Full Text Available Objective: To present a rare case of gigantism. Case Report: A 25-year-old lady presented with increased statural growth and enlarged body parts noticed since the age of 14 years, primary amenorrhea, and frontal headache for the last 2 years.She has also been suffering from non-inflammatory low back pain with progressive kyphosis and pain in the knees, ankles, and elbows for the last 5 years. There was no history of visual disturbance, vomiting, galactorrhoea, cold intolerance. She had no siblings. Family history was non-contributory.Blood pressure was normal. Height 221 cm, weight 138 kg,body mass index (BMI28. There was coarsening of facial features along with frontal bossing and prognathism, large hands and feet, and small goitre. Patient had severe kyphosis and osteoarthritis of knees. Confrontation perimetry suggested bitemporal hemianopia. Breast and pubic hair were of Tanner stage 1. Serum insulin like growth factor-1 (IGF1 was 703 ng/ml with all glucose suppressedgrowth hormone (GHvalues of >40 ng/ml. Prolactin was 174 ng/ml. Basal serum Lutenising Hormone (LH, follicle stimulating Hormone (FSH was low. Oral glucose tolerance test (OGTT, liver and renal function tests, basal cortisol and thyroid profile, Calcium, phosphorus and Intact Parathyroid hormone (iPTH were normal.Computed tomographyscan of brain showed large pituitary macroadenoma. Automated perimetry confirmed bitemporal hemianopia. A diagnosis of gigantism due to GH secreting pituitary macroadenoma with hypogonadotrophichypogonadism was made. Debulking pituitary surgery followed by somatostatin analogue therapy with gonadal steroid replacement had been planned, but the patient refused further treatment.
Bhattacharjee, Rana; Roy, Ajitesh; Goswami, Soumik; Selvan, Chitra; Chakraborty, Partha P; Ghosh, Sujoy; Biswas, Dibakar; Dasgupta, Ranen; Mukhopadhyay, Satinath; Chowdhury, Subhankar
To present a rare case of gigantism. A 25-year-old lady presented with increased statural growth and enlarged body parts noticed since the age of 14 years, primary amenorrhea, and frontal headache for the last 2 years. She has also been suffering from non-inflammatory low back pain with progressive kyphosis and pain in the knees, ankles, and elbows for the last 5 years. There was no history of visual disturbance, vomiting, galactorrhoea, cold intolerance. She had no siblings. Family history was non-contributory. Blood pressure was normal. Height 221 cm, weight 138 kg, body mass index (BMI)28. There was coarsening of facial features along with frontal bossing and prognathism, large hands and feet, and small goitre. Patient had severe kyphosis and osteoarthritis of knees. Confrontation perimetry suggested bitemporal hemianopia. Breast and pubic hair were of Tanner stage 1. Serum insulin like growth factor-1 (IGF1) was 703 ng/ml with all glucose suppressedgrowth hormone (GH)values of >40 ng/ml. Prolactin was 174 ng/ml. Basal serum Lutenising Hormone (LH), follicle stimulating Hormone (FSH) was low. Oral glucose tolerance test (OGTT), liver and renal function tests, basal cortisol and thyroid profile, Calcium, phosphorus and Intact Parathyroid hormone (iPTH) were normal. Computed tomographyscan of brain showed large pituitary macroadenoma. Automated perimetry confirmed bitemporal hemianopia. A diagnosis of gigantism due to GH secreting pituitary macroadenoma with hypogonadotrophichypogonadism was made. Debulking pituitary surgery followed by somatostatin analogue therapy with gonadal steroid replacement had been planned, but the patient refused further treatment.
Lu, P W; Silink, M; Johnston, I; Cowell, C T; Jimenez, M
A case of pituitary gigantism resulting from a pituitary adenoma which secreted growth hormone is described. The patient was successfully treated by surgery, which led to the normalisation of endogenous growth hormone secretion. An acceptable final height was achieved with high dose intramuscular testosterone treatment. Images Figure 1 PMID:1520009
Lu, P W; Silink, M; Johnston, I; Cowell, C T; Jimenez, M
A case of pituitary gigantism resulting from a pituitary adenoma which secreted growth hormone is described. The patient was successfully treated by surgery, which led to the normalisation of endogenous growth hormone secretion. An acceptable final height was achieved with high dose intramuscular testosterone treatment.
Rana Bhattacharjee; Ajitesh Roy; Soumik Goswami; Chitra Selvan; Partha P Chakraborty; Sujoy Ghosh; Dibakar Biswas; Ranen Dasgupta; Satinath Mukhopadhyay; Subhankar Chowdhury
Objective: To present a rare case of gigantism. Case Report: A 25-year-old lady presented with increased statural growth and enlarged body parts noticed since the age of 14 years, primary amenorrhea, and frontal headache for the last 2 years. She has also been suffering from non-inflammatory low back pain with progressive kyphosis and pain in the knees, ankles, and elbows for the last 5 years. There was no history of visual disturbance, vomiting, galactorrhoea, cold intolerance. She had no si...
Creo, Ana L; Lteif, Aida N
Pituitary gigantism (PG) is a rare pediatric disease with poorly defined long-term outcomes. Our aim is to describe the longitudinal clinical course in PG patients using a single-center, retrospective cohort study. Patients younger than 19 years diagnosed with PG were identified. Thirteen cases were confirmed based on histopathology of a GH secreting adenoma or hyperplasia and a height >2 SD for age and gender. Laboratory studies, initial pathology, and imaging were abstracted. Average age at diagnosis was 13 years with an average initial tumor size of 7.4×3.8 mm. Initial transsphenoidal surgery was curative in 3/12 patients. Four of the nine patients who failed the initial surgery required a repeat procedure. Octreotide successfully normalized GH levels in 1/6 patients with disease refractory to surgery (1/6). Two out of five patients received pegvisomant after failing octreotide but only one patient responded to treatment. Five patients were ultimately treated with radiosurgery or radiation patients were followed for an average of 10 years. PG is difficult to treat. In most patients, the initial transsphenoidal surgery failed to normalize GH levels. If the initial surgery was unsuccessful, repeat surgery was unlikely to control GH secretion. Treatment with octreotide or pegvisomant was successful in less than half the patients failing surgery. Radiosurgery was curative, but is not an optimal treatment for pediatric patients. Despite the small sample, our study suggests that the treatment outcome of pediatric PG may be different than adults.
Rostomyan, Liliya; Daly, Adrian F; Beckers, Albert
Acromegaly and pituitary gigantism are very rare conditions resulting from excessive secretion of growth hormone (GH), usually by a pituitary adenoma. Pituitary gigantism occurs when GH excess overlaps with the period of rapid linear growth during childhood and adolescence. Until recently, its etiology and clinical characteristics have been poorly understood. Genetic and genomic causes have been identified in recent years that explain about half of cases of pituitary gigantism. We describe these recent discoveries and focus on some important settings in which gigantism can occur, including familial isolated pituitary adenomas (FIPA) and the newly described X-linked acrogigantism (X-LAG) syndrome. Copyright © 2015 Elsevier Masson SAS. All rights reserved.
Peillon, F; Philippon, J; Brandi, A M; Fohanno, D; Laplane, D; Dubois, M P; Decourt, J
A prolactin-secreting pituitary adenoma was removed trans-sphenoidally from a 37 years old man with gigantism (218 cm). Serum levels of prolactin (PRL) were elevated pre-operatively and decreased after administration of L-Dopa with no increase after TRH as is usually observed in PRL-secreting adenomas. Growth hormone (GH) and somatomedin serum levels were normal with no modification of GH after insulin hypoglycemia, oral glucose loading or L-Dopa. Morphological examination of the tumour demonstrated the presence of lactotrophs by light and electron microscopy and by immunofluorescense staining. No somatotrophs were found. In this unique case, the relationship between a PRL-secreting adenoma and gigantism is discussed.
Gläsker, Sven; Vortmeyer, Alexander O; Lafferty, Antony R A; Hofman, Paul L; Li, Jie; Weil, Robert J; Zhuang, Zhengping; Oldfield, Edward H
We report hereditary pituitary hyperplasia. The objective of the study was to describe the results of the clinical and laboratory analysis of this rare instance of hereditary pituitary hyperplasia. The study is a retrospective analysis of three cases from one family. The study was conducted at the National Institutes of Health, a tertiary referral center. A mother and both her sons had very early-onset gigantism associated with high levels of serum GH and prolactin. The condition was treated by total hypophysectomy. We performed clinical, pathological, and molecular evaluations, including evaluation basal and provocative endocrine testing, neuroradiological assessment, and assessment of the pituitary tissue by microscopic evaluation, immunohistochemistry, and electron microscopy. All three family members had very early onset of gigantism associated with abnormally high serum levels of GH and prolactin. Serum GHRH levels were not elevated in either of the boys. The clinical, radiographic, surgical, and histological findings indicated mammosomatotroph hyperplasia. The pituitary gland of both boys revealed diffuse mammosomatotroph hyperplasia of the entire pituitary gland without evidence of adenoma. Prolactin and GH were secreted by the same cells within the same secretory granules. Western blot and immunohistochemistry demonstrated expression of GHRH in clusters of cells distributed throughout the hyperplastic pituitary of both boys. This hereditary condition seems to be a result of embryonic pituitary maldevelopment with retention and expansion of the mammosomatotrophs. The findings suggest that it is caused by paracrine or autocrine pituitary GHRH secretion during pituitary development.
Lodish, Maya B; Trivellin, Giampaolo; Stratakis, Constantine A
To provide an update on the mechanisms leading to pituitary gigantism, as well as to familiarize the practitioner with the implication of these genetic findings on treatment decisions. Prior studies have identified gigantism as a feature of a number of monogenic disorders, including mutations in the aryl hydrocarbon receptor interacting protein gene, multiple endocrine neoplasia types 1 and 4, McCune Albright syndrome, Carney complex, and the paraganglioma, pheochromocytoma, and pituitary adenoma association because of succinate dehydrogenase defects. We recently described a previously uncharacterized form of early-onset pediatric gigantism caused by microduplications on chromosome Xq26.3 and we termed it X-LAG (X-linked acrogigantism). The age of onset of increased growth in X-LAG is significantly younger than other pituitary gigantism cases, and control of growth hormone excess is particularly challenging. Knowledge of the molecular defects that underlie pituitary tumorigenesis is crucial for patient care as they guide early intervention, screening for associated conditions, genetic counseling, surgical approach, and choice of medical management. Recently described microduplications of Xq26.3 account for more than 80% of the cases of early-onset pediatric gigantism. Early recognition of X-LAG may improve outcomes, as successful control of growth hormone excess requires extensive anterior pituitary resection and are difficult to manage with medical therapy alone.
Alvi, N S; Kirk, J M
Although growth hormone excess (acromegaly) in association with glucose intolerance and diabetes mellitus is well documented in adult medicine, it is much less common in the paediatric age group. We report the case of a 13 year-old boy who presented with tall stature secondary to a large growth hormone secreting adenoma of the pituitary gland. Random growth hormone was 630 mIU/l and did not suppress during an oral glucose tolerance test. Following debulking of the tumour, he developed diabetic ketoacidosis requiring insulin treatment, but after further surgery glucose handling returned to normal. He has been started on testosterone to arrest further increase in height.
Ali, Omar; Banerjee, Swati; Kelly, Daniel F; Lee, Phillip D K
Pituitary gigantism, a condition of endogenous growth hormone (GH) hypersecretion prior to epiphyseal closure, is a rare condition. In the adult condition of GH excess, acromegaly, the occurrence of type 2 diabetes mellitus (T2DM) and diabetic ketoacidosis (DKA) have been reported, with resolution following normalization of GH levels. We report the case of a 16-year-old male with pituitary gigantism due to a large invasive suprasellar adenoma who presented with T2DM and DKA. Despite surgical de-bulking, radiotherapy and medical treatment with cabergoline and pegvisomant, GH and insulin-like growth factor-I (IGF-I) levels remained elevated. However, the T2DM and recurrent DKA were successfully managed with metformin and low-dose glargine insulin, respectively. We review the pathophysiology of T2DM and DKA in growth hormone excess and available treatment options.
Rhee, Noorisaem; Jeong, Kumi; Yang, Eun Mi; Kim, Chan Jong
Gigantism indicates excessive secretion of growth hormones (GH) during childhood when open epiphyseal growth plates allow for excessive linear growth. Case one involved a 14.7-year-old boy presented with extreme tall stature. His random serum GH level was 38.4 ng/mL, and failure of GH suppression was noted during an oral glucose tolerance test (OGTT; nadir serum GH, 22.7 ng/mL). Magnetic resonance imaging (MRI) of the brain revealed a 12-mm-sized pituitary adenoma. Transsphenoidal surgery was performed and a pituitary adenoma displaying positive immunohistochemical staining for GH was reported. Pituitary MRI scan was performed 4 months after surgery and showed recurrence/residual tumor. Medical treatment with a long-acting somatostatin analogue for six months was unsuccessful. As a result, secondary surgery was performed. Three months after reoperation, the GH level was 0.2 ng/mL and insulin-like growth factor 1 was 205 ng/mL. Case two involved a 14.9-year-old boy, who was referred to our department for his tall stature. His basal GH level was 9.3 ng/mL, and failure of GH suppression was reported during OGTT (nadir GH, 9.0 ng/mL). Pituitary MRI showed a 6-mm-sized pituitary adenoma. Surgery was done and histopathological examination demonstrated a pituitary adenoma with positive staining for GH. Three months after surgery, the GH level was 0.2 ng/mL and nadir GH during OGTT was less than 0.1 ng/mL. Pituitary MRI scans showed no residual tumor. We present two cases of gigantism caused by a GH-secreting pituitary adenoma with clinical and microscopic findings.
Full Text Available Gigantism indicates excessive secretion of growth hormones (GH during childhood when open epiphyseal growth plates allow for excessive linear growth. Case one involved a 14.7-year-old boy presented with extreme tall stature. His random serum GH level was 38.4 ng/mL, and failure of GH suppression was noted during an oral glucose tolerance test (OGTT; nadir serum GH, 22.7 ng/mL. Magnetic resonance imaging (MRI of the brain revealed a 12-mm-sized pituitary adenoma. Transsphenoidal surgery was performed and a pituitary adenoma displaying positive immunohistochemical staining for GH was reported. Pituitary MRI scan was performed 4 months after surgery and showed recurrence/residual tumor. Medical treatment with a long-acting somatostatin analogue for six months was unsuccessful. As a result, secondary surgery was performed. Three months after reoperation, the GH level was 0.2 ng/mL and insulin-like growth factor 1 was 205 ng/mL. Case two involved a 14.9-year-old boy, who was referred to our department for his tall stature. His basal GH level was 9.3 ng/mL, and failure of GH suppression was reported during OGTT (nadir GH, 9.0 ng/mL. Pituitary MRI showed a 6-mm-sized pituitary adenoma. Surgery was done and histopathological examination demonstrated a pituitary adenoma with positive staining for GH. Three months after surgery, the GH level was 0.2 ng/mL and nadir GH during OGTT was less than 0.1 ng/mL. Pituitary MRI scans showed no residual tumor. We present two cases of gigantism caused by a GH-secreting pituitary adenoma with clinical and microscopic findings.
Rhee, Noorisaem; Jeong, Kumi; Yang, Eun Mi
Gigantism indicates excessive secretion of growth hormones (GH) during childhood when open epiphyseal growth plates allow for excessive linear growth. Case one involved a 14.7-year-old boy presented with extreme tall stature. His random serum GH level was 38.4 ng/mL, and failure of GH suppression was noted during an oral glucose tolerance test (OGTT; nadir serum GH, 22.7 ng/mL). Magnetic resonance imaging (MRI) of the brain revealed a 12-mm-sized pituitary adenoma. Transsphenoidal surgery was performed and a pituitary adenoma displaying positive immunohistochemical staining for GH was reported. Pituitary MRI scan was performed 4 months after surgery and showed recurrence/residual tumor. Medical treatment with a long-acting somatostatin analogue for six months was unsuccessful. As a result, secondary surgery was performed. Three months after reoperation, the GH level was 0.2 ng/mL and insulin-like growth factor 1 was 205 ng/mL. Case two involved a 14.9-year-old boy, who was referred to our department for his tall stature. His basal GH level was 9.3 ng/mL, and failure of GH suppression was reported during OGTT (nadir GH, 9.0 ng/mL). Pituitary MRI showed a 6-mm-sized pituitary adenoma. Surgery was done and histopathological examination demonstrated a pituitary adenoma with positive staining for GH. Three months after surgery, the GH level was 0.2 ng/mL and nadir GH during OGTT was less than 0.1 ng/mL. Pituitary MRI scans showed no residual tumor. We present two cases of gigantism caused by a GH-secreting pituitary adenoma with clinical and microscopic findings. PMID:25077093
Stock, J.M.; Ghatak, N.R.; Oppenheimer, J.H.
A unique example of a clinically unsuspected large parasellar meningioma is described in a 36-yr-old pituitary giant who had been treated initially with conventional irradiation, subsequently by surgical excision of an acidophil adenoma, and ultimately with medroxyprogesterone acetate (MPA) prior to his demise. The patient died of increased intracranial pressure resulting from a combined mass effect of the meningioma and recurrent tumor. The relationship between radiation and the development of the meningioma is discussed, as well as the fine ultrastructure of a highly functioning acidophil adenoma.
Stock, J.M.; Ghatak, N.R.; Oppenheimer, J.H.
A unique example of a clinically unsuspected large parasellar meningioma is described in a 36-yr-old pituitary giant who had been treated initially with conventional irradiation, subsequently by surgical excision of an acidophil adenoma, and ultimately with medroxyprogesterone acetate (MPA) prior to his demise. The patient died of increased intracranial pressure resulting from a combined mass effect of the meningioma and recurrent tumor. The relationship between radiation and the development of the meningioma is discussed, as well as the fine ultrastructure of a highly functioning acidophil adenoma
Rhee, Noorisaem; Jeong, Kumi; Yang, Eun Mi; Kim, Chan Jong
Gigantism indicates excessive secretion of growth hormones (GH) during childhood when open epiphyseal growth plates allow for excessive linear growth. Case one involved a 14.7-year-old boy presented with extreme tall stature. His random serum GH level was 38.4 ng/mL, and failure of GH suppression was noted during an oral glucose tolerance test (OGTT; nadir serum GH, 22.7 ng/mL). Magnetic resonance imaging (MRI) of the brain revealed a 12-mm-sized pituitary adenoma. Transsphenoidal surgery was...
Goldenberg, Naila; Racine, Michael S; Thomas, Pamela; Degnan, Bernard; Chandler, William; Barkan, Ariel
Treatment of pituitary gigantism is complex and the results are usually unsatisfactory. The objective of the study was to describe the results of therapy of three children with pituitary gigantism by a GH receptor antagonist, pegvisomant. This was a descriptive case series of up to 3.5 yr duration. The study was conducted at a university hospital. Patients included three children (one female, two males) with pituitary gigantism whose GH hypersecretion was incompletely controlled by surgery, somatostatin analog, and dopamine agonist. The intervention was administration of pegvisomant. Plasma IGF-I and growth velocity were measured. In all three children, pegvisomant rapidly decreased plasma IGF-I concentrations. Growth velocity declined to subnormal or normal values. Statural growth fell into lower growth percentiles and acromegalic features resolved. Pituitary tumor size did not change in two children but increased in one boy despite concomitant therapy with a somatostatin analog. Pegvisomant may be an effective modality for the therapy of pituitary gigantism in children. Titration of the dose is necessary for optimal efficacy, and regular surveillance of tumor size is mandatory.
Arisaka, O; Hall, R; Hughes, I A
An 11 year old, tall boy presented with symptoms typical of pituitary apoplexy. A large necrotic and haemorrhagic tumour was removed, which was shown to be an adenoma secreting growth hormone and prolactin. Subsequent treatment comprised cranial irradiation and hormone replacement. Eighteen months after operation growth was static and plasma growth hormone and prolactin concentrations were undetectable. Treatment of pituitary apoplexy should comprise excision of the tumour and postoperative irradiation; such treatment after early recognition of the condition offers the best chance of preserving normal pituitary function in children with gigantism.
Arisaka, O; Hall, R; Hughes, I A
An 11 year old, tall boy presented with symptoms typical of pituitary apoplexy. A large necrotic and haemorrhagic tumour was removed, which was shown to be an adenoma secreting growth hormone and prolactin. Subsequent treatment comprised cranial irradiation and hormone replacement. Eighteen months after operation growth was static and plasma growth hormone and prolactin concentrations were undetectable. Treatment of pituitary apoplexy should comprise excision of the tumour and postoperative irradiation; such treatment after early recognition of the condition offers the best chance of preserving normal pituitary function in children with gigantism. PMID:6311318
Wang, Xian-Ling; Dou, Jing-Tao; Lü, Zhao-Hui; Zhong, Wen-Wen; Ba, Jian-Ming; Jin, Du; Lu, Ju-Ming; Pan, Chang-Yu; Mu, Yi-Ming
Subclinical apoplexy of pituitary functional adenoma can cause spontaneous remission of hormone hypersecretion. The typical presence of pituitary growth hormone (GH) adenoma is gigantism and/or acromegaly. We investigated the clinical characteristics of patients with spontaneous partial remission of acromegaly or gigantism due to subclinical apoplexy of GH adenoma. Six patients with spontaneous remission of acromegaly or gigantism were enrolled. The clinical characteristics, endocrinological evaluation and imageological characteristics were retrospectively analyzed. In these cases, the initial clinical presences were diabetes mellitus or hypogonadism. No abrupt headache, vomiting, visual function impairment, or conscious disturbance had ever been complained of. The base levels of GH and insulin growth factor-1 (IGF-1) were normal or higher, but nadir GH levels were all still > 1 µg/L in 75 g oral glucose tolerance test. Magnetic resonance imaging detected enlarged sella, partial empty sella and compressed pituitary. The transsphenoidal surgery was performed in 2 cases, and the other patients were conservatively managed. All the patients were in clinical remission. When the clinical presences, endocrine evaluation, biochemical examination and imageology indicate spontaneous remission of GH hypersecretion in patients with gigantism or acromegaly, the diagnosis of subclinical apoplexy of pituitary GH adenoma should be presumed. To these patients, conservative therapy may be appropriate.
Espiner, E A; Carter, T A; Abbott, G D; Wrