Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome.

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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.

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

Prevalence of Neurobehavioral, Social, and Emotional Dysfunction in Patients Treated for Childhood Craniopharyngioma: A Systematic Literature Review

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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

Prevalence of neurobehavioral, social, and emotional dysfunction in patients treated for childhood craniopharyngioma: a systematic literature review.

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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.

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

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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.

Effects of T3 treatment on brown adipose tissue and energy expenditure in a patient with craniopharyngioma and hypothalamic obesity.

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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.

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

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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.

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

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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.

Craniopharyngioma: a clinicopathological study of 141 cases.

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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.

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

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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.

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

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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

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

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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

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

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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

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

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

CURRENT PRINCIPLES FOR CRANIOPHARYNGIOMA TREATMENT

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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. 

Diagnostics, treatment, and follow-up in craniopharyngioma

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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.

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

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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.

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

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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.

Treatment of cystic craniopharyngioma with phosphorus-32 intracavitary irradiation

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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

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

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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

Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients

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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.

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

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

Intracystic Therapies for Cystic Craniopharyngioma

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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.

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,

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

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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.

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

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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.

Role of radiation treatment in craniopharyngioma

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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.

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

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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.

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.

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

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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

Energy expenditure in obesity associated with craniopharyngioma

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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

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

Treatment of craniopharyngioma estimated by follow-up CT

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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.

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)

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

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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

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

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

Craniopharyngioma: treatment by conservative surgery and radiation therapy.

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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.

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

Obesity and craniopharyngioma

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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.

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

Fusiform dilatation of the internal carotid artery following childhood craniopharyngioma resection treated by endovascular flow diversion-A case report and literature review.

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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.

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

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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.

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)

Parent observed neuro-behavioral and pro-social improvements with oxytocin following surgical resection of craniopharyngioma.

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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.

The limits of transsellar/transtuberculum surgery for craniopharyngioma.

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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.

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

Primary Ectopic Ethmoidal Craniopharyngioma.

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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.

Pituitary Tumors in Childhood: an update in their diagnosis, treatment and molecular genetics

OpenAIRE

Keil, Margaret F.; Stratakis, Constantine A.

2008-01-01

Pituitary tumors are rare in childhood and adolescence, with a reported prevalence of up to 1 per million children. Only 2 - 6% of surgically treated pituitary tumors occur in children. Although pituitary tumors in children are almost never malignant and hormonal secretion is rare, these tumors may result in significant morbidity. Tumors within the pituitary fossa are of two types mainly, craniopharyngiomas and adenomas; craniopharyngiomas cause symptoms by compressing normal pituitary, causi...

Malignant Transformation of Radiotherapy-Naïve Craniopharyngioma.

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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.

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

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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.

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 ...

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)

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.

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

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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.

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

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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.

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)

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

Craniopharyngioma - Transnasal Endoscopic Approach

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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.

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

Treatment Options for Childhood Craniopharyngioma

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... factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment options ... the brain where it was first found. Treatment Option Overview Key Points There are different types of ...

Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

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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

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...

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)

Endoscopic Endonasal Management of Craniopharyngioma.

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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.

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)

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

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

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.

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.

BRAF V600E mutations in papillary craniopharyngioma

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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

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)

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 ...

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)

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.

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.

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.

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.

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

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.

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

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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.

[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.

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

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.

Primary ectopic frontotemporal extradural craniopharyngioma

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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.

Craniopharyngioma: Survivin expression and ultrastructure

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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

Interstitial irradiation for craniopharyngioma

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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)

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.

Phosphorus-32 therapy for cystic craniopharyngiomas

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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.

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

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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...

Clinical equipoise: protons and the child with craniopharyngioma

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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.

Clinical equipoise: Protons and the child with craniopharyngioma.

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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.

Craniopharyngioma

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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.

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

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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

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

Quality of life and growth after childhood craniopharyngioma: results of the multinational trial KRANIOPHARYNGEOM 2007.

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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.

Craniopharyngioma: a roadmap for scientific translation.

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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.

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

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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.

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

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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

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

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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

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

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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.

Cushing's disease and craniopharyngioma.

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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

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)

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)

Hypothalamic glioma masquerading as craniopharyngioma

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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.

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

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.

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

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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.

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.

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

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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.

Metastatic craniopharyngioma: case report and literature review.

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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.

[Acute visual loss in pregnancy caused by craniopharyngioma].

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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.

Endocavitary treatment of craniopharyngioma cysts by 186-rhenium. Traitement endocavitaire par le rhenium 186 des kystes de craniopharyngiomes

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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).

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.

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.

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

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.

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

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

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 ...

Potential evolution of neurosurgical treatment paradigms for craniopharyngioma based on genomic and transcriptomic characteristics.

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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.

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

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

, aspiration of cystic fluid in 2-day interval for continuously 7-10 days, and the delayed tumor resection. This combined treatment modality maybe an effective method to treat children with giant cystic craniopharyngiomas.

MR findings of craniopharyngioma

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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.

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

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...

Early Ectopic Recurrence of Craniopharyngioma in the Cerebellopontine Angle.

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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.

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

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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.

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

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

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

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 ...

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.)

Infrasellar craniopharyngioma

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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.

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.

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

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

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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

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

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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.

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

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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 .

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

Pre- and postoperative MR imaging of craniopharyngiomas

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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.).

Peculiarities of radiation induced craniopharyngioma

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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.

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

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...

CCT and sonographic findings in congenital craniopharyngioma

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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.

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.

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.

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

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.)

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

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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.

A Cross-Sectional Cohort Study of Cerebrovascular Disease and Late Effects After Radiation Therapy for Craniopharyngioma.

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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.

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

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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.

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

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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

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.

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.)

CRANIOPHARYNGIOMA PRESENTING AS ‘MANIA’—CASE REPORT

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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

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 .

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.

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)

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.

Pituitary Tumors in Childhood: an update in their diagnosis, treatment and molecular genetics

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Keil, Margaret F.; Stratakis, Constantine A.

2009-01-01

Pituitary tumors are rare in childhood and adolescence, with a reported prevalence of up to 1 per million children. Only 2 - 6% of surgically treated pituitary tumors occur in children. Although pituitary tumors in children are almost never malignant and hormonal secretion is rare, these tumors may result in significant morbidity. Tumors within the pituitary fossa are of two types mainly, craniopharyngiomas and adenomas; craniopharyngiomas cause symptoms by compressing normal pituitary, causing hormonal deficiencies and producing mass effects on surrounding tissues and the brain; adenomas produce a variety of hormonal conditions such as hyperprolactinemia, Cushing disease and acromegaly or gigantism. Little is known about the genetic causes of sporadic lesions, which comprise the majority of pituitary tumors, but in children, more frequently than in adults, pituitary tumors may be a manifestation of genetic conditions such as multiple endocrine neoplasia type 1 (MEN 1), Carney complex, familial isolated pituitary adenoma (FIPA), and McCune-Albright syndrome. The study of pituitary tumorigenesis in the context of these genetic syndromes has advanced our knowledge of the molecular basis of pituitary tumors and may lead to new therapeutic developments. PMID:18416659

CT and MR imaging of craniopharyngioma

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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.)

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.)

ON THE BENEFITS AND RISKS OF PROTON THERAPY IN PEDIATRIC CRANIOPHARYNGIOMA

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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

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

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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).

Microstructure alterations in the hypothalamus in cranially radiated childhood leukaemia survivors but not in craniopharyngioma patients unaffected by hypothalamic damage.

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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.

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

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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...

Craniopharyngioma with hyperprolactinaemia due to a prolactinoma.

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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

Malignant astrocytoma following radiotherapy for craniopharyngioma

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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.

Radiation therapy in the management of childhood cancer

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Kun, L.E.

1987-01-01

Over the past two decades, multimodality treatment regimens have produced significant improvement in survival rates for most types of childhood cancer. The role of radiation therapy has been critically evaluated in prospective clinical trials that established the importance of irradiation in assuring local and regional control of disease central to ultimate survival. Indications for cranial and craniospinal irradiation in acute lymphoblastic leukemia are reviewed, as is difficult technical factors important for successful management. The role of radiation therapy in neuroblastoma and Wilms tumor is reviewed in the context of tumor biology and increasing data from multi-institutional trials. Interactions of irradiation with surgery and chemotherapy are stressed in childhood rhabdomyosarcoma and Ewing sarcoma. Current results in the more common central nervous tumors of childhood are presented, including the central role of radiation therapy in medulloblastoma, astrocytoma, and craniopharyngioma. Concerns regarding late effects of radiation therapy are balanced with the importance of achieving disease control

MR differentiation of craniopharyngioma from pituitary macroadenoma

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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.

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

MR and CT findings of craniopharyngioma during and after radiation therapy

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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.)

MR and CT findings of craniopharyngioma during and after radiation therapy

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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.)

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

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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.

Intrinsic third ventricular craniopharyngioma: A case report

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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.

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

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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.

Efficacy of phosphorus-32 brachytherapy without external-beam radiation for long-term tumor control in patients with craniopharyngioma.

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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

Predicting Behavioral Problems in Craniopharyngioma Survivors after Conformal Radiation Therapy

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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

SU-E-J-122: Detecting Treatment-Induced Metabolic Abnormalities in Craniopharyngioma Patients Undergoing Surgery and Proton Therapy

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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

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

Gamma knife therapy for craniopharyngioma

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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.)

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

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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.

Childhood vitiligo: Treatment paradigms

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Amrinder Jit Kanwar

2012-01-01

Full Text Available Childhood vitiligo differs from the adults by showing a higher incidence in females, segmental vitiligo being more common and less frequent association with other systemic autoimmune and endocrine disorders.Childhood vitiligo is often associated with a marked psychosocial and long lasting effect on the self-esteem of the affected children and their parents, hence an adequate treatment is very essential. Treatment of vitiligo is indeed a tough challenge for the dermatologists′ more so in the background of childhood vitiligo. Although multiple therapeutic modalities are available in the therapeutic armamentarium, not all can be used in children. This brief report updates regarding various therapies available in the treatment of childhood vitiligo.

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

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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

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

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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

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

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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.

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

Craniopharyngioma in the temporal lobe: a case report

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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.

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

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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.

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

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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.

Sleeve gastrectomy leads to easy management of hormone replacement therapy and good weight loss in patients treated for craniopharyngioma.

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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.

Craniopharyngioma in the Temporal Lobe: A Case Report

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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

Internal irradiation for cystic craniopharyngioma

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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.)

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

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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.

Abscess inside craniopharyngioma: diagnostic and management implications.

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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.

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

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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

Polysomnographic findings in craniopharyngioma patients.

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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.

Do craniopharyngioma molecular signatures correlate with clinical characteristics?

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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.

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

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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

Role of gamma knife radiosurgery in craniopharyngioma

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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.

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

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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.

Ectopic recurrence of craniopharyngioma: Reporting three new cases.

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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.

Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

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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

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

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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.

Craniopharyngioma in adults

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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.

Nutritional assessment of a population with a history of childhood craniopharyngioma seen at Hospital "Prof. Dr. Juan P. Garrahan".

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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

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

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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

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.

A Rare Case of Craniopharyngioma in the Temporal Lobe

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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.

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.

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...

Huge cystic craniopharyngioma. Changes of cyst density on computed tomography

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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.

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

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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.

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.

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.)

Nuchal Skinfold Thickness: A Novel Parameter for Assessment of Body Composition in Childhood Craniopharyngioma

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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

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 .

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

[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).

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.

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.

On the Benefits and Risks of Proton Therapy in Pediatric Craniopharyngioma

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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

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.

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.

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.

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)

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.

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

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

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.

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

Effects of gamma knife radiosurgery for pediatric craniopharyngiomas

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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)

Diagnostic imaging of craniopharyngioma

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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)

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

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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.

A case study of the neuropsychological outcomes following microsurgery, conventional radiotherapy and stereotactic radiotherapy for an adult's recurrent craniopharyngioma.

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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.

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

Long-term visual outcomes of craniopharyngioma in children.

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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.

Haemorrhagic Presentation of a Craniopharyngioma in a Pregnant Woman

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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.

Dosimetry of {sup 32}P radiocolloid for treatment of cystic craniopharyngioma

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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.

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

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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.

Bypass surgery to treat symptomatic fusiform dilation of the internal carotid artery following craniopharyngioma resection: report of 2 cases.

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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.

Periostin activates pathways involved in epithelial-mesenchymal transition in adamantinomatous craniopharyngioma.

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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.

Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report

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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.

Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report.

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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.

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

Learning and Memory Following Conformal Radiation Therapy for Pediatric Craniopharyngioma and Low-Grade Glioma

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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.

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.

Expanded Endoscopic Endonasal Resection of Retrochiasmatic Craniopharyngioma.

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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 .

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

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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.

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)

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

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)

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

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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

Treatment of Childhood Obesity: A Systematic Review

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Staniford, Leanne J.; Breckon, Jeff D.; Copeland, Robert J.

2012-01-01

Childhood obesity trends have increased dramatically over the past three decade's. The purpose of this quantitative systematic review is to provide an update of the evidence, illustrating the efficacy of childhood obesity treatment, considering whether treatment fidelity has been measured and/or reported and whether this related to the treatment…

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

The supraorbital eyebrow approach for removal of craniopharyngioma in children: a case series.

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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

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

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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.

Effect of Preserving the Pituitary Stalk During Resection of Craniopharyngioma in Children on the Diabetes Insipidus and Relapse Rates and Long-Term Outcomes.

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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.

Childhood Thyroid Cancer Treatment (PDQ®)—Patient Version

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Childhood thyroid cancer treatment usually includes surgery and may include radioactive iodine therapy, targeted therapy, and hormone replacement therapy. Learn more about the diagnosis and treatment of childhood thyroid cancer in this expert-reviewed summary.

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

Genetically engineered mouse models of craniopharyngioma: an opportunity for therapy development and understanding of tumor biology.

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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.

Childhood Nasopharyngeal Cancer Treatment (PDQ®)—Patient Version

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Childhood nasopharyngeal cancer treatment options include chemotherapy, external and internal radiation therapy, surgery, and immunotherapy (interferon). Learn more about the risk factors, symptoms, tests to diagnose, and treatment of childhood nasopharyngeal cancer in this expert-reviewed summary.

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

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.

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

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.

Hypothalamic Obesity following Craniopharyngioma Surgery: Results of a Pilot Trial of Combined Diazoxide and Metformin Therapy

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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.

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

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.)

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

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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

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.

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)

Long-term effects of gamma knife radiosurgery for pediatric craniopharyngioma

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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)

Management of craniopharyngiomas: Role of conservative strategies

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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.

Contrast enhancement in the craniopharyngioma cyst wall caused by irradiation

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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.).

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.)

Childhood Ependymoma Treatment (PDQ®)—Patient Version

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Childhood ependymoma is often treated with surgery, radiation therapy, and/or chemotherapy. Get information about the types of ependymoma, symptoms, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood ependymomas in this expert-reviewed summary.

The difference in pediatric blood pressure between middle childhood and late childhood prior to dental treatment

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Fitri Anissa Syaimima bt. Syaiful Azim

2018-01-01

Full Text Available Every child will go through several stages in his or her life. They are different from each other as they are in the process of development of cognition, physics, emotion, and personality. For many children, a visit to the dentist can raise their anxiety. This anxiousness will lead to stress that influences the cardiovascular function in the body. The purpose of this research was to determine the difference in pediatric blood pressure between middle childhood and late childhood prior to dental treatment. This research was a clinical trial, pure experimental study. The sample consisted of 30 children within the range of 4-12 years old where they were divided into two groups of age; middle childhood (4-7 years old and late childhood (8-12 years old. The blood pressures were measured before any dental treatment began and the values were recorded. The data were then analyzed using the One-Sample T-Test analysis. The results of blood pressure in middle childhood and late childhood were compared to the average mean values for each age group. It showed that there was a significant difference in the systolic pressure, which was found higher in the middle childhood group compared to the late childhood. From the result can be concluded that there was a difference in the pediatric blood pressure between middle childhood and late childhood prior to dental treatment.

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 .

Malignant astrocytoma following radiotherapy for craniopharyngioma

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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.

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)

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...

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.

Childhood Laryngeal Tumors Treatment (PDQ®)—Patient Version

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Childhood laryngeal (throat) tumors are tumors of the larynx (voice box). They can be benign (papillomatosis) or cancer. Treatment depends on the type of cell the cancer grew from. Get information about the symptoms, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood laryngeal tumors in this expert-reviewed summary.

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.

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

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

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.

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.

Vasospasm After Craniopharyngioma Surgery: Can We Prevent It?

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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.

Sir Victor Horsley: pioneer craniopharyngioma surgeon.

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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

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)

Partial removal and post-operative irradiation for craniopharyngioma

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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).

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.

Childhood Liver Cancer Treatment (PDQ®)—Patient Version

Science.gov (United States)

Childhood liver cancer treatment options include surgery, watchful waiting, chemotherapy, radiation therapy, ablation therapy, and antiviral therapy. Learn more about newly diagnosed and recurrent childhood liver cancer in this expert-reviewed summary.

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.

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

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.

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

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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.

Long-term outcomes for adult craniopharyngioma following radiation therapy

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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.

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.

Treatment intensity and childhood apraxia of speech

NARCIS (Netherlands)

Namasivayam, Aravind K.; Pukonen, Margit; Goshulak, Debra; Hard, Jennifer; Rudzicz, Frank; Rietveld, Toni; Maassen, Ben; Kroll, Robert; van Lieshout, Pascal

BackgroundIntensive treatment has been repeatedly recommended for the treatment of speech deficits in childhood apraxia of speech (CAS). However, differences in treatment outcomes as a function of treatment intensity have not been systematically studied in this population. AimTo investigate the

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

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)

Treatment Intensity and Childhood Apraxia of Speech

Science.gov (United States)

Namasivayam, Aravind K.; Pukonen, Margit; Goshulak, Debra; Hard, Jennifer; Rudzicz, Frank; Rietveld, Toni; Maassen, Ben; Kroll, Robert; van Lieshout, Pascal

2015-01-01

Background: Intensive treatment has been repeatedly recommended for the treatment of speech deficits in childhood apraxia of speech (CAS). However, differences in treatment outcomes as a function of treatment intensity have not been systematically studied in this population. Aim: To investigate the effects of treatment intensity on outcome…

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.)

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

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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.)

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.

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.

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.

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.

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

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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.

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

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

Energy Technology Data Exchange (ETDEWEB)

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.

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

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.

Nonpharmacologic Treatments for Childhood Constipation : Systematic Review

NARCIS (Netherlands)

Tabbers, Merit M.; Boluyt, Nicole; Berger, Marjolein Y.; Benninga, Marc A.

2011-01-01

OBJECTIVE: To summarize the evidence and assess the reported quality of studies concerning nonpharmacologic treatments for childhood constipation, including fiber, fluid, physical movement, prebiotics, probiotics, behavioral therapy, multidisciplinary treatment, and forms of alternative medicine.

Nonpharmacologic treatments for childhood constipation: systematic review

NARCIS (Netherlands)

Tabbers, Merit M.; Boluyt, Nicole; Berger, Marjolein Y.; Benninga, Marc A.

2011-01-01

To summarize the evidence and assess the reported quality of studies concerning nonpharmacologic treatments for childhood constipation, including fiber, fluid, physical movement, prebiotics, probiotics, behavioral therapy, multidisciplinary treatment, and forms of alternative medicine. We

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.

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

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.

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

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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.

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.

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

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.

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

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.

Childhood Esthesioneuroblastoma Treatment (PDQ®)—Patient Version

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Esthesioneuroblastoma (olfactory neuroblastoma) forms in nasal tissues involved with smell (olfactory bulbs). Treatment may include surgery, radiation therapy, or chemotherapy. Get information about the symptoms, diagnosis, prognosis, and treatment of childhood esthesioneuroblastoma in this expert-reviewed summary.

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.

Childhood Astrocytoma Treatment (PDQ®)—Patient Version

Science.gov (United States)

Childhood astrocytomas can be benign (not cancer) or malignant (cancer). Learn more about the types of astrocytoma, signs and symptoms, diagnosis, prognosis, and treatment of astrocytomas that are newly diagnosed or have come back after treatment in this expert-reviewed summary.

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

Fertility Treatment and Childhood Epilepsy - a Nationwide Cohort Study

DEFF Research Database (Denmark)

Kettner, Laura Ozer; Kesmodel, Ulrik Schiøler; Ramlau-Hansen, Cecilia Høst

2017-01-01

BACKGROUND: Fertility treatment includes hormonal stimulation of the woman and in vitro manipulation of gametes and embryos that may influence prenatal brain development. We aimed to investigate the association between fertility treatment and childhood epilepsy, including specific types of treatm......BACKGROUND: Fertility treatment includes hormonal stimulation of the woman and in vitro manipulation of gametes and embryos that may influence prenatal brain development. We aimed to investigate the association between fertility treatment and childhood epilepsy, including specific types...... of treatment and indications, as well as subtypes of epilepsy. METHODS: In this nationwide birth cohort study, we included all pregnancies in Denmark resulting in live-born singletons, 1995-2003. Children conceived by fertility treatment and children developing epilepsy (until 2013) were identified from Danish...... national registers. RESULTS: A total of 565,116 pregnancies were included; 8,071 children (1.4%) developed epilepsy. Children conceived after ovulation induction or intrauterine insemination had a slightly higher risk of childhood epilepsy (hazard ratio [HR]: 1.15; 95% confidence interval [CI]: 1.00, 1...

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

Fertility treatment and childhood type 1 diabetes mellitus

DEFF Research Database (Denmark)

Kettner, Laura Ozer; Matthiesen, Niels Bjerregaard; Ramlau-Hansen, Cecilia Høst

2016-01-01

OBJECTIVE: To investigate the association between specific types of fertility treatment and childhood type 1 diabetes mellitus. DESIGN: Nationwide birth cohort study. SETTING: Not applicable. PATIENT(S): All pregnancies resulting in a live-born singleton child in Denmark from 1995 to 2003....... INTERVENTION(S): Not applicable. MAIN OUTCOME MEASURE(S): Childhood type 1 diabetes mellitus identified from redeemed prescriptions for insulin until 2013. RESULT(S): The study included 565,116 singleton pregnancies. A total of 14,985 children were conceived by ovulation induction or intrauterine insemination......, and 8,490 children were conceived by in vitro fertilization or intracytoplasmic sperm injection. During the follow-up period, 2,011 (0.4%) children developed type 1 diabetes mellitus. The primary analyses showed no association between fertility treatment and childhood type 1 diabetes mellitus...

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 .

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.

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.)

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.

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.

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

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

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

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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.

Mixed pituitary adenoma/craniopharyngioma: clinical, morphological, immunohistochemical and ultrastructural study of a case, review of the literature, and pathogenetic and nosological considerations.

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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.

Childhood Brain Stem Glioma Treatment (PDQ®)—Patient Version

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Childhood brain stem glioma can be a benign (not cancer) or malignant (cancer) condition where abnormal cells form in the tissues of the brain stem. Get information about the symptoms, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood brain stem glioma in this expert-reviewed summary.

Childhood obesity treatment and prevention. Psychological perspectives of clinical approaches

OpenAIRE

Maria Catena Quattropani; Teresa Buccheri

2013-01-01

Objective: This work focuses on clinical psychologist’ presence within childhood obesity prevention programmes in several countries. Method: The Authors collected articles considering psychological, biological and social aspects linked to childhood obesity. Results: Studies reveal that childhood obesity prevention programmes are based on biological, medical and educational aspects; clinical psychologists up until now have been engaged almost exclusively in the treatment of obesity. Conclusion...

Childhood Midline Tract Carcinoma Treatment (PDQ®)—Patient Version

Science.gov (United States)

Childhood midline tract carcinoma occurs in the respiratory tract or other places along the center line of the body. It is sometimes caused by a change in the NUT gene (NUT midline carcinoma). Get information about childhood midline tract carcinoma, including symptoms, tests, and multimodality treatment in this expert-reviewed summary.

Effects of Surgery and Proton Therapy on Cerebral White Matter of Craniopharyngioma Patients

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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

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

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

Childhood Liver Cancer Treatment (PDQ®)—Health Professional Version

Science.gov (United States)

Childhood liver cancer has two major histologic subgroups: hepatoblastoma and hepatocellular carcinoma. Less common histologies are undifferentiated embryonal sarcoma of the liver, infantile choriocarcinoma, and vascular liver tumors. Get detailed information about newly diagnosed and recurrent childhood liver cancers including tumor biology, presentation, prognosis, staging, and treatment in this summary for clinicians.

Childhood Tuberculosis in Nigeria: Disease Presentation and Treatment Outcomes

Directory of Open Access Journals (Sweden)

Chidubem L Ogbudebe

2018-02-01

Full Text Available Objectives: Understanding the factors that influence tuberculosis (TB treatment outcomes in children is key to designing interventions to address them. This study aimed to determine the case category distribution of childhood TB in Nigeria and assess which clinical and demographic factors are associated with different treatment outcomes in childhood TB. Materials and methods: This was a retrospective cohort study involving a review of medical records of children (0-14 years with TB in 3 states in Nigeria in 2015. Results: Of 724 childhood TB cases registered during the review period, 220 (30.4% were aged 0–4 years. A high proportion of patients had pulmonary TB 420/724 (58.0%, new TB infection 713/724 (98.5%, and human immunodeficiency virus (HIV coinfection 108/724 (14.7%. About 28% (n = 201 were bacteriologically diagnosed. The proportion of TB treatment success was 601/724 (83.0%. Treatment success was significantly higher in children aged 5–14 years than those 0–4 years (85.3% vs 77.7%, P  = .01. Factors associated with unsuccessful outcomes in patients aged 0–4 years are male sex (adjusted odds ratio [aOR]: 1.2, HIV-positive status (aOR: 1.2, and clinical method of diagnosis (aOR: 5.6. Conclusions: Efforts should be made to improve TB treatment outcomes in children by ensuring early and accurate diagnosis, focused training of health workers on childhood TB-HIV care, and effective adherence counseling of caregivers.

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.

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.

Childhood maltreatment and motivation for treatment in girls in compulsory residential care

NARCIS (Netherlands)

Leenarts, L.E.W.; Hoeve, M.; van de Ven, P.M.; Lodewijks, H.P.B.; Dorelijers, T.A.H.

2013-01-01

The first objective of the current study was to examine the relationship between childhood maltreatment, trauma-related symptoms and motivation for treatment in girls in compulsory residential treatment facilities. The second objective was to examine the extent to which various forms of childhood

Treatment-related mortality in relapsed childhood acute lymphoblastic leukemia

DEFF Research Database (Denmark)

Oskarsson, Trausti; Söderhäll, Stefan; Arvidson, Johan

2018-01-01

BACKGROUND: Treatment of relapsed childhood acute lymphoblastic leukemia (ALL) is particularly challenging due to the high treatment intensity needed to induce and sustain a second remission. To improve results, it is important to understand how treatment-related toxicity impacts survival...

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

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.

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.

Craniopharyngioma identification by CT and MR imaging at 1.5 T

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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.).

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

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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.

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

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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

Treatment of childhood sexual abuse: an updated review.

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Cummings, Marissa; Berkowitz, Steven J; Scribano, Philip V

2012-12-01

Childhood sexual abuse (CSA) involves multiple complex factors that make the evaluation of therapeutic interventions especially complicated. PTSD prevalence rates of CSA are approximately 37 % -53 %. Several other psychiatric sequelae of CSA exist. CSA appears to disrupt brain and body physiology. One co-located service delivery model reported a 52 % linkage rate of CSA survivors with mental health treatment. This article reviews current literature on the treatment of CSA, including psychosocial interventions, pharmacotherapy, and early preventative interventions. It also provides an update on the short- and long-term sequelae of CSA and implications for future research directions. A literature search of papers published in the last 3 years was conducted using the keywords treatment, sexual abuse, childhood, epigenetics, resilience and review, and searching the following databases: PsycInfo, PubMed, Substance Abuse and Mental Health Services Administration, and Centers for Disease Control.

Combined endoscopic approach in the management of suprasellar craniopharyngioma.

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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

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.

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

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

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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.)

[Influence of interleukin-1 beta gene polymorphism and childhood maltreatment on antidepressant treatment].

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Chen, Ying; Zhang, Zhijun; Xu, Zhi; Pu, Mengjia; Geng, Leiyu

2015-12-01

To explore the influence of interleukin-1 beta (IL1B) gene polymorphism and childhood maltreatment on antidepressant treatment. Two hundred and four patients with major depressive disorder (MDD) have received treatment with single antidepressant drugs and were followed up for 8 weeks. Hamilton depression scale-17 (HAMD-17) was used to evaluate the severity of depressive symptoms and therapeutic effect. Childhood maltreatment was assessed using Childhood Trauma Questionnaire, a 28-item Short Form (CTQ-SF). Single nucleotide polymorphism (SNP) of the IL1B gene was determined using a SNaPshot method. Correlation of rs16944 gene polymorphism with response to treatment was analyzed using Unphased 3.0.13 software. The main and interactive effects of SNP and childhood maltreatment on the antidepressant treatment were analyzed using Logistic regression analysis. No significant difference of gender, age, year of education, family history, episode time, and antidepressant agents was detected between the remitters and non-remitters. Association analysis has found that the SNP rs16944 in the IL1B AA genotype carriers antidepressant response was poorer (χ2=3.931, P=0.047). No significant difference was detected in the CTQ scores between the two groups. Genetic and environmental interaction analysis has demonstrated a significant correlation between rs16944 AA genotype and childhood maltreatment and poorer response to antidepressant treatment. The SNP rs16944 in the IL1B gene and its interaction with childhood maltreatment may influence the effect of antidepressant treatment for patients with MDD.

Results of radiotherapy in craniopharyngiomas analysed by the linear quadratic model

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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.).

Childhood Hodgkin Lymphoma Treatment (PDQ®)—Health Professional Version

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In childhood Hodgkin lymphoma, current treatment uses risk-adapted, response-based paradigms to determine the length and intensity of treatment. Get detailed information about newly diagnosed and recurrent classical and nodular lymphocyte predominant Hodgkin lymphoma, including presentation, diagnosis and staging, prognosis, and treatment in this summary for clinicians.

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

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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.

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

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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.

Treatment of Childhood Depression: The State of the Art.

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Clarizio, Harvey F.

1986-01-01

This article reviews various aspects of seven approaches to the treatment of childhood depression--psychoanalytic, behavioral, cognitive, familial, rational-emotive, multimodal, and drug interventions. Implications for practitioners are considered in terms of target selection, choice of treatment methods, rational evaluation based on developmental…

A review of Craniopharyngioma and the retrospective study on 100 patients

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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.

Pharmacogenetics Influence Treatment Efficacy in Childhood Acute Lymphoblastic Leukemia

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Devidsen, M.L.; Dalhoff, K.; Schmiegelow, K.

2008-01-01

in treatment resistance and toxic side effects. As most childhood acute lymphoblastic leukemia treatment protocols include up to 13 different chemotherapeutic agents, the impact of individual SNPs has been difficult to evaluate. So far Focus has mainly been on the widely used glucocorticosteroids, methotrexate...

Anaesthetic management of children with craniopharyngioma

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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.

Long term results of childhood dysphonia treatment.

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Mackiewicz-Nartowicz, Hanna; Sinkiewicz, Anna; Bielecka, Arleta; Owczarzak, Hanna; Mackiewicz-Milewska, Magdalena; Winiarski, Piotr

2014-05-01

The aim of this study was to assess the long term results of treatment and rehabilitation of childhood dysphonia. This study included a group of adolescents (n=29) aged from 15 to 20 who were treated due to pediatric hyperfunctional dysphonia and soft vocal fold nodules during their pre-mutational period (i.e. between 5 and 12 years of age). The pre-mutational therapy was comprised of proper breathing pattern training, voice exercises and psychological counseling. Laryngostroboscopic examination and perceptual analysis of voice were performed in each patient before treatment and one to four years after mutation was complete. The laryngostroboscopic findings, i.e. symmetry, amplitude, mucosal wave and vocal fold closure, were graded with NAPZ scale, and the GRBAS scale was used for the perceptual voice analysis. Complete regression of the childhood dysphonia was observed in all male patients (n=14). Voice disorders regressed completely also in 8 out of 15 girls, but symptoms of dysphonia documented on perceptual scale persisted in the remaining seven patients. Complex voice therapy implemented in adolescence should be considered as either the treatment or preventive measure of persistent voice strain, especially in girls. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Childhood Tuberculosis: Epidemiology, Diagnosis, Treatment, and Vaccination

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Kuo-Sheng Tsai

2013-10-01

Full Text Available Despite the existence of a government-run tuberculosis (TB control program, the current nationwide burden of TB continues to be a public health problem in Taiwan. Intense current and previous efforts into diagnostic, therapeutic, and preventive interventions have focused on TB in adults, but childhood TB has been relatively neglected. Children are particularly vulnerable to severe disease and death following infection, and children with latent infections become reservoirs for future transmission following disease reactivation in adulthood, thus fueling future epidemics. Additional research, understanding, and prevention of childhood TB are urgently needed. This review assesses the epidemiology, diagnosis, treatment, and relevant principles of TB vaccine development and presents efficacy data for the currently licensed vaccines.

Neurocognitive Effects of Treatment for Childhood Cancer

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Butler, Robert W.; Haser, Jennifer K.

2006-01-01

We review research on the neuropsychological effects that central nervous system (CNS) cancer treatments have on the cognitive abilities of children and adolescents. The authors focus on the two most common malignancies of childhood: leukemias and brain tumors. The literature review is structured so as to separate out earlier studies, generally…

Combined Interhemispheric and Transsylvian Approach for Resection of Craniopharyngioma.

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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 .

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

A craniopharyngioma in a seven-year-old dog

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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.

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

Feedback Frequency in Treatment for Childhood Apraxia of Speech

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Maas, Edwin; Butalla, Christine E.; Farinella, Kimberly A.

2012-01-01

Purpose: To examine the role of feedback frequency in treatment for childhood apraxia of speech (CAS). Reducing the frequency of feedback enhances motor learning, and recently, such feedback frequency reductions have been recommended for the treatment of CAS. However, no published studies have explicitly compared different feedback frequencies in…

Transcallosal, Transchoroidal Resection of a Recurrent Craniopharyngioma.

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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 .

Extended Endoscopic Endonasal Approach for Suprasellar Craniopharyngioma.

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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 .

Endoscopic Endonasal Approach to a Suprasellar Craniopharyngioma.

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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 .

Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)—Health Professional Version

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Childhood non-Hodgkin lymphoma (NHL) has three main types (aggressive mature B-cell [Burkitt, diffuse large B-cell, primary mediastinal B-cell], lymphoblastic and anaplastic large cell lymphoma) and other less common types of NHL. Get detailed information about the presentation, diagnosis, staging, prognosis, and treatment of all types of newly diagnosed and recurrent childhood NHL and lymphoproliferative disease in this summary for clinicians.

Frontal dermoid cyst coexisting with suprasellar craniopharyngioma: a spectrum of ectodermally derived epithelial-lined cystic lesions?

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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.

Gamma knife treatment of intracranial disorders in children

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Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki (Komaki City Hospital, Aichi (Japan))

1993-06-01

Radiosurgical treatment with a gamma knife was performed on 160 patients with intracranial lesions at Komaki City Hospital from May 1991 through February 1992. Twenty seven of the patients were under 15 years of age: 19 had arteriovenous malformations (AVM), two had craniopharyngiomas and one each had optic glioma, ganglioglioma, pineal teratoma, ependymoma, medulloblastoma and glioblastoma. It is still too early to evaluate the results of treatment, but some early effects have been found in some cases. There was no immediate side effect of this treatment. A decrease of the nidus was found by MRI angiography (MRA) 7 months after treatment in one patient with AVM. In one of the craniopharyngioma patients the low-intensity area (LIA) of the tumor was larger on T[sub 1]-weighted MRI but smaller 6 months after treatment. An intratumoral LIA was also found in the optic glioma patient 6 months after treatment. There are difficulties in the gamma knife treatment of children, since general anesthesia is needed below 12 years of age, and it is difficult to apply the system below 2 years of age. However, there are advantages: higher radiosensitivity in pediatric AVM and the curability of inoperable lesions without neurological deficit. Radiosurgery for pediatric brain tumors has different indications from those for adults. Patients with small circumscribed benign tumors, such as craniopharyngiomas, optic gliomas, gangliogliomas or pineal tumors are good candidates for radiosurgery. (author).

Childhood Acute Myeloid Leukemia Treatment (PDQ®)—Patient Version

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Childhood acute myeloid leukemia and other myeloid malignancies treatment may include chemotherapy, radiation therapy, stem cell transplant, and targeted therapy. Learn more about AML and myelodysplastic/myeloproliferative diseases in this expert-reviewed summary.

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.)

[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

Adolescent substance use in the multimodal treatment study of attention-deficit/hyperactivity disorder (ADHD) (MTA) as a function of childhood ADHD, random assignment to childhood treatments, and subsequent medication.

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Molina, Brooke S G; Hinshaw, Stephen P; Eugene Arnold, L; Swanson, James M; Pelham, William E; Hechtman, Lily; Hoza, Betsy; Epstein, Jeffery N; Wigal, Timothy; Abikoff, Howard B; Greenhill, Laurence L; Jensen, Peter S; Wells, Karen C; Vitiello, Benedetto; Gibbons, Robert D; Howard, Andrea; Houck, Patricia R; Hur, Kwan; Lu, Bo; Marcus, Sue

2013-03-01

To determine long-term effects on substance use and substance use disorder (SUD), up to 8 years after childhood enrollment, of the randomly assigned 14-month treatments in the multisite Multimodal Treatment Study of Children with Attention-Deficit/Hyperactivity Disorder (MTA; n = 436); to test whether medication at follow-up, cumulative psychostimulant treatment over time, or both relate to substance use/SUD; and to compare substance use/SUD in the ADHD sample to the non-ADHD childhood classmate comparison group (n = 261). Mixed-effects regression models with planned contrasts were used for all tests except the important cumulative stimulant treatment question, for which propensity score matching analysis was used. The originally randomized treatment groups did not differ significantly on substance use/SUD by the 8-year follow-up or earlier (mean age = 17 years). Neither medication at follow-up (mostly stimulants) nor cumulative stimulant treatment was associated with adolescent substance use/SUD. Substance use at all time points, including use of two or more substances and SUD, were each greater in the ADHD than in the non-ADHD samples, regardless of sex. Medication for ADHD did not protect from, or contribute to, visible risk of substance use or SUD by adolescence, whether analyzed as randomized treatment assignment in childhood, as medication at follow-up, or as cumulative stimulant treatment over an 8-year follow-up from childhood. These results suggest the need to identify alternative or adjunctive adolescent-focused approaches to substance abuse prevention and treatment for boys and girls with ADHD, especially given their increased risk for use and abuse of multiple substances that is not improved with stimulant medication. Clinical trial registration information-Multimodal Treatment Study of Children With Attention Deficit and Hyperactivity Disorder (MTA); http://clinical trials.gov/; NCT00000388. Copyright © 2013 American Academy of Child and Adolescent

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....

Early loss of teeth after treatment for childhood leukemia

International Nuclear Information System (INIS)

Herrmann, T.; Doerr, W.; Lesche, A.; Lehmann, D.; Koy, S.

2004-01-01

Background: only few reports of effects of radiotherapy in childhood on the dental apparatus are available in the literature. The basis for early loss of teeth appears to be a reduction of the root surface area after radiation exposure. These effects in the periodontium are a consequence of combined radiochemotherapy usually applied for treatment of childhood neoplasia. Chemotherapy alone also results in changes of periodontal development. Case report: a 33-year-old patient is reported, who, at the age of 11 years, received high-dose chemotherapy and radiotherapy of neuroaxis and cranium for acute lymphatic leukemia with relapse. The patient consulted the Implant Section of the Department of Oral and Maxillofacial Surgery because of severe dental changes and tooth loss despite adequate dental care and oral hygiene. Radiation doses given to the superior maxilla and mandible at the age of 11 were estimated to be in the range of 8-25 Gy. Conclusion: intense, life-long dental care and follow-up of patients cured from malignant disease in childhood must hence be postulated in order to minimize dental treatment sequelae by supportive measures, but also to initiate timely adequate dental and prosthetic management. (orig.)

Fluoxetine treatment is effective in a rat model of childhood-induced post-traumatic stress disorder

OpenAIRE

Ariel, Lior; Inbar, Sapir; Edut, Schachaf; Richter-Levin, Gal

2017-01-01

Although selective serotonin reuptake inhibitors (SSRIs) are first-line treatment for post-traumatic stress disorder (PTSD) patients, their therapeutic efficacy is limited. Childhood adversities are considered a risk factor for developing PTSD in adulthood but may trigger PTSD without additional trauma in some individuals. Nevertheless, just as childhood is considered a vulnerable period it may also be an effective period for preventive treatment. Using a rat model of childhood-induced PTSD, ...

Proton Beam Therapy Versus Conformal Photon Radiation Therapy for Childhood Craniopharyngioma: Multi-institutional Analysis of Outcomes, Cyst Dynamics, and Toxicity

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Bishop, Andrew J. [Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas (United States); Greenfield, Brad [Department of Radiation Oncology, Baylor College of Medicine, Houston, Texas (United States); Mahajan, Anita [Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas (United States); Paulino, Arnold C. [Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas (United States); Department of Radiation Oncology, Baylor College of Medicine, Houston, Texas (United States); Okcu, M. Fatih [Department of Pediatrics, Texas Children' s Cancer and Hematology Center, Baylor College of Medicine, Houston, Texas (United States); Allen, Pamela K. [Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas (United States); Chintagumpala, Murali [Department of Pediatrics, Texas Children' s Cancer and Hematology Center, Baylor College of Medicine, Houston, Texas (United States); Kahalley, Lisa S. [Section of Psychology, Texas Children' s Cancer and Hematology Center, Baylor College of Medicine, Houston, Texas (United States); McAleer, Mary F.; McGovern, Susan L. [Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas (United States); Whitehead, William E. [Department of Neurosurgery, Texas Children' s Cancer and Hematology Center, Baylor College of Medicine, Houston, Texas (United States); Grosshans, David R., E-mail: dgrossha@mdanderson.org [Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas (United States)

2014-10-01

Purpose: We compared proton beam therapy (PBT) with intensity modulated radiation therapy (IMRT) for pediatric craniopharyngioma in terms of disease control, cyst dynamics, and toxicity. Methods and Materials: We reviewed records from 52 children treated with PBT (n=21) or IMRT (n=31) at 2 institutions from 1996-2012. Endpoints were overall survival (OS), disease control, cyst dynamics, and toxicity. Results: At 59.6 months' median follow-up (PBT 33 mo vs IMRT 106 mo; P<.001), the 3-year outcomes were 96% for OS, 95% for nodular failure-free survival and 76% for cystic failure-free survival. Neither OS nor disease control differed between treatment groups (OS P=.742; nodular failure-free survival P=.546; cystic failure-free survival P=.994). During therapy, 40% of patients had cyst growth (20% requiring intervention); immediately after therapy, 17 patients (33%) had cyst growth (transient in 14), more commonly in the IMRT group (42% vs 19% PBT; P=.082); and 27% experienced late cyst growth (32% IMRT, 19% PBT; P=.353), with intervention required in 40%. Toxicity did not differ between groups. On multivariate analysis, cyst growth was related to visual and hypothalamic toxicity (P=.009 and .04, respectively). Patients given radiation as salvage therapy (for recurrence) rather than adjuvant therapy had higher rates of visual and endocrine (P=.017 and .024, respectively) dysfunction. Conclusions: Survival and disease-control outcomes were equivalent for PBT and IMRT. Cyst growth is common, unpredictable, and should be followed during and after therapy, because it contributes to late toxicity. Delaying radiation therapy until recurrence may result in worse visual and endocrine function.

Late somatic sequelae after treatment of childhood cancer in Slovenia

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Erman Nuša

2012-05-01

Full Text Available Abstract Background This is a long-term follow-up clinical study of adolescents and adults, survivors of childhood cancer. We evaluate and analyze the late somatic sequelae of childhood cancer treatment. Many such studies are susceptible to a strong selection bias, i.e., they employ a limited non-systematic sample of patients, based on a clinical hospital that provided the cancer treatment or performed the follow-up. To address the issue of selection bias, we perform here an analysis of late sequelae on a systematic database of the entire population of the children treated for cancer in Slovenia. Due to the specifics of cancer treatment procedures in Slovenia, they have all been treated and followed-up in the same clinic. Methods The data are based on the centralized registry of cancer patients in Slovenia and present a controlled and homogeneous collection. Late sequelae are evaluated following a modified CTCAE, i.e., the National Cancer Institute’s Common Terminology Criteria for Adverse Events version 3.0. We use survival analysis method to estimate the incidence of and risk for late sequelae, where the time variable is measured in years from the diagnosis date, while we follow the event of incidence of late sequelae scored other than none. Survival analysis is performed using KaplanMeier estimator and Cox regression model. Results The incidence of mild, moderate, or severe late sequelae of childhood cancer treatment significantly decreased from 75% in the group of patients diagnosed before 1975 to 55% for those diagnosed after 1995. The Cox regression analysis of the risk factors for the incidence of late sequelae identifies three significant factors: treatment modalities, age at diagnosis, and primary diagnosis. Conclusions The change of treatment modalities in terms of replacement of surgery and radiotherapy with chemotherapy is the main reason for the decrease of the incidence and the risk for late sequelae of childhood cancer treatment

Childhood Ependymoma Treatment (PDQ®)—Health Professional Version

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In childhood ependymoma, cancer forms in the tissues of the brain and spinal cord. The cause of ependymoma is unknown. Find out about the molecular and clinical features, diagnosis, prognosis, treatment, and clinical trials for pediatric ependymoma, subependymoma, myxopapillary ependymoma, anaplastic ependymoma, and RELA fusion–positive ependymoma.

[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.

Comparing survival outcomes of gross total resection and subtotal resection with radiotherapy for craniopharyngioma: a meta-analysis.

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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.

Interleukin‑6 induces an epithelial‑mesenchymal transition phenotype in human adamantinomatous craniopharyngioma cells and promotes tumor cell migration.

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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.

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

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%)

Parental Infertility, Fertility Treatment, and Childhood Epilepsy

DEFF Research Database (Denmark)

Kettner, Laura O; Ramlau-Hansen, Cecilia Høst; Kesmodel, Ulrik S

2016-01-01

. RESULTS: A total of 60 440 pregnancies were included, and 0.8% of the children developed epilepsy.The primary analyses showed no association between parental infertility or fertility treatment, and the overall risk of childhood epilepsy (hazard rate ratios (HRs); 95% confidence intervals (CIs): 1.08 (0......BACKGROUND: A few studies have indicated an increased risk of epilepsy in children conceived by fertility treatment possibly due to characteristics of the infertile couple rather than the treatment. We therefore aimed to investigate the association between parental infertility, fertility treatment......, and epilepsy in the offspring, including the subtypes of epilepsy; idiopathic generalised epilepsy and focal epilepsy. METHODS: This cohort included all pregnancies resulting in liveborn singletons from the Aarhus Birth Cohort, Denmark (1995-2013). Information on time to pregnancy and fertility treatment...

Family involvement in the treatment of childhood obesity

DEFF Research Database (Denmark)

Grønbæk, Helle Nergaard; Madsen, Svend Aage Lykke; Michaelsen, Kim F.

2009-01-01

INTRODUCTION: The objective of this study was to assess the impacts of a family-based childhood obesity treatment on anthropometry and predictors of dropout and successful weight loss. MATERIALS AND METHODS: The 18-month treatment consisted of a intensive period (IP) including physical exercise...... had limited education and in immigrant families. CONCLUSIONS: This treatment with a psychological approach is feasible and results in significant weight loss during the programme. Future research should focus on how to improve the results of families with limited education and immigrants with non...

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

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

Childhood Nasopharyngeal Cancer Treatment (PDQ®)—Health Professional Version

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Nasopharyngeal carcinoma is strongly associated with Epstein-Barr virus (EBV) infection in children. Get comprehensive information about the risk factors, clinical presentation, diagnostic and staging evaluation, prognosis, and treatment of childhood nasopharyngeal carcinoma in this summary for clinicians.

Childhood Atypical Teratoid/Rhabdoid Tumor Treatment (PDQ®)—Patient Version

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Atypical teratoid/rhabdoid tumor (AT/RT) is a fast-growing tumor of the brain or spinal cord. Treatment may include surgery, radiation therapy, and chemotherapy. Get information about the symptoms, diagnosis, prognosis, and treatment of newly diagnosed and recurrent childhood atypical teratoid/rhabdoid tumors in this expert-reviewed summary.

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.

Multiplexed Immunofluorescence Reveals Potential PD-1/PD-L1 Pathway Vulnerabilities in Craniopharyngioma.

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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.

Endoscopic Endonasal Transplanum Transtuberculum Approach for the Resection of a Large Suprasellar Craniopharyngioma.

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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 .

Craniopharyngiomas: identification of different semiological patterns with MRI

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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.)

Childhood Laryngeal Tumors Treatment (PDQ®)—Health Professional Version

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Laryngeal tumors in children are rare and can be benign (papillomatosis) or malignant. Rhabdomyosarcoma is the most common cancer of the larynx in children. Get comprehensive information about childhood laryngeal tumors, including histology, presentation, and treatment in this summary for clinicians.

Childhood Vascular Tumors Treatment (PDQ®)—Patient Version

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Childhood vascular tumors form from cells that make blood vessels or lymph vessels. They can be benign (not cancer) or malignant (cancer). Get information about the symptoms, tests to diagnose, prognosis, and treatment of the most common type of vascular tumor, infantile hemangioma, and other vascular tumors in this expert-reviewed summary.

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.

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

Predicting parental distress among children newly diagnosed with craniopharyngioma.

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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.

Childhood obesity treatment; Effects on BMI SDS, body composition, and fasting plasma lipid concentrations

DEFF Research Database (Denmark)

Nielsen, Tenna Ruest Haarmark; Fonvig, Cilius Esmann; Dahl, Maria

2018-01-01

Objective The body mass index (BMI) standard deviation score (SDS) may not adequately reflect changes in fat mass during childhood obesity treatment. This study aimed to investigate associations between BMI SDS, body composition, and fasting plasma lipid concentrations at baseline and during......, and 80% improved their lipid concentrations. Conclusion Reductions in the degree of obesity during multidisciplinary childhood obesity treatment are accompanied by improvements in body composition and fasting plasma lipid concentrations. Even in individuals increasing their BMI SDS, body composition...... childhood obesity treatment. Methods 876 children and adolescents (498 girls) with overweight/obesity, median age 11.2 years (range 1.6±21.7), and median BMI SDS 2.8 (range 1.3±5.7) were enrolled in a multidisciplinary outpatient treatment program and followed for a median of 1.8 years (range 0...

Improved Binocular Outcomes Following Binocular Treatment for Childhood Amblyopia.

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Kelly, Krista R; Jost, Reed M; Wang, Yi-Zhong; Dao, Lori; Beauchamp, Cynthia L; Leffler, Joel N; Birch, Eileen E

2018-03-01

Childhood amblyopia can be treated with binocular games or movies that rebalance contrast between the eyes, which is thought to reduce depth of interocular suppression so the child can experience binocular vision. While visual acuity gains have been reported following binocular treatment, studies rarely report gains in binocular outcomes (i.e., stereoacuity, suppression) in amblyopic children. Here, we evaluated binocular outcomes in children who had received binocular treatment for childhood amblyopia. Data for amblyopic children enrolled in two ongoing studies were pooled. The sample included 41 amblyopic children (6 strabismic, 21 anisometropic, 14 combined; age 4-10 years; ≤4 prism diopters [PD]) who received binocular treatment (20 game, 21 movies; prescribed 9-10 hours treatment). Amblyopic eye visual acuity and binocular outcomes (Randot Preschool Stereoacuity, extent of suppression, and depth of suppression) were assessed at baseline and at 2 weeks. Mean amblyopic eye visual acuity (P suppression (P = 0.003) were reduced from baseline at the 2-week visit (87% game adherence, 100% movie adherence). Depth of suppression was reduced more in children aged suppression was correlated with a larger depth of suppression reduction at 2 weeks (P = 0.001). After 2 weeks, binocular treatment in amblyopic children improved visual acuity and binocular outcomes, reducing the extent and depth of suppression and improving stereoacuity. Binocular treatments that rebalance contrast to overcome suppression are a promising additional option for treating amblyopia.

High precision conformal radiotherapy employing conservative margins in childhood benign and low-grade brain tumours

International Nuclear Information System (INIS)

Jalali, Rakesh; Budrukkar, Ashwini; Sarin, Rajiv; Sharma, Dayananda S.

2005-01-01

Background and purpose: To report local control and follow up outcome data of high precision conformal radiotherapy in childhood brain tumours. Materials and methods: Between December 1999 and December 2002, 26 children (17 boys and 9 girls, median age 11.5 years) with incompletely excised or recurrent benign and low-grade brain tumours [13 craniopharyngiomas, 11 low-grade gliomas (LGG) and 2 others] were treated with three-dimensional (3D) conformal radiotherapy (CRT) (12 patients) and stereotactic conformal radiotherapy (SCRT) (14 patients). Gross tumour volume (GTV) included neuro-imaging based visible tumour and/or resected tumour bed. Clinical target volume (CTV) consisted of GTV + 5 mm margin and planning target volume (PTV) consisted of additional 5 mm margin for CRT and 2 mm for SCRT. Treatment was delivered with 3-9 conformal fixed fields to a median dose of 54 Gy/30 fractions. Results: The actuarial 2 and 3 year disease free and overall survival was 96 and 100%, respectively (median follow up: 25 months, range 12-47 months). Radiological follow up available in 25 patients revealed complete response in 1, partial regression in 10, stable disease in 13 and progression in 1 patient (within the CTV). One patient with craniopharyngioma on a routine imaging revealed a mild asymptomatic cyst enlargement, which resolved with conservative management. A patient with chiasmatic glioma developed cystic degeneration and hydrocephalus 9 months after SCRT requiring cyst drainage and placement of a ventriculoperitoneal shunt. Conclusion: High-precision conformal techniques delivering irradiation to a computer generated target volume employing 7-10 mm 3D margins beyond the visible tumour and/or resected tumour bed appear to be safe in children with incompletely resected or recurrent benign and low-grade brain tumours, based on these data

Treatment Of Brain Tumours In Childhood

International Nuclear Information System (INIS)

Stancokova, T.

2007-01-01

Children tumours are the second most common oncologic diseases in childhood (20 %) with highest incidence of mortality in children oncology. Brain tumours form a heterogenous group of tumours with their classification,diagnostic criteria and therapeutic modalities. General principles of treatment involve neurosurgery, which is a prognostic factor, its radicality depends on localization. Radiotherapy has limitations in children until 3 years for possible late effects. Chemotherapy is effective in tumours with high growing rate. These days challenge is to improve therapeutic outcomes and minimalize toxicity of therapy. (author)

Gene expression profiles reveal key genes for early diagnosis and treatment of adamantinomatous craniopharyngioma.

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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

Fluoxetine treatment is effective in a rat model of childhood-induced post-traumatic stress disorder.

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Ariel, Lior; Inbar, Sapir; Edut, Schachaf; Richter-Levin, Gal

2017-11-30

Although selective serotonin reuptake inhibitors (SSRIs) are first-line treatment for post-traumatic stress disorder (PTSD) patients, their therapeutic efficacy is limited. Childhood adversities are considered a risk factor for developing PTSD in adulthood but may trigger PTSD without additional trauma in some individuals. Nevertheless, just as childhood is considered a vulnerable period it may also be an effective period for preventive treatment. Using a rat model of childhood-induced PTSD, pre-pubertal stress (juvenile stress, JVS), we compared the therapeutic effects of fluoxetine and examined the effectiveness of 1 month of fluoxetine treatment following JVS and into adulthood compared to treatment in adulthood. Since not all individuals develop PTSD following a trauma, comparing only group means is not the adequate type of analysis. We employed a behavioral profiling approach, which analyzes individual differences compared to the normal behavior of a control group. Animals exposed to JVS exhibited a higher proportion of affected animals as measured using the elevated plus maze 8 weeks after JVS. Fluoxetine treatment following the JVS significantly decreased the proportion of affected animals as measured in adulthood. Fluoxetine treatment in adulthood was not effective. The results support the notion that childhood is not only a vulnerable period but also an effective period for preventive treatment.

Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome

NARCIS (Netherlands)

Teeninga, N.; Kist-van Holthe, J.E.; Rijswijk, N. van; Mos, N.I. de; Hop, W.C.J.; Wetzels, J.F.M.; Heijden, A.J. van der; Nauta, J.

2013-01-01

Prolonged prednisolone treatment for the initial episode of childhood nephrotic syndrome may reduce relapse rate, but whether this results from the increased duration of treatment or a higher cumulative dose remains unclear. We conducted a randomized, double-blind, placebo-controlled trial in 69

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 .

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...

Family information needs at childhood cancer treatment completion.

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Wakefield, Claire E; Butow, Phyllis; Fleming, Catharine A K; Daniel, Gunar; Cohn, Richard J

2012-04-01

Despite the recognized importance of information provision across the cancer trajectory, little research has investigated family information needs recently after childhood cancer. This mixed-methods, multiperspective, study explored the information needs of families of childhood cancer survivors in the first year post-treatment. In total, 112 semi-structured telephone interviews were conducted with 19 survivors (mean age 16.2 years, off treatment for ≤36 months), 44 mothers, 34 fathers, and 15 siblings. Interviews were analyzed inductively, line-by-line, using the framework of Miles and Huberman. Emergent themes were cross-tabulated by sample characteristics using QSR NVivo8. Participant views were mixed regarding the need for a "finishing treatment review" with their oncologist (the primary information source for most families); however, many mothers (29/44) and fathers (17/34) and most siblings (14/15) reported receiving insufficient information post-treatment. Information regarding fertility and how to prepare for likely post-treatment challenges were the most cited unmet needs. Online support was ranked highest by survivors (mean score: 7/2/10) and siblings (7.4/10), whilst parents preferred an information booklet (often due to concerns about accessing accurate and relevant information from the Internet). While many participants reported feelings of isolation/loneliness, many were reluctant to attend face-to-face support groups/seminars. Family members of survivors may experience the most acute unmet needs for information about fertility and in preparation for post-treatment challenges. However, provision of the correct amount of information at the right time for each family member during a highly stressful period remains clinically challenging. Copyright © 2011 Wiley Periodicals, Inc.

Pseudotumor of the pituitary due to PROP-1 deletion.

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Teinturier, C; Vallette, S; Adamsbaum, C; Bendaoud, M; Brue, T; Bougnères, P F

2002-01-01

Hypopituitarism associated with pituitary mass in childhood is most frequently the consequence of craniopharyngioma or Rathke's cleft cyst. We report a patient with an intrasellar pseudotumor associated with hypopituitarism, which led us to a misdiagnosis of intrasellar craniopharyngioma. After spontaneous involution of the mass, diagnosis was revised. DNA analysis showed a deletion in the Prophet of Pit-1 (PROP-1) gene, a pituitary transcription factor. It is important to recognize that a PROP-1 deletion can cause pituitary pseudotumor that can be mistaken for a craniopharyngioma or Rathke's pouch cyst.

Childhood maltreatment and differential treatment response and recurrence in adult major depressive disorder.

Science.gov (United States)

Harkness, Kate L; Bagby, R Michael; Kennedy, Sidney H

2012-06-01

A substantial number of patients with major depressive disorder (MDD) do not respond to treatment, and recurrence rates remain high. The purpose of this study was to examine a history of severe childhood abuse as a moderator of response following a 16-week acute treatment trial, and of recurrence over a 12-month follow-up. Participants included 203 adult outpatients with MDD (129 women; age 18-60). The design was a 16-week single-center randomized, open label trial of interpersonal psychotherapy, cognitive-behavioral therapy, or antidepressant medication, with a 12-month naturalistic follow-up, conducted at a university psychiatry center in Canada. The main outcome measure was Hamilton Depression Rating Scale scores at treatment end point. Childhood maltreatment was assessed at the completion of treatment using an interview-based contextual measure of childhood physical, sexual, and emotional abuse. Multiple imputation was adopted to estimate missing values. Patients with severe maltreatment were significantly less likely to respond to interpersonal psychotherapy than to cognitive-behavioral therapy or medication (OR = 3.61), whereas no differences among treatments were found in those with no history of maltreatment (ORs therapy than from interpersonal psychotherapy. However, these patients remain vulnerable to recurrence regardless of treatment modality.

Treatment-associated subsequent neoplasms among long-term survivors of childhood cancer: the experience of the Childhood Cancer Survivor Study

Energy Technology Data Exchange (ETDEWEB)

Robison, Leslie L. [St. Jude Children' s Research Hospital, Department of Epidemiology and Cancer Control, Memphis, TN (United States)

2009-02-15

With improvements in survival among individuals diagnosed and treated for cancer there is an increasing recognition of the risk of long-term adverse effects of therapy. Second neoplasms represent one of the more serious late effects of treatment and are associated with a substantial level of morbidity and mortality. Survivors of childhood cancers, because of their potential longevity, are at particular risk for this adverse outcome. The Childhood Cancer Survivor Study is a large cohort consisting of adult survivors of childhood cancer diagnosed and treated between 1970 and 1986. The CCSS has provided important data to quantify radiation-associated risk for subsequent cancers including neoplasms of the breast, thyroid and central nervous system. (orig.)

Treatment-associated subsequent neoplasms among long-term survivors of childhood cancer: the experience of the Childhood Cancer Survivor Study

International Nuclear Information System (INIS)

Robison, Leslie L.

2009-01-01

With improvements in survival among individuals diagnosed and treated for cancer there is an increasing recognition of the risk of long-term adverse effects of therapy. Second neoplasms represent one of the more serious late effects of treatment and are associated with a substantial level of morbidity and mortality. Survivors of childhood cancers, because of their potential longevity, are at particular risk for this adverse outcome. The Childhood Cancer Survivor Study is a large cohort consisting of adult survivors of childhood cancer diagnosed and treated between 1970 and 1986. The CCSS has provided important data to quantify radiation-associated risk for subsequent cancers including neoplasms of the breast, thyroid and central nervous system. (orig.)

Randomized controlled trial of a good practice approach to treatment of childhood obesity in Malaysia: Malaysian Childhood Obesity Treatment Trial (MASCOT).

Science.gov (United States)

Wafa, Sharifah W; Talib, Ruzita A; Hamzaid, Nur H; McColl, John H; Rajikan, Roslee; Ng, Lai O; Ramli, Ayiesah H; Reilly, John J

2011-06-01

Few randomized controlled trials (RCTs) of interventions for the treatment of childhood obesity have taken place outside the Western world. To test whether a good practice intervention for the treatment of childhood obesity would have a greater impact on weight status and other outcomes than a control condition in Kuala Lumpur, Malaysia. Assessor-blinded RCT of a treatment intervention in 107 obese 7- to 11-year olds. The intervention was relatively low intensity (8 hours contact over 26 weeks, group based), aiming to change child sedentary behavior, physical activity, and diet using behavior change counselling. Outcomes were measured at baseline and six months after the start of the intervention. Primary outcome was BMI z-score, other outcomes were weight change, health-related quality of life (Peds QL), objectively measured physical activity and sedentary behavior (Actigraph accelerometry over 5 days). The intervention had no significant effect on BMI z score relative to control. Weight gain was reduced significantly in the intervention group compared to the control group (+1.5 kg vs. +3.5 kg, respectively, t-test p < 0.01). Changes in health-related quality of life and objectively measured physical activity and sedentary behavior favored the intervention group. Treatment was associated with reduced rate of weight gain, and improvements in physical activity and quality of life. More substantial benefits may require longer term and more intensive interventions which aim for more substantive lifestyle changes.

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

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 .

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.

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

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

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 .

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.

A Treatment for Dysprosody in Childhood Apraxia of Speech

Science.gov (United States)

Ballard, Kirrie J.; Robin, Donald A.; McCabe, Patricia; McDonald, Jeannie

2010-01-01

Purpose: Dysprosody is considered a core feature of childhood apraxia of speech (CAS), especially impaired production of lexical stress. Few studies have tested the effects of intervention for dysprosody. This Phase II study with 3 children investigated the efficacy of a treatment targeting improved control of relative syllable durations in…

Effects of a Family-Based Childhood Obesity Treatment Program on Parental Weight Status.

Science.gov (United States)

Trier, Cæcilie; Dahl, Maria; Stjernholm, Theresa; Nielsen, Tenna R H; Bøjsøe, Christine; Fonvig, Cilius E; Pedersen, Oluf; Hansen, Torben; Holm, Jens-Christian

2016-01-01

The aim of this study was to investigate the prevalence of overweight/obesity among parents of children entering childhood obesity treatment and to evaluate changes in the parents' weight statuses during their child's treatment. The study included parents of 1,125 children and adolescents aged 3-22 years, who were enrolled in a multidisciplinary childhood obesity treatment program. At baseline, weight and height of the parents were obtained by self-reported information and parental body mass index (BMI) was calculated. Weight and height of the children were measured in the clinic and BMI standard deviation scores were calculated. Furthermore, anthropometric data from parents of 664 children were obtained by telephone interview after a mean of 2.5 years of treatment (ranging 16 days to 7 years), and changes in parental BMI were analyzed. Data on changes in BMI were available in 606 mothers and 479 fathers. At baseline, the median BMI of the mothers was 28.1 kg/m2 (range: 16.9-66.6), and the median BMI of the fathers was 28.9 kg/m2 (range: 17.2-48.1). Seventy percent of the mothers and 80% of the fathers were overweight or obese at the time of their child's treatment initiation. Both the mothers and fathers lost weight during their child's treatment with a mean decrease in BMI in the mothers of 0.5 (95% CI: 0.2-0.8, p = 0.0006) and in the fathers of 0.4 (95% CI: 0.2-0.6, p = 0.0007). Of the overweight/obese parents, 60% of the mothers and 58% of the fathers lost weight during their child's treatment. There is a high prevalence of overweight/obesity among parents of children entering childhood obesity treatment. Family-based childhood obesity treatment with a focus on the child has a positive effect on parental BMI with both mothers and fathers losing weight. ClinicalTrials.gov NCT00928473.

Collecting and Storing Blood and Brain Tumor Tissue Samples From Children With Brain Tumors

Science.gov (United States)

2017-12-11

Childhood Atypical Teratoid/Rhabdoid Tumor; Childhood Central Nervous System Germ Cell Tumor; Childhood Choroid Plexus Tumor; Childhood Craniopharyngioma; Childhood Grade I Meningioma; Childhood Grade II Meningioma; Childhood Grade III Meningioma; Childhood High-grade Cerebral Astrocytoma; Childhood Infratentorial Ependymoma; Childhood Low-grade Cerebral Astrocytoma; Childhood Oligodendroglioma; Childhood Supratentorial Ependymoma; Newly Diagnosed Childhood Ependymoma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma

Random versus Blocked Practice in Treatment for Childhood Apraxia of Speech

Science.gov (United States)

Maas, Edwin; Farinella, Kimberly A.

2012-01-01

Purpose: To compare the relative effects of random vs. blocked practice schedules in treatment for childhood apraxia of speech (CAS). Although there have been repeated suggestions in the literature to use random practice in CAS treatment, no systematic studies exist that have directly compared random with blocked practice in this population.…

Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)—Patient Version

Science.gov (United States)

Childhood non-Hodgkin lymphoma has different types including Burkitt, diffuse large B-cell, primary mediastinal B-cell, lymphoblastic, and anaplastic large cell lymphoma. Get information about the risk factors, symptoms, tests to diagnose, staging, and treatment of all types of newly diagnosed and recurrent NHL and lymphoproliferative disease in this expert-reviewed summary.

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.

Childhood optic atrophy.

Science.gov (United States)

Mudgil, A V; Repka, M X

2000-02-01

To determine the causes, and relative incidence of the common causes, of optic nerve atrophy in children under 10 years old and to compare prevalent aetiologies with those given in previous studies. The Wilmer Information System database was searched to identify all children, diagnosed between 1987 and 1997 with optic atrophy, who were under 10 years old at diagnosis. The medical records of these children were reviewed retrospectively A total of 272 children were identified, Complications from premature birth were the most frequent aetiology of optic atrophy (n = 44, 16%); 68% of these premature infants having a history of intraventricular haemorrhage. Tumour was the second most common aetiology (n = 40, 15%). The most frequent tumour was pilocytic astrocytoma (50%), followed by craniopharyngioma (17%). Hydrocephalus, unrelated to tumour, was the third most common aetiology (n = 26, 10%). In 114 cases (42%), the cause of optic atrophy became manifest in the perinatal period and/or could be attributed to adverse events in utero. A cause was not determined in 4% of cases. In the last decade, prematurity and hydrocephalus appear to have become important causes of optic atrophy in childhood. This trend is probably the result of improved survival of infants with extremely low birth weight.

Brachytherapy in childhood rhabdomyosarcoma treatment

International Nuclear Information System (INIS)

Novaes, Paulo Eduardo Ribeiro dos Santos

1995-01-01

A retrospective study of 21 children with rhabdomyosarcoma treated by brachytherapy to the primary site of the tumor at the Radiotherapy Department of the A.C.Camargo Hospital between january/1980 to june/1993 was undertaken. The main objectives were to comprove the utility of brachytherapy in childhood rhabdomyosarcoma, to evaluate the local control and survival, in association with chemotherapy, to analyze the late effects of the treatment and to determinate the preferential technique to each clinical situation. All patients received brachytherapy to the tumor site. The radioactive isotopes employed were Gold 198 , Cesium 137 and Iridium 192 . The brachytherapy techniques depended on the tumor site, period of treatment, availability of the radioactive material and stage of the disease. Patients treated exclusively by brachytherapy received 40 Gy to 60 Gy. When brachytherapy was associated with external radiotherapy the dose ranged from 20 Gy to 40 Gy. Local control was achieved in 18 of 20 patients (90%). The global survival and local control survival rates were 61.9% (13/21 patients) and 72,2% (13/18 patients) respectively. (author)

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.

Second Malignant Neoplasms After Treatment of Childhood Acute Lymphoblastic Leukemia

DEFF Research Database (Denmark)

Schmiegelow, K.; Levinsen, Mette Frandsen; Attarbaschi, Andishe

2013-01-01

PURPOSE: Second malignant neoplasms (SMNs) after diagnosis of childhood acute lymphoblastic leukemia (ALL) are rare events. PATIENTS AND METHODS: We analyzed data on risk factors and outcomes of 642 children with SMNs occurring after treatment for ALL from 18 collaborative study groups between 19...

Social Learning Family Therapy and the Treatment of Conduct Disorder in Early Childhood: Premise, Procedures and Prospects.

Science.gov (United States)

Mpofu, Elias

1997-01-01

Discusses social learning family therapy hypotheses on the development and sustenance of conduct disorder in early childhood, together with treatment approaches that use parents as the primary agents of change. Reviews research showing that parent training procedures hold much promise for the treatment of conduct disorder in childhood. (JPB)

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.

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

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

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

Late effects of treatment in survivors of childhood acute lymphoblastic leukaemia

International Nuclear Information System (INIS)

Roux, P.

1987-01-01

The overall aim of this study was a comprehensive assessment of the nature and severity of the late effects of treatment in a group of children surviving acute lymphoblastic leukaemia. In the absence of damage preceding treatment, late effects could be ascribed to treatment. Cranial irradiation, methotrexate, L-asparaginase and cytosine arabinoside are therapeutic modalities most likely to cause injury to the central nervous system. Survivors of childhood leukaemia also showed an increase in weight-for-height during and after therapy which appeared to be the consequence of a loss in statural growth as well as increasing weight-for-age. Assessment of endocrine function in leukaemia survivors indicated abnormalities in the regulation of growth hormone and thyroid stimulating hormone in some patients. Survivors of childhood leukaemia were shown to have an intellectual deficit compared with their siblings and a high incidence of visual-perceptual defects. The intellectual effects of lower doses of cranial irradiation are as yet unknown. A variety of minor neurological abnormalities were detected among leukaemia survivors and thought to be related to preceding central nervous system 'prophylactic' chemotherapy and irradiation. A new instrument, the functional deficit score, was derived to reflect overall outcome in survivors of childhood leukaemia. With few exceptions, leukaemia survivors in this study had received 2400 rads of deep x-ray therapy as cranial irradiation. This dosage has since been reduced world-wide. Current cranial irradiation 'prophylaxis' consists of 1800 rad of megavoltage radiotherapy

Late effects of treatment in survivors of childhood acute lymphoblastic leukaemia

Energy Technology Data Exchange (ETDEWEB)

Roux, P

1987-01-01

The overall aim of this study was a comprehensive assessment of the nature and severity of the late effects of treatment in a group of children surviving acute lymphoblastic leukaemia. In the absence of damage preceding treatment, late effects could be ascribed to treatment. Cranial irradiation, methotrexate, L-asparaginase and cytosine arabinoside are therapeutic modalities most likely to cause injury to the central nervous system. Survivors of childhood leukaemia also showed an increase in weight-for-height during and after therapy which appeared to be the consequence of a loss in statural growth as well as increasing weight-for-age. Assessment of endocrine function in leukaemia survivors indicated abnormalities in the regulation of growth hormone and thyroid stimulating hormone in some patients. Survivors of childhood leukaemia were shown to have an intellectual deficit compared with their siblings and a high incidence of visual-perceptual defects. The intellectual effects of lower doses of cranial irradiation are as yet unknown. A variety of minor neurological abnormalities were detected among leukaemia survivors and thought to be related to preceding central nervous system 'prophylactic' chemotherapy and irradiation. A new instrument, the functional deficit score, was derived to reflect overall outcome in survivors of childhood leukaemia. With few exceptions, leukaemia survivors in this study had received 2400 rads of deep x-ray therapy as cranial irradiation. This dosage has since been reduced world-wide. Current cranial irradiation 'prophylaxis' consists of 1800 rad of megavoltage radiotherapy.

Childhood Soft Tissue Sarcoma Treatment (PDQ®)—Health Professional Version

Science.gov (United States)

Pediatric soft tissue sarcomas are a heterogenous group of malignant tumors that originate from primitive mesenchymal tissue and account for 7% of all childhood tumors. Get detailed information about clinical presentation, diagnosis, prognosis, and treatment of newly diagnosed and recurrent soft tissue sarcoma in this summary for clinicians.

Childhood Thyroid Cancer Treatment (PDQ®)—Health Professional Version