was conducted at HIT-Endo, KRANIOPHARYNGEOM 2000/2007. Patients: Participants included 94 CP patients and 75 controls. Interventions: There were no interventions. Main Outcome Measures: Association of NST with BC and BP in 43 CP and 43 controls was measured. Results: NST correlated with BMI SD score (SDS; r = 0.78; P < .001; n = 169) and WHtR (r = 0.85; P < .001; n = 86) in the total cohort and CP patients (NST-BMI SDS: r = 0.77, P < .001, n = 94); NST-WHtR: r = 0.835, P < .001, n=43) and controls (NST-BMI SDS: r = 0.792, P < .001, n = 75; NST-WHtR: r = 0.671, P < .001, n = 43). In CP, systolic BP correlated with NST (r = 0.575, P < .001), BMI SDS (r = 0.434, P = .004), and WHtR (r = 0.386, P = .011). Similar results were observed for diastolic BP in CP. In multivariate analyses, NST had a predictive value for hypertension in postpubertal CP and controls (odds ratio 6.98, 95% confidence interval [1.65, 29.5], P = .008). During a longitudinal follow-up, changes in NST correlated with changes in BMI SDS (P < .001) and WHtR (P = .01) but not with changes in BP and functional capacity. Conclusions: Because monitoring of magnetic resonance imaging and BC is essential for follow-up in CP, NST could serve as a novel and clinically relevant parameter for longitudinal assessment of BC and CVD risk in CP. PMID:27680877

  1. De-masking oxytocin-deficiency in craniopharyngioma and assessing its link with affective function.

    Science.gov (United States)

    Gebert, Dorothea; Auer, Matthias K; Stieg, Mareike R; Freitag, Martin T; Lahne, Madlén; Fuss, Johannes; Schilbach, Katharina; Schopohl, Jochen; Stalla, Günter K; Kopczak, Anna

    2018-02-01

    Despite the high prevalence of panhypopituitarism and diabetes insipidus in patients with craniopharyngioma (CP), little is known about the functioning of the neuropeptide oxytocin in these patients. This is of special interest as tumor-associated lesions often impair sites critical for oxytocin production and release, and affective dysfunction in CP links with elsewhere reported prosocial, antidepressant and anxiolytic oxytocin effects. Using a prospective study-design, we tested whether oxytocin is reduced in CP-patients, and whether altered oxytocin levels account for affective and emotional dysfunction. 26 adult CP-patients and 26 healthy controls matched in sex and age underwent physical exercise, a stimulus previously shown to induce oxytocin release. Baseline and stimulated salivary oxytocin levels, as well as empathy, depression and anxiety scores were measured. Results showed that patients overall did not present with lower baseline oxytocin levels than controls (F[1,30]=0.21, p=0.649), but baseline oxytocin levels were indeed reduced in patients with hypothalamic damage, as assessed by MRI-based grading (F[2,9.79]=4.54, p=0.040). In response to exercise-induced stimulation, all CP-patients showed a blunted oxytocin-release compared to controls (F[1,30]=9.36, p=0.005). DI was not associated with oxytocin levels. Regarding affective function, unexpectedly, higher baseline oxytocin was related to higher trait anxiety (b=2.885, t(43)=2.421, p=0.020, CI[.478; 5.292]); the positive link with higher depression failed to reach statistical significance (b=1.928, t(43)=1.949, p=0.058, CI[-0.070; 3.927]). A blunted oxytocin-release was linked with higher state anxiety (b=-0.133, t(43)=-2.797, p=0.008, CI[-0.230; -0.037]). Empathy was not associated with oxytocin measures. In conclusion, we observed reduced baseline oxytocin levels only in CP-patients with hypothalamic damage. Exercise-induced stimulation de-masked an oxytocin-deficiency in all CP-patients. Baseline

  2. Interleukin‑6 induces an epithelial‑mesenchymal transition phenotype in human adamantinomatous craniopharyngioma cells and promotes tumor cell migration.

    Science.gov (United States)

    Zhou, Jie; Zhang, Chao; Pan, Jun; Chen, Ligang; Qi, Song-Tao

    2017-06-01

    Total resection of adamantinomatous craniopharyngioma (ACP) is complex and often leads to postoperative recurrence. This is due to the tendency of the tumor to invade the surrounding brain tissue and the generation of a local inflammatory state between the tumor cells and parenchyma. While there is evidence to suggest that interleukin‑6 (IL‑6) induces craniopharyngioma (CP)‑associated inflammation, particularly in ACP, the role of IL‑6 in the progression of ACP remains unclear. The results of the present study demonstrated that CP inflammation was associated with pathological classification, extent of surgery, degree of calcification and postoperative hypothalamic status scale. Cytokine antibody arrays were conducted to measure the expression of IL‑6 and other inflammatory factors in tumor tissues in response to various levels of inflammatory exposure. IL‑6, IL‑6 receptor (IL‑6R) and glycoprotein 130 expression was detected by immunohistochemistry. In addition, an ELISA was performed to quantify the levels of soluble IL‑6R (sIL‑6R) in the cystic fluid and supernatants of ACP cells and tumor‑associated fibroblasts. These measurements demonstrated that ACP cells produce IL‑6 and its associated proteins. In addition, the results revealed that while the viability of ACP cells was not affected, the migration of ACP cells was promoted by IL‑6 treatment in a concentration‑dependent manner. Conversely, treatment with an IL‑6‑blocking monoclonal antibody significantly decreased the migration of ACP cells. In addition, IL‑6 treatment increased the expression of vimentin and decreased the expression of E‑cadherin in a dose‑dependent manner. The findings of the present study demonstrate that IL‑6 may promote migration in vitro via the classic‑ and trans‑signaling pathways by inducing epithelial‑mesenchymal transition in ACP cell cultures.

  3. Interleukin-6 induces an epithelial-mesenchymal transition phenotype in human adamantinomatous craniopharyngioma cells and promotes tumor cell migration

    Science.gov (United States)

    Zhou, Jie; Zhang, Chao; Pan, Jun; Chen, Ligang; Qi, Song-Tao

    2017-01-01

    Total resection of adamantinomatous craniopharyngioma (ACP) is complex and often leads to postoperative recurrence. This is due to the tendency of the tumor to invade the surrounding brain tissue and the generation of a local inflammatory state between the tumor cells and parenchyma. While there is evidence to suggest that interleukin-6 (IL-6) induces craniopharyngioma (CP)-associated inflammation, particularly in ACP, the role of IL-6 in the progression of ACP remains unclear. The results of the present study demonstrated that CP inflammation was associated with pathological classification, extent of surgery, degree of calcification and postoperative hypothalamic status scale. Cytokine antibody arrays were conducted to measure the expression of IL-6 and other inflammatory factors in tumor tissues in response to various levels of inflammatory exposure. IL-6, IL-6 receptor (IL-6R) and glycoprotein 130 expression was detected by immunohistochemistry. In addition, an ELISA was performed to quantify the levels of soluble IL-6R (sIL-6R) in the cystic fluid and supernatants of ACP cells and tumor-associated fibroblasts. These measurements demonstrated that ACP cells produce IL-6 and its associated proteins. In addition, the results revealed that while the viability of ACP cells was not affected, the migration of ACP cells was promoted by IL-6 treatment in a concentration-dependent manner. Conversely, treatment with an IL-6-blocking monoclonal antibody significantly decreased the migration of ACP cells. In addition, IL-6 treatment increased the expression of vimentin and decreased the expression of E-cadherin in a dose-dependent manner. The findings of the present study demonstrate that IL-6 may promote migration in vitro via the classic- and trans-signaling pathways by inducing epithelial-mesenchymal transition in ACP cell cultures. PMID:28487953

  4. Orbitopterional Approach for the Resection of a Suprasellar Craniopharyngioma: Adapting the Strategy to the Microsurgical and Pathologic Anatomy.

    Science.gov (United States)

    Nguyen, Vincent; Basma, Jaafar; Klimo, Paul; Sorenson, Jeffrey; Michael, L Madison

    2018-04-01

    Objectives  To describe the orbitopterional approach for the resection of a suprasellar craniopharyngioma with emphasis on the microsurgical and pathological anatomy of such lesions. Design  After completing the orbitopterional craniotomy in one piece including a supraorbital ridge osteotomy, the Sylvian fissure was split in a distal to proximal direction. The ipsilateral optic nerve and internal carotid artery were identified. Establishing a corridor to the tumor through both the opticocarotid and optic cisterns allowed for a wide angle of attack. Using both corridors, a microsurgical gross total resection was achieved. A radical resection required transection of the stalk at the level of the hypothalamus. Photographs of the region are borrowed from Dr Rhoton's laboratory to illustrate the microsurgical anatomy. Understanding the cisternal and topographic relationships of the optic nerve, optic chiasm, and internal carotid artery is critical to achieving gross total resection while preserving normal anatomy. Participants  The surgery was performed by the senior author assisted by Dr. Jaafar Basma. The video was edited by Dr. Vincent Nguyen. Outcome Measures  Outcome was assessed with extent of resection and postoperative visual function. Results  A gross total resection of the tumor was achieved. The patient had resolution of her bitemporal hemianopsia. She had diabetes insipidus with normal anterior pituitary function. Conclusions  Understanding the microsurgical anatomy of the suprasellar region and the pathological anatomy of craniopharyngiomas is necessary to achieve a good resection of these tumors. The orbitopterional approach provides the appropriate access for such endeavor. The link to the video can be found at: https://youtu.be/Be6dtYIGqfs .

  5. Cystic tumors of the pituitary infundibulum: seminal autopsy specimens (1899 to 1904) that allowed clinical-pathological craniopharyngioma characterization.

    Science.gov (United States)

    Pascual, José M; Prieto, Ruth; Rosdolsky, Maria; Strauss, Sewan; Castro-Dufourny, Inés; Hofecker, Verena; Winter, Eduard; Carrasco, Rodrigo; Ulrich, Walter

    2018-04-21

    A heterogeneous group of epithelial cystic tumors developed at the infundibulum and the third ventricle disconcerted pathologists at the dawn of the twentieth century. Very little was known at that time about the physiological role played by the pituitary gland, and there was almost complete ignorance regarding the function of the hypothalamus. Acromegaly, or enlargement of acral body parts, described in 1886 by Pierre Marie, was the only disease linked to primary hypertrophies of the pituitary gland, known as "pituitary strumas". A growing number of young patients manifesting an unexplained combination of physical and mental symptoms, including absent or delayed sexual maturation, progressive obesity, abnormal somnolence, and dementia-like changes in behavior were reported to present large solid-cystic tumors which characteristically expanded within the infundibulum and third ventricle, above an anatomically intact pituitary gland. Between 1899 and 1904, five seminal autopsy studies from different countries thoroughly described the anatomical relationships and histological features of this newly recognized type of infundibular tumors. These cases were instrumental in fostering the systematic investigation of similar lesions by the Austrian pathologist Jakob Erdheim (1874-1937), who in 1904 was able to classify these infundibulo-tuberal cysts under the common category of hypophyseal duct tumors. The pioneering American neurosurgeon Harvey Cushing (1869-1939) unsuccessfully attempted to surgically remove one of these cysts, for the first time in history, in 1902. The term "craniopharyngioma", chosen by Cushing in 1929 to designate these lesions, would eventually prevail over Erdheim's more accurate denomination, which linked their origin to squamous cell remnants derived from the embryological structures that give rise to the pituitary gland. This paper presents a comprehensive, renewed account of the five clinical-pathological reports which laid the groundwork for

  6. Monte Carlo and analytical calculations of dose distributions in craniopharyngioma cysts treated with radiocolloids containing {sup 32}P or {sup 186}Re

    Energy Technology Data Exchange (ETDEWEB)

    Sadeghi, Mahdi [Agricultural, Medical and Industrial Research School, Nuclear Science and Technology Research Institute, P.O. Box 31485/498, Karaj (Iran, Islamic Republic of)], E-mail: msadeghi@nrcam.org; Karimi, Elham; Sardari, Darush [Faculty of Engineering, Research and Science Campus, Islamic Azad University, Tehran (Iran, Islamic Republic of)

    2009-09-15

    In radiation treatments of some types of brain tumors, such as craniopharyngiomas, selection of an appropriate radionuclide is critical. The aim of this work was to calculate distributions of dose rates from {sup 32}P and {sup 186}Re in radiocolloids injected into craniopharyngioma cysts. The calculations were performed with the MCNP4C radiation transport code. Analytical calculations based on the Loevinger formula were also performed for {sup 32}P with the MATLAB software. The results of the two techniques for identical models were compared. The effects of the cyst wall type and of the density of the cyst inner fluid were investigated. The {sup 32}P activities required for providing 200, 250, and 300 Gy to cysts of different sizes were calculated.

  7. Trans-eyebrow supraorbital approach in large suprasellar craniopharyngioma surgery in adults: analysis of optic nerve length and extent of tumor resection. Original article.

    Science.gov (United States)

    Prat, Ricardo; Galeano, Inma; Evangelista, Rocío; Pancucci, Giovanni; Guarín, Juliana; Ayuso, Angel; Misra, Mukesh

    2017-05-01

    One of the main drawbacks in the surgery of large craniopharyngiomas is the presence of a prefixed optic chiasm. Our main objective in this study is to compare the predictive value of the optic nerve length and optic chiasm location on large craniopharyngiomas' extent of resection. We retrospectively studied 21 consecutive patients with large craniopharyngiomas who underwent tumor resection through the trans-eyebrow supraorbital approach. Clinical and radiological findings on preoperative MRI were recorded, including the optic chiasm location classified as prefixed, postfixed or normal. We registered the optic nerve length measured intraoperatively prior to tumor removal and confirmed the measurements on preoperative MRI. Using a linear regression model, we calculated a prediction formula of the percentage of the extent of resection as a function of optic nerve length. On preoperative MRI, 15 patients were considered to have an optic chiasm in a normal location, 3 cases had a prefixed chiasm, and the remaining 3 had a postfixed chiasm. In the group with normal optic chiasm location, a wide range of percentage of extent of resection was observed (75-100%). The percentage of extent of resection of large craniopharyngiomas was observed to be dependent on the optic nerve length in a linear regression model (p < 0.0001). According to this model in the normal optic chiasm location group, we obtained an 87% resection in 9-mm optic nerve length patients, a 90.5% resection in 10-mm optic nerve length patients and 100% resection in 11-mm optic nerve length patients. Optic chiasm location provides useful information to predict the percentage of resection in both prefixed and postfixed chiasm patients but not in the normal optic chiasm location group. Optic nerve length was proven to provide a more accurate way to predict the percentage of resection than the optic chiasm location in the normal optic chiasm location group.

  8. Dosimetric comparison of {sup 90}Y, {sup 32}P, and {sup 186}Re radiocolloids in craniopharyngioma treatments

    Energy Technology Data Exchange (ETDEWEB)

    Sadeghi, Mahdi; Karimi, Elham; Hosseini, S. Hamed [Agricultural, Medical and Industrial Research School, Nuclear Science and Technology Research Institute, P.O. Box 31485/498, Karaj (Iran, Islamic Republic of); Faculty of Engineering, Research and Science Campus, Islamic Azad University, P.O. Box 14155/4933, Tehran (Iran, Islamic Republic of)

    2009-11-15

    Purpose: In the radionuclide treatment of some forms of brain tumors such as craniopharyngiomas, the selection of the appropriate radionuclide for therapy is a key element in treatment planning. The aim was to study the influence by considering the beta-emitter radionuclide dose rate in an intracranial cyst. Methods: Dosimetry was performed using the MCNP4C radiation transport code. Analytical dosimetry was additionally performed using the Loevinger and the Berger formulas in the MATLAB software. Each result was compared under identical conditions. The advantages and disadvantages of using {sup 90}Y versus {sup 32}P and {sup 186}Re were investigated. Results: The dose rate at the inner surface of the cyst wall was estimated to be 400 mGy/h for a 1 MBq/ml concentration of {sup 90}Y. Under identical conditions of treatment, the corresponding dose rates were 300 mGy/h for {sup 32}P and 160 mGy/h for {sup 186}Re. For a well-defined cyst radius and identical wall thickness, higher dose rates resulted for {sup 90}Y. Conclusions: To achieve the same radiological burden, the required amount of physical activity of injectable solution is lower for {sup 32}P. This is found to be a consequence of both the radionuclide physical half-life and the pattern of energy deposition from the emitted radiation. According to the half-life and dose-rate results, {sup 90}Y would be a good substitute for {sup 32}P.

  9. Stem cell senescence drives age-attenuated induction of pituitary tumours in mouse models of paediatric craniopharyngioma.

    Science.gov (United States)

    Mario Gonzalez-Meljem, Jose; Haston, Scott; Carreno, Gabriela; Apps, John R; Pozzi, Sara; Stache, Christina; Kaushal, Grace; Virasami, Alex; Panousopoulos, Leonidas; Neda Mousavy-Gharavy, Seyedeh; Guerrero, Ana; Rashid, Mamunur; Jani, Nital; Goding, Colin R; Jacques, Thomas S; Adams, David J; Gil, Jesus; Andoniadou, Cynthia L; Martinez-Barbera, Juan Pedro

    2017-11-28

    Senescent cells may promote tumour progression through the activation of a senescence-associated secretory phenotype (SASP), whether these cells are capable of initiating tumourigenesis in vivo is not known. Expression of oncogenic β-catenin in Sox2+ young adult pituitary stem cells leads to formation of clusters of stem cells and induction of tumours resembling human adamantinomatous craniopharyngioma (ACP), derived from Sox2- cells in a paracrine manner. Here, we uncover the mechanisms underlying this paracrine tumourigenesis. We show that expression of oncogenic β-catenin in Hesx1+ embryonic precursors also results in stem cell clusters and paracrine tumours. We reveal that human and mouse clusters are analogous and share a common signature of senescence and SASP. Finally, we show that mice with reduced senescence and SASP responses exhibit decreased tumour-inducing potential. Together, we provide evidence that senescence and a stem cell-associated SASP drive cell transformation and tumour initiation in vivo in an age-dependent fashion.

  10. Hypothalamic Obesity in Craniopharyngioma Patients: Disturbed Energy Homeostasis Related to Extent of Hypothalamic Damage and Its Implication for Obesity Intervention

    Directory of Open Access Journals (Sweden)

    Christian L. Roth

    2015-09-01

    Full Text Available Hypothalamic obesity (HO occurs in patients with tumors and lesions in the medial hypothalamic region. Hypothalamic dysfunction can lead to hyperinsulinemia and leptin resistance. This review is focused on HO caused by craniopharyngiomas (CP, which are the most common childhood brain tumors of nonglial origin. Despite excellent overall survival rates, CP patients have substantially reduced quality of life because of significant long-term sequelae, notably severe obesity in about 50% of patients, leading to a high rate of cardiovascular mortality. Recent studies reported that both hyperphagia and decreased energy expenditure can contribute to severe obesity in HO patients. Recognized risk factors for severe obesity include large hypothalamic tumors or lesions affecting several medial and posterior hypothalamic nuclei that impact satiety signaling pathways. Structural damage in these nuclei often lead to hyperphagia, rapid weight gain, central insulin and leptin resistance, decreased sympathetic activity, low energy expenditure, and increased energy storage in adipose tissue. To date, most efforts to treat HO have shown disappointing long-term success rates. However, treatments based on the distinct pathophysiology of disturbed energy homeostasis related to CP may offer options for successful interventions in the future.

  11. Expression of SRY-related HMG Box Transcription Factors (Sox) 2 and 9 in Craniopharyngioma Subtypes and Surrounding Brain Tissue.

    Science.gov (United States)

    Thimsen, Vivian; John, Nora; Buchfelder, Michael; Flitsch, Jörg; Fahlbusch, Rudolf; Stefanits, Harald; Knosp, Engelbert; Losa, Marco; Buslei, Rolf; Hölsken, Annett

    2017-11-20

    Stem cells have been discovered as key players in the genesis of different neoplasms including craniopharyngioma (CP), a rare tumour entity in the sellar region. Sox2 and Sox9 are well-known stem cell markers involved in pituitary development. In this study we analysed the expression of both transcription factors using immunohistochemistry in a large cohort of 64 adamantinomatous (aCP) and 9 papillary CP (pCP) and quantitative PCR in 26 aCP and 7 pCP. Whereas immunohistochemically Sox2+ cells were verifiable in only five aCP (7.8%) and in 39.1% of the respective surrounding cerebral tissue, pCP specimens appeared always negative. In contrast, Sox9 was detectable in all tumours with a significantly higher expression in aCP compared to pCP (protein, p < 0.0001; mRNA p = 0.0484) This was also true for the respective tumour adjacent CNS where 63 aCP (98.4%) and six pCP (66.7%) showed Sox9+ cells. We further confirmed absence of Sox9 expression in nuclear β-catenin accumulating cells of aCP. Our results point to the conclusion that Sox2 and Sox9, seem to play essential roles not only in the specific formation of aCP, but also in processes involving the cerebral tumour environment, which needs to be illuminated in the future.

  12. Microstructure alterations in the hypothalamus in cranially radiated childhood leukaemia survivors but not in craniopharyngioma patients unaffected by hypothalamic damage.

    Science.gov (United States)

    Follin, Cecilia; Fjalldal, Sigridur; Svärd, Daniel; van Westen, Danielle; Gabery, Sanaz; Petersén, Åsa; Lätt, Jimmy; Rylander, Lars; Erfurth, Eva Marie

    2017-10-01

    Metabolic complications are frequent in childhood leukaemia (ALL) survivors treated with cranial radiotherapy (CRT). These complications are potentially mediated by damage to the hypothalamus (HT), as childhood onset (CO) craniopharyngioma (CP) survivors without HT involvement are spared overt obesity. Diffusion tensor imaging (DTI) shows brain tissue microstructure alterations, by fractional anisotrophy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD). We used DTI to determine the integrity of the microstructure of the HT in ALL survivors. Case-control study. Three groups were included: (i) 27 CRT treated ALL survivors on hormone supplementation, (ii) 17 CO-CP survivors on hormone supplementation but without HT involvement and (iii) 27 matched controls. DTI parameters of the HT were measured and body composition. Microstructural alterations in the HT were more severe in ALL survivors with a BMI ≥25 than with BMI <25. Compared to controls, ALL survivors had reduced FA (P=.04), increased MD (P<.001), AD (P<.001) and RD (P<.001) in the right and left HT. In the right HT, ALL survivors with a BMI ≥25 showed elevated MD (P=.03) and AD (P=.02) compared to ALL survivors with BMI <25. In contrast, DTI parameters did not differ between CP survivors and controls. Long-term follow-up after CRT for ALL DTI measures were affected in the HT despite complete hormone replacement. The present data suggest that ALL survivors have demyelination and axonal loss in the HT. © 2017 John Wiley & Sons Ltd.

  13. Spectroscopic techniques (Mössbauer spectrometry, NMR, ESR,…) as tools to resolve doubtful NMR images: Study of the craniopharyngioma tumor

    Science.gov (United States)

    Rimbert, J. N.; Dumas, F.; Lafargue, C.; Kellershohn, C.; Brunelle, F.; Lallemand, D.

    1990-07-01

    Craniopharyngioma, an intracranial tumor, exhibits hyperintensity in the Spin-Echo-T2-NMR image and a hyposignal in the SE-T1-image. However, in some cases (15-20% cases), hypersignals are seen in both SE-T1 and T2-MRI. Using spectroscopic techniques, Mössbauer spectrometry in particular, we have demonstrated that the T1 hypersignal is due to ferritin, dissolved in the cystic liquid, after tumor cell lysis, in the course of time. Other possible reasons inducing a shortening of the T1 relaxation time (presence of lipids, intratumoral hemorrhage) have been rejected.

  14. The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature

    International Nuclear Information System (INIS)

    Habrand, Jean-Louis; Ganry, Oliver; Couanet, Dominique; Rouxel, Valerie; Levy-Piedbois, Christine; Pierre-Kahn, Alain; Kalifa, Chantal

    1999-01-01

    Purpose: To review the outcome and quality of life at 5 years and more of 37 children treated with radiation therapy combined or not with surgical resection for a craniopharyngioma in a single institution. Methods and Materials: From January 1969 through December 1992, 37 children received external therapy at the Institut Gustave Roussy (Villejuif, France). Age ranged between 1 and 15 years (mean 7.4), M/F sex ratio was 0.76. In approximately one-half of the cases (18/37), radiation therapy was applied in conjunction with a surgical resection, and in almost one-half of the cases (18/37) as part of a salvage program following local failure. Total dose ranged between 45 and 56 Gy (median 50) given with a conventional fractionation in most children. Survival (S), event-free survival (EFS) were computerized according to the Kaplan-Meier method and prognostic factors for local failure and functional status analyzed. Functional outcome was evaluated according to the Wen score in 4 grades (gr 1: normal with/without hormonal replacement, gr 4: totally dependent, gr 2 and 3: intermediate disabilities). Results: At the time of analysis, 24 children (65%) were alive with NED, 4 (11%) alive after failure, and 9 (24%) dead of various causes. Following therapy, S and EFS regularly degraded and didn't seem to reach a plateau before 9 years (5 and 10 year S and EFS, respectively, 91, 65, and 78 and 56.5%). This was due to the occurrence of late failures (5 and 8.5 years) and late lethal complications (1 in-field glioblastoma multiforme at 9 years). A significant gain on EFS followed the introduction of modern imaging (p = 0.03), the association of surgical resection with RT (p = 0.01) and of higher doses of radiation superior or equal to 55 Gy (p = 0.05); a similar gain on S was observed in patients with a good initial performance status (p = 0.05). It is remarkable that surgical salvage of local failures following RT could induce prolonged remission in 4 children. Functional

  15. Analysis of short- and long-term metabolic effects of growth hormone replacement therapy in adult patients with craniopharyngioma and non-functioning pituitary adenoma.

    Science.gov (United States)

    Profka, E; Giavoli, C; Bergamaschi, S; Ferrante, E; Malchiodi, E; Sala, E; Verrua, E; Rodari, G; Filopanti, M; Beck-Peccoz, P; Spada, A

    2015-04-01

    Adult patients operated for craniopharyngioma develop more frequently GH deficiency (GHD) than patients operated for non-functioning pituitary adenoma (NFPA). The aim of the study was to compare both short- (1 year) and long-term (5 years) effects of rhGH in 38 GHD adult patients (19 operated for Craniopharyngioma (CP) and 19 for NFPA). IGF-I levels, body composition (BF%), BMI, lipid profile and glucose homeostasis were evaluated in all patients. Pituitary MRI was performed at baseline and during follow-up, as needed. At baseline no difference between the two groups was observed, apart from a higher prevalence of diabetes insipidus in CP patients (79 vs 21%). After 12 months, IGF-I SDS normalized and BF% significantly decreased only in the NFPA group. During long-term treatment, decrease in BF% and improvement in lipid profile shown by reduction in total- and LDL-cholesterol were present in NFPA group only, while increase in insulin levels and HbA1c and decrease of QUICKI were observed in CP patients only. Accordingly, after long-term therapy, the prevalence of metabolic syndrome (MS) was significantly higher in CP than in NFPA group (37% in CP and in 5% in NFPA group; p < 0.05). The present data suggest that CP patients are less sensitive to the positive rhGH effects on lipid profile and BF% and more prone to insulin sensitivity worsening than NFPA patients, resulting in increased prevalence of MS in CP only.

  16. N-terminal pro-B-type Natriuretic Peptide in three different mechanisms of dysnatremia onset after a child's craniopharyngioma surgery.

    Science.gov (United States)

    Spatenkova, Vera; Hradil, Jan; Suchomel, Petr

    2017-10-01

    Craniopharyngioma, due to its sellar location, can be perioperatively complicated by different types of dysnatremia. We present a rare postoperative onset of a combination of three different mechanisms of dysnatremia with N-terminal pro-B-type Natriuretic Peptide (NT-proBNP) and renal function parameters in a boy with a good outcome after craniopharyngioma surgery: 1/ Central diabetes insipidus (CDI) onset immediately after the operation, hypernatremia with peak serum sodium (SNa) 158 mmol/l) caused by free water polyuria (electrolyte-free water clearance, EWC 0.104 ml/s), NT-proBNP 350 pg/ml; 2/ cerebral salt wasting (CSW) onset on day 7, hyponatremia (SNa 128 mmol/l) with hypoosmolality (measured serum osmolality, SOsm 265 mmol/kg) caused by natriuresis (sodium - daily output 605 mmol/day, fractional excretion 0.035), NT-proBNP 191 pg/ml; 3/ Polydypsia onset on day 11 caused hyponatremia (SNa 132 mmol/l), EWC 0.015, NT-proBNP 68 pg/ml.

  17. Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes.

    Science.gov (United States)

    Sterkenburg, Anthe S; Hoffmann, Anika; Gebhardt, Ursel; Warmuth-Metz, Monika; Daubenbüchel, Anna M M; Müller, Hermann L

    2015-07-01

    Quality of life (QoL) and long-term prognosis are frequently, and often severely, impaired in craniopharyngioma (CP) patients. Knowledge of risk factors for long-term outcome is important for optimization of treatment. Overall survival (OS) and progression-free survival (PFS), body mass index (BMI), neuropsychological status (EORTCQLQ-C30, MFI-20), and psychosocial status were analyzed in 261 patients with childhood-onset CP diagnosed before 2000 and longitudinally observed in HIT-Endo. Twenty-year OS was lower (P = .006) in CP with hypothalamic involvement (HI) (n = 132; 0.84 ± 0.04) when compared with CP without HI (n = 82; 0.95 ± 0.04). OS was not related to degree of resection, sex, age at diagnosis, or year of diagnosis (before/after 1990). PFS (n = 168; 0.58 ± 0.05) was lower in younger patients (12 years of follow-up, patients with HI presented no further increase in BMI. QoL in CP patients with HI was impaired by obesity, physical fatigue, reduced motivation, dyspnea, diarrhea, and nonoptimal psychosocial development. OS and QoL are impaired by HI in long-term survivors of CP. HI is associated with severe obesity, which plateaus after 12 years. OS/PFS are not related to degree of resection, but gross-total resection should be avoided in cases of HI to prevent further hypothalamic damage, which exacerbates sequelae. © The Author(s) 2015. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  18. A membranous structure separating the adenohypophysis and neurohypophysis: an anatomical study and its clinical application for craniopharyngioma.

    Science.gov (United States)

    Lu, Yun-Tao; Qi, Song-Tao; Xu, Jia-Ming; Pan, Jun; Shi, Jin

    2015-06-01

    OBJECT This study aimed to identify the membranous septation between the adeno- and neurohypophysis. The clinical impact of this septation in the surgical removal of infradiaphragmatic craniopharyngioma (Id-CP) is also clarified. METHODS The sellar regions from 8 fetal and 6 adult cadavers were dissected. After staining first with H & E and then with picro-Sirius red, the membranous structures were observed and measured under normal light and polarization microscopy. The pre- and postsurgical images and intraoperative procedures in 28 cases of childhood Id-CP were reviewed and analyzed. RESULTS There is a significant membranous septation (termed the adenoneurohypophysis septation [ANHS]) lying behind the intermediate lobe to separate the adeno- and neurohypophysis. The average thicknesses are 21.9 ± 16.9 μm and 79.1 ± 43.2 μm in fetal and adult heads, respectively. The median segment of the septation is significantly thicker than the upper and lower segments. The ANHS extends from the suprasellar pars tuberalis to the sellar floor, where it is fused with the pituitary capsule. During Id-CP surgery performed via a transcranial approach, the ANHS can be identified to reserve the neurohypophysis. Moreover, by understanding the anatomy of this membrane, the pituitary stalk was preserved in 3 patients (10.7%). CONCLUSIONS There is a significant membrane separating the anterior and posterior lobes of the pituitary gland, which lies behind the intermediate lobe. Understanding the anatomy of this septation is important for identifying and preserving the neurohypophysis and pituitary stalk during Id-CP surgery.

  19. Outcome of Radical Surgical Resection for Craniopharyngioma with Hypothalamic Preservation: A Single-Center Retrospective Study of 1054 Patients.

    Science.gov (United States)

    Shi, Xiang'en; Zhou, Zhongqing; Wu, Bin; Zhang, Yongli; Qian, Hai; Sun, Yuming; Yang, Yang; Yu, Zaitao; Tang, Zhiwei; Lu, Shuaibin

    2017-06-01

    A retrospective review of the surgical outcome for patients with craniopharyngioma (CP) treated in a single neurosurgical center with surgical resection using visualization to ensure hypothalamic preservation. The study included 1054 patients. Before 2003, a pterional cranial approach was preferred for 78% of patients; after 2004, the unifrontal basal interhemispheric approach was performed in 79.1% of patients. Complete tumor resection was achieved in 89.6% of patients; vision improved in 47.1% of patients who had preoperative vision impairment. However, diabetes insipidus worsened in 70.4% of patients and new-onset diabetes insipidus occurred in 29.7% of the remaining patients. Pituitary stalk preservation occurred in 48.9% of cases. There were 89.6% of patients with total tumor removal; 13.3% of patients showed tumor recurrence within an average of 2.8 years. Of 69 follow-up patients with a subtotal or partial resection, 94.2% showed tumor recurrence within an average of 4.3 months. Of the total patients, 82.3% fully recovered. This study has shown that radical surgical resection of CP using microsurgical excision can be effective with a good patient outcome without more limitations on each individual tumor of distinct features despite the impact of recent endoscopic techniques on CP surgery. The surgical approach depends on a direct and wider visualization of CP located in the midline with preserving hypothalamic structures by identifying some hypothalamic landmark structures. After surgery, most patients can resume their normal activities even after aggressive tumor removal, although patients require postoperative hormonal replacement. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Quality of life and growth after childhood craniopharyngioma: results of the multinational trial KRANIOPHARYNGEOM 2007.

    Science.gov (United States)

    Heinks, Kerstin; Boekhoff, Svenja; Hoffmann, Anika; Warmuth-Metz, Monika; Eveslage, Maria; Peng, Junxiang; Calaminus, Gabriele; Müller, Hermann L

    2018-02-01

    Quality of life (QoL) after childhood-onset craniopharyngioma (CP) is frequently impaired due to tumor and/or treatment-related factors such as endocrine deficits and hypothalamic involvement/lesions. In a multinational trial, we prospectively analyzed parental and self-assessment of CP patient QoL at 3 months, 1 and 3 years after CP diagnosis related to growth hormone (GH) substitution. 47 of 194 CP recruited between 2007 and 2015 in KRANIOPHARYNGEOM 2007 were analyzed for QoL 1 and 3 years after CP diagnosis. QoL was assessed by Pediatric Quality of Life (PEDQOL) questionnaire and PEDQOL scores of parental and self-assessed QoL during 3 years follow-up after CP diagnosis were analyzed. Parents estimated QoL of their children worse than patients did themselves. GH substitution had no relevant effect on short-term weight and height development. CP patients GH-treated at 3 years follow-up presented at baseline (1 year after diagnosis, before GH substitution) with reduced self-assessed QoL when compared with GH non-treated CP. QoL stabilized during 1-3 years of follow-up in GH-treated patients, whereas non GH-treated patients experienced decreases in autonomy (p = 0.03), cognition (p = 0.01), and physical function (p = 0.04). Parents assess QoL in CP survivors worse than their children. GH substitution should be considered as a therapeutic option to ameliorate imminent impairments of QoL after CP.

  1. Relative Risks of Contributing Factors to Morbidity and Mortality in Adults With Craniopharyngioma on Growth Hormone Replacement.

    Science.gov (United States)

    Yuen, Kevin C J; Mattsson, Anders F; Burman, Pia; Erfurth, Eva-Marie; Camacho-Hubner, Cecilia; Fox, Janet L; Verhelst, Johan; Geffner, Mitchell E; Abs, Roger

    2018-02-01

    In adults, craniopharyngioma (CP) of either childhood-onset (CO-CP) or adult-onset (AO-CP) is associated with increased morbidity and mortality, but data on the relative risks (RRs) of contributing factors are lacking. To assess the RRs of factors contributing to morbidity and mortality in adults with CO-CP and AO-CP. Data on 1669 patients with CP from KIMS (Pfizer International Metabolic Database) were analyzed using univariate and multiple Poisson and Cox regression methods. When CO-CP and AO-CP groups were combined, history of stroke and hyperlipidemia increased cardiovascular risk, higher body mass index (BMI) and radiotherapy increased cerebrovascular risk, and increased waist circumference increased the risk of developing diabetes mellitus (DM). Compared with patients with CO-CP, patients with AO-CP had a threefold higher risk of tumor recurrence, whereas being female and previous radiotherapy exposure conferred lower risks. Radiotherapy and older age with every 10 years from disease onset conferred a 2.3- to 3.5-fold risk for developing new intracranial tumors, whereas older age, greater and/or increasing BMI, history of stroke, and lower insulinlike growth factor I (IGF-I) standard deviation score measured at last sampling before death were related to increased all-cause mortality. Compared with the general population, adults with CP had 9.3-, 8.1-, and 2.2-fold risks of developing DM, new intracranial tumors, and early death, respectively. Conventional factors that increase the risks of cardio- and cerebrovascular diseases and DM and risks for developing new intracranial tumors contributed to excess morbidity and mortality. In addition, lower serum IGF-I level measured from the last sample before death was inversely associated with mortality risk in patients with CP. Copyright © 2017 Endocrine Society

  2. The development of hypothalamic obesity in craniopharyngioma patients: A risk factor analysis in a well-defined cohort.

    Science.gov (United States)

    van Iersel, Laura; Meijneke, Ruud W H; Schouten-van Meeteren, Antoinette Y N; Reneman, Liesbeth; de Win, Maartje M; van Trotsenburg, A S Paul; Bisschop, Peter H; Finken, Martijn J J; Vandertop, W Peter; van Furth, Wouter R; van Santen, Hanneke M

    2018-05-01

    Hypothalamic obesity (HO) is a major concern in patients treated for craniopharyngioma (CP). The influence of degree of resection on development of HO, event-free survival (EFS), and neuroendocrine sequelae is an issue of debate. A retrospective cohort consisting of all CP patients treated between 2002 and 2012 in two university hospitals was identified. Multivariable logistic regression was used to study the associations between preoperative BMI, age at diagnosis, tumor volume, performed surgical resection, and presence of HO at follow-up. Thirty-five patients (21 children and 14 adults) were included. Median follow-up time was 35.6 months (4.1-114.7). Four patients were obese at diagnosis. HO was present in 19 (54.3%) patients at last follow-up of whom eight were morbidly obese. Thirteen (37.1%) patients underwent partial resection (PR) and 22 (62.9%) gross total resection (GTR). GTR was related to HO (OR 9.19, 95% CI 1.43-59.01), but for morbid HO, obesity at diagnosis was the only risk factor (OR 12.92, 95% CI 1.05-158.73). EFS in patients after GTR was 86%, compared to 42% after PR (log-rank 9.2, P = 0.003). Adjuvant radiotherapy after PR improved EFS (log-rank 8.2, P = 0.004). Panhypopituitarism, present in 15 patients, was mainly seen after GTR. HO is less frequent after PR than after GTR, but PR cannot always prevent the development of morbid obesity in patients with obesity at diagnosis. PR reduces the occurrence of panhypopituitarism. When developing a treatment algorithm, all these factors should be considered. © 2018 Wiley Periodicals, Inc.

  3. Preoperative Assessment of Craniopharyngioma Adherence: Magnetic Resonance Imaging Findings Correlated with the Severity of Tumor Attachment to the Hypothalamus.

    Science.gov (United States)

    Prieto, Ruth; Pascual, José M; Rosdolsky, Maria; Barrios, Laura

    2018-02-01

    Craniopharyngioma (CP) adherence represents a heterogeneous pathologic feature that critically influences the potentially safe and radical resection. The aim of this study was to define the magnetic resonance imaging (MRI) predictors of CP adherence severity. This study retrospectively investigated a cohort of 200 surgically treated CPs with their corresponding preoperative conventional MRI scans. MRI findings related to the distortions of anatomic structures along the sella turcica-third ventricle axis caused by CPs, in addition to the tumor's shape and calcifications, were analyzed and correlated with the definitive type of CP adherence observed during the surgical procedures. CP adherence is defined by 3 components, as follows: 1) the specific structures attached to the tumor, 2) the adhesion's extent, and 3) its strength. Combination of these 3 components determines 5 hierarchical levels of adherence severity with gradually increasing surgical risk of hypothalamic injury. Multivariate analysis identified 4 radiologic variables that allowed a correct overall prediction of the levels of CP adherence severity in 81.5% of cases: 1) the position of the hypothalamus in relation to the tumor-the most discriminant factor; 2) the type of pituitary stalk distortion; 3) the tumor shape; and 4) the presence of calcifications. A binary logistic regression model including the first 3 radiologic variables correctly identified the CPs showing the highest level of adherence severity (severe/critical) in almost 90% of cases. A position of the hypothalamus around the middle portion of the tumor, an amputated or infiltrated appearance of the pituitary stalk, and the elliptical shape of the tumor are reliable predictors of strong and extensive CP adhesions to the hypothalamus. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Effects of dark brooders and overhangs on free-range use and behaviour of slow-growing broilers

    NARCIS (Netherlands)

    Stadig, L.M.; Rodenburg, T.B.; Reubens, B.; Ampe, B.; Tuyttens, F.A.M.

    2017-01-01

    Broiler chickens often make limited use of the free-range area. Range use is influenced by type of shelter available. Range use may possibly be improved by a more gradual transition from the house to the range and by using dark brooders (secluded warm, dark areas in the home pen) that mimic aspects

  5. Spontaneous and induced loss of chromosomes in slow-growing somatic hybrid calli of Solanum tuberosum and Nicotiana plumbaginifolia

    NARCIS (Netherlands)

    Tempelaar, MJ; Drenth - Diephuis, L.J.; SAAT, TAWM; Jacobsen, E.

    Rate and extent of spontaneous and induced chromosome loss have been determined at the callus level of somatic hybrids of mutants of Solanum tuberosum and Nicotiana plumbaginifolia. AEC (amino ethyl cystein) resistance in potato and Nitrate-Reductase deficiency in N. plumbaginifolia have been used

  6. Mycobacterium angelicum sp. nov., a non-chromogenic, slow-growing species isolated from fish and related to Mycobacterium szulgai.

    Science.gov (United States)

    Pourahmad, Fazel; Pate, Mateja; Ocepek, Matjaž; Borroni, Emanuele; Cabibbe, Andrea M; Capitolo, Eleonora; Cittaro, Davide; Frizzera, Eliana; Jenčič, Vlasta; Mariottini, Alessandro; Marumo, Kenji; Vaggelli, Guendalina; Cirillo, Daniela M; Tortoli, Enrico

    2015-12-01

    The name 'Mycobacterium angelicum' dates back to 2003 when it was suggested for a slowly growing mycobacterium isolated from freshwater angelfish. This name is revived here and the novel species is proposed on the basis of the polyphasic characterization of four strains including the original one. The four strains presented 100 % 16S rRNA gene sequence similarity with Mycobacterium szulgai but clearly differed from M. szulgai for the milky white aspect of the colonies. The sequence similarity with the type strain of M. szulgai ranged, in eight additionally investigated genetic targets, from 78.9 to 94.3 %, an evident contrast with the close relatedness that emerged at the level of 16S rRNA gene. The average nucleotide identity between the genomes of M. szulgai DSM 44166T and strain 126/5/03T (type strain of the novel species) was 92.92 %, and supported the status of independent species. The confirmation of the name Mycobacterium angelicum sp. nov. is proposed, with strain 126/5/03T ( = CIP 109313T = DSM 45057T) as the type strain.

  7. Deep Sequencing Analysis of miRNA Expression in Breast Muscle of Fast-Growing and Slow-Growing Broilers

    Directory of Open Access Journals (Sweden)

    Hongjia Ouyang

    2015-07-01

    Full Text Available Growth performance is an important economic trait in chicken. MicroRNAs (miRNAs have been shown to play important roles in various biological processes, but their functions in chicken growth are not yet clear. To investigate the function of miRNAs in chicken growth, breast muscle tissues of the two-tail samples (highest and lowest body weight from Recessive White Rock (WRR and Xinghua Chickens (XH were performed on high throughput small RNA deep sequencing. In this study, a total of 921 miRNAs were identified, including 733 known mature miRNAs and 188 novel miRNAs. There were 200, 279, 257 and 297 differentially expressed miRNAs in the comparisons of WRRh vs. WRRl, WRRh vs. XHh, WRRl vs. XHl, and XHh vs. XHl group, respectively. A total of 22 highly differentially expressed miRNAs (fold change > 2 or < 0.5; p-value < 0.05; q-value < 0.01, which also have abundant expression (read counts > 1000 were found in our comparisons. As far as two analyses (WRRh vs. WRRl, and XHh vs. XHl are concerned, we found 80 common differentially expressed miRNAs, while 110 miRNAs were found in WRRh vs. XHh and WRRl vs. XHl. Furthermore, 26 common miRNAs were identified among all four comparisons. Four differentially expressed miRNAs (miR-223, miR-16, miR-205a and miR-222b-5p were validated by quantitative real-time RT-PCR (qRT-PCR. Regulatory networks of interactions among miRNAs and their targets were constructed using integrative miRNA target-prediction and network-analysis. Growth hormone receptor (GHR was confirmed as a target of miR-146b-3p by dual-luciferase assay and qPCR, indicating that miR-34c, miR-223, miR-146b-3p, miR-21 and miR-205a are key growth-related target genes in the network. These miRNAs are proposed as candidate miRNAs for future studies concerning miRNA-target function on regulation of chicken growth.

  8. Extended Endoscopic Endonasal Resection of a Suprasellar and Third Ventricular Retrochiasmatic Craniopharyngioma with a Narrow Pituitary Gland-Optic Chiasm Interval: Techniques to Optimize Resection.

    Science.gov (United States)

    Kenning, Tyler J; Pinheiro-Neto, Carlos D

    2018-04-01

    The extended endoscopic endonasal approach can be utilized to surgically treat pathology within the suprasellar space. This relies on a sufficient corridor and interval between the superior aspect of the pituitary gland and the optic chiasm. Tumors located in the retrochiasmatic space and within the third ventricle, however, may not have a widened interval through which to work. With mass effect on the superior and posterior aspect of the optic chiasm, the corridor between the chiasm and the pituitary gland might even be further narrowed. This may negate the possibility of utilizing the endoscopic endonasal approach for the management of pathology in this location. We present a case of a retrochiasmatic craniopharyngioma with a narrow resection corridor that was treated with the extended endoscopic approach and we review techniques to potentially overcome this limitation. The link to the video can be found at: https://youtu.be/ogRZj-aBqeQ .

  9. A flexible endoscope-assisted interhemispheric transcallosal approach through the contralateral ventricle for the removal of a third ventricle craniopharyngioma: A technical report.

    Science.gov (United States)

    Yano, Shigetoshi; Hide, Takuichiro; Shinojima, Naoki; Ueda, Yutaka; Kuratsu, Jun-Ichi

    2015-01-01

    Intraventricular craniopharyngiomas are difficult to remove. We combined an interhemispheric transcallosal approach with a flexible endoscope (videoscope) for successful tumor removal. A 52-year-old male complained of general fatigue and memory disturbance. Magnetic resonance imaging revealed a well-enhanced third ventricle mass with dilatation of lateral ventricles. During removal with the interhemispheric transcallosal approach, a videoscope that was inserted into the left lateral ventricle revealed the interface of the tumor and the ventricular wall. The tumor was pushed to the right using forceps and removed totally through the right foramen of Monro without any fornix injury. This procedure is a safe option for removing third ventricular tumors especially in the case with hydrocephalus.

  10. Carbohydrate-lipid profile and use of metformin with micronized fenofibrate in reducing metabolic consequences of craniopharyngioma treatment in children: single institution experience.

    Science.gov (United States)

    Kalina, Maria Aleksandra; Wilczek, Marta; Kalina-Faska, Barbara; Skała-Zamorowska, Eliza; Mandera, Marek; Małecka Tendera, Ewa

    2015-01-01

    To evaluate auxology and metabolic disturbances in children with craniopharyngioma, and to present observational results of treatment of metabolic sequels with metformin and micronized fenofibrate. The studied group comprised 22 children [median age at diagnosis 10.5 (0.17-16.75) years; median follow-up 5.1 years]. Assessment included height standard deviations (SDS), body mass index (BMI) SDS, concentrations of lipids, glucose and insulin (fasting or oral glucose tolerance test) and homeostatic model assessment of insulin resistance (HOMA-IR) index. Ten adolescents with hyperinsulinemia and dyslipidemia received therapy with metformin (500-1500 mg/daily) and micronized fenofibrate (160 mg/daily). At diagnosis, median hSDS was -1.66 (range: -4.08; +0.1). Nine (40.9%) children were growth hormone-treated. There was gradual increase of BMI SDS, 18 (81.8%) patients being overweight at the final assessment. Dyslipidaemia was found in 19 patients (86.4%), hyperinsulinaemia in 11 patients (50%) and elevated HOMA-IR in 15 patients (68.2%). Decrease of triglycerides [median 263.5 (171-362) mg/dL vs. 154 (102-183) mg/dL] and HOMA-IR [8.64 (5.08-12.65) vs. 4.68 (0.7-7.9)] was significant in the group treated with metformin and fenofibrate for 6 months. Significant auxologic changes and metabolic abnormalities were found in children treated for craniopharyngioma. The use of metformin and fenofibrate seemed to attenuate these disturbances in a short-term observation.

  11. Combined proton and photon irradiation for craniopharyngioma: Long-term results of the early cohort of patients treated at Harvard Cyclotron Laboratory and Massachusetts General Hospital

    International Nuclear Information System (INIS)

    Fitzek, Markus M.; Linggood, Rita M.; Adams, Judy; Munzenrider, John E.

    2006-01-01

    Purpose: We report the results of the early cohort of patients treated for craniopharyngioma with combined proton-photon irradiation at the Massachusetts General Hospital and the Harvard Cyclotron Laboratory. Methods and Materials: Between 1981 and 1988, 15 patients with craniopharyngioma were treated in part or entirely with fractionated 160 MeV proton beam therapy. The group consisted of 5 children (median age, 15.9 years) and 10 adults (median age, 36.2 years). Median dose prescribed to the tumor was 56.9 cobalt Gray equivalent (CGE; 1 proton Gray = 1.1 CGE). The median proton component was 26.9 CGE. Patients were treated after documented recurrence after initial surgery (n = 6) or after subtotal resection or biopsy (n = 9). None had had prior radiation therapy. Results: Median observation period of surviving patients (n = 11) was 13.1 years from radiotherapy. One patient was lost to follow-up with tumor control after 5.2 years. Actuarial 10-year survival rate was 72%. Four patients have died 5-9.1 years after treatment, two from local failure. Actuarial 5- and 10-year local control rates were 93% and 85%, respectively. The functional status of the living adult patients is unaltered from their preradiotherapy status; all of them continued leading normal or near normal working lives. None of the patients treated as a child had experienced recurrence of tumor. One child shows learning difficulties and slight retardation, comparable to his preradiotherapy status. The others have professional achievements within the normal range. Conclusion: Results in terms of survival and local control are comparable with other contemporary series. Although no formal neuropsychological testing was performed, the surrogate measures of lifestyle and professional accomplishments appear to be satisfactory

  12. Pilocytic astrocytoma following radiotherapy for craniopharyngioma: case report Astrocitoma pilocítico pós-radioterapia para tratamento de craniofaringioma: relato de caso

    Directory of Open Access Journals (Sweden)

    LEONARDO BONILHA

    2000-09-01

    Full Text Available Administration of fractionated doses of irradiation is part of the adjutant therapy for CNS tumours such as craniopharyngiomas and pituitary adenomas. It can maximise cure rates or expand symptom-free period. Among the adverse effects of radiotherapy, the induction of a new tumour within the irradiated field has been frequently described. The precise clinical features that correlate irradiation and oncogenesis are not completely defined, but some authors have suggested that tumors are radiation induced when they are histologically different from the treated ones, arise in greater frequency in irradiated patients than among normal population and tend to occur in younger people with an unusual aggressiveness. In this article, we report a case of a papillary astrocytoma arising in a rather unusual latency period following radiotherapy for craniopharyngioma.A administração de doses fracionadas de radiação é parte do tratamento adjuvante de tumores do sistema nervoso central como craniofaringiomas ou adenomas hipofisários. A radioterapia pode aumentar as chances de cura ou expandir o período livre de sintomas. Dentre seus efeitos adversos, é conhecida a possibilidade de aparecimento de novo tumor na região irradiada. Os critérios que definem se um novo tumor é de fato induzido por radiação ainda não são completamente definidos. Adimite-se, no entanto, que se o novo tumor é histologicamente diferente do primeiro, aparece em indivíduos mais jovens e é mais agressivo que o habitual, provavelmente sua origem é devida à oncogênese da irradiação. Descrevemos um caso de astrocitoma pilocítico possivelmente induzido por radioterapia, com surgimento em tempo de latência inesperadamente curto.

  13. Non-coplanar volumetric-modulated arc therapy (VMAT) for craniopharyngiomas reduces radiation doses to the bilateral hippocampus: a planning study comparing dynamic conformal arc therapy, coplanar VMAT, and non-coplanar VMAT

    International Nuclear Information System (INIS)

    Uto, Megumi; Mizowaki, Takashi; Ogura, Kengo; Hiraoka, Masahiro

    2016-01-01

    Recent studies suggest that radiation-induced injuries to the hippocampus play important roles in compromising neurocognitive functioning for patients with brain tumors and it could be important to spare the hippocampus using modern planning methods for patients with craniopharyngiomas. As bilateral hippocampus are located on the same level as the planning target volume (PTV) in patients with craniopharyngioma, it seems possible to reduce doses to hippocampus using non-coplanar beams. While the use of non-coplanar beams in volumetric-modulated arc therapy (VMAT) of malignant intracranial tumors has recently been reported, no dosimetric comparison has yet been made between VMAT using non-coplanar arcs (ncVMAT) and VMAT employing only coplanar arcs (coVMAT) among patients with craniopharyngiomas. We performed a planning study comparing dose distributions to the PTV, hippocampus, and other organs at risk (OAR) of dynamic conformal arc therapy (DCAT), coVMAT, and ncVMAT. DCAT, coVMAT, and ncVMAT plans were created for 10 patients with craniopharyngiomas. The prescription dose was 52.2 Gy in 29 fractions, and 99 % of each PTV was covered by 90 % of the prescribed dose. The maximum dose was held below 107 % of the prescribed dose. CoVMAT and ncVMAT plans were formulated to satisfy the following criteria: the doses to the hippocampus were minimized, and the doses to the OAR were similar to or lower than those of DCAT. The mean equivalent doses in 2-Gy fractions to 40 % of the volumes of the bilateral hippocampus [EQD 2 (40% hippos )] were 15.4/10.8/6.5 Gy for DCAT/coVMAT/ncVMAT, respectively. The EQD 2 (40% hippos ) for ncVMAT were <7.3 Gy, which is the threshold predicting cognitive impairment, as defined by Gondi et al.. The mean doses to normal brain tissue and the conformity indices were similar for the three plans, and the homogeneity indices were significantly better for coVMAT and ncVMAT compared with DCAT. NcVMAT is more appropriate than DCAT and coVMAT for

  14. Stages of Childhood Craniopharyngioma

    Science.gov (United States)

    ... and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves ). Enlarge Anatomy ... field of vision (the total area in which objects can be seen). This test measures both central ...

  15. Childhood Craniopharyngioma Treatment

    Science.gov (United States)

    ... and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves ). Enlarge Anatomy ... field of vision (the total area in which objects can be seen). This test measures both central ...

  16. MAPK pathway control of stem cell proliferation and differentiation in the embryonic pituitary provides insights into the pathogenesis of papillary craniopharyngioma.

    Science.gov (United States)

    Haston, Scott; Pozzi, Sara; Carreno, Gabriela; Manshaei, Saba; Panousopoulos, Leonidas; Gonzalez-Meljem, Jose Mario; Apps, John R; Virasami, Alex; Thavaraj, Selvam; Gutteridge, Alice; Forshew, Tim; Marais, Richard; Brandner, Sebastian; Jacques, Thomas S; Andoniadou, Cynthia L; Martinez-Barbera, Juan Pedro

    2017-06-15

    Despite the importance of the RAS-RAF-MAPK pathway in normal physiology and disease of numerous organs, its role during pituitary development and tumourigenesis remains largely unknown. Here, we show that the over-activation of the MAPK pathway, through conditional expression of the gain-of-function alleles BrafV600E and KrasG12D in the developing mouse pituitary, results in severe hyperplasia and abnormal morphogenesis of the gland by the end of gestation. Cell-lineage commitment and terminal differentiation are disrupted, leading to a significant reduction in numbers of most of the hormone-producing cells before birth, with the exception of corticotrophs. Of note, Sox2 + stem cells and clonogenic potential are drastically increased in the mutant pituitaries. Finally, we reveal that papillary craniopharyngioma (PCP), a benign human pituitary tumour harbouring BRAF p.V600E also contains Sox2 + cells with sustained proliferative capacity and disrupted pituitary differentiation. Together, our data demonstrate a crucial function of the MAPK pathway in controlling the balance between proliferation and differentiation of Sox2 + cells and suggest that persistent proliferative capacity of Sox2 + cells may underlie the pathogenesis of PCP. © 2017. Published by The Company of Biologists Ltd.

  17. Norman M. Dott, master of hypothalamic craniopharyngioma surgery: the decisive mentoring of Harvey Cushing and Percival Bailey at Peter Bent Brigham Hospital.

    Science.gov (United States)

    Prieto, Ruth; Pascual, José M

    2017-10-01

    Norman McOmish Dott (1897-1973) developed surgical neurology in Edinburgh, Scotland, and was a scholar of worldwide renown. One of Dott's most notable contributions to neurosurgery was his understanding of hypothalamic physiology, mostly acquired through the comprehensive study of patients with lesions involving this region of the diencephalon, particularly craniopharyngiomas (CPs). Recognition of symptoms caused by hypothalamic disturbances allowed him to predict the accurate anatomical relationships between CPs and the hypothalamus, despite the rudimentary radiological methods available during the 1930s. His sophisticated knowledge permitted Dott to perform radical removals of CPs originating within the third ventricle floor with acceptable success. Between 1934 and 1937, he operated on 4 CP cases originating in the hypothalamus, achieving a satisfactory postoperative outcome in 3 of the 4 patients. Aware of the strong attachment of hypothalamic CPs to the infundibulo-tuberal area, Dott used a double transbasal and transventricular approach to these lesions, a strategy providing an optimal view and control of the tumor boundaries. The decisive mentorship of several legendary figures of physiology and neurosurgery greatly influenced Dott's surgical evolution. The experimental pituitary gland work he performed with Sir Edward Sharpey-Schäfer at the beginning of his career stirred Dott's curiosity about the issue of hypothalamus-pituitary relationships. As a result, he decided to move to Peter Bent Brigham Hospital (Boston, Massachusetts) in 1923, to train in neurosurgery and neuropathology under the guidance of the leaders in these fields, Harvey Williams Cushing (1869-1939) and Percival Sylvester Bailey (1892-1973). They inspired the young Dott and shared with him their clinical and pathological expertise, in addition to their surgical strategies for best approaching and removing these challenging tumors. In time, Dott would come to surpass his mentors. This

  18. Dosimetry for the treatment of cystic craniopharyngioma through radiocolloids with {sup 186} Re; Dosimetria para el tratamiento de craneofaringiomas quisticos mediante radiocoloide con {sup 186} Re

    Energy Technology Data Exchange (ETDEWEB)

    Rojas, E.L.; Al-dweri, F.M.O.; Lallena, A.M. [Depto. de Fisica Moderna, Universidad de Granada (Spain); Bodineau, C.; Galan, P. [Servicio de Radiofisica Hospitalaria, Hospital Regional Universitario ' Carlos Haya' , 29010 Malaga (Spain)

    2003-07-01

    The cystic craniopharyngioma (CQ) are histologically benign tumors that can affect important organs, as the hypothalamus or the optic nerve. They are treated introducing, inside the cyst, a radioactive colloid. The wall constitutes the white volume and the dosimetry is usually carried out starting from analytic formulas that only are valid in the case of an homogeneous media, uniform and infinite. In this work it is studied the dosimetry of the CQ by means of Monte Carlo simulation (MC), taking in account the different materials and interfaces that conform them. We present the obtained results for the radionuclide {sup 186} Re. We have used the Monte Carlo code Penelope and we follow 5x10{sup 6} histories in each simulation. We supposed a size of CQ of 1.75 cm of radio and a wall thickness of 1 mm and we have varied the constituent materials of the interior of the cyst and of the wall. The analytic calculations that we carry out show an excellent agreement with the MC results for an unique media (water), as much for the beta radiation as for those originated of the Rhenium disintegration. However, when it takes in account the gel that is introduced in the tumor for the treatment and we vary the constituent material of the wall, we find important differences. Of the analysis of our results we can conclude that the dosimetry for the treatment of CQ based in the usual analytic formulas overestimates the doses really deposited in the wall of the CQ. (Author)

  19. Predicting pituitary stalk position by in vivo visualization of the hypothalamo-hypophyseal tract in craniopharyngioma using diffusion tensor imaging tractography.

    Science.gov (United States)

    Wang, Fuyu; Jiang, Jinli; Zhang, Jiashu; Wang, Qun

    2018-07-01

    The pituitary stalk (PS) is crucial to endocrine function and water-electrolyte equilibrium. Preservation of the PS during craniopharyngioma (CP) surgery is critical; however, in a pathological state, it is difficult to identify. The hypothalamo-hypophyseal tract (HHT) connects the hypothalamus and the posterior pituitary gland and projects through the PS. Thus, visualization of the HHT can help locate the PS. Preoperative visualization of the neural fasciculus has been widely achieved using diffusion tensor imaging (DTI) tractography. Therefore, this study evaluated the use of DTI tractography to identify and characterize the human HHT. We used DTI tractography to track the HHT in 10 patients with CP and compared the location of the tract with the intraoperative view of the PS in these patients. We successfully trac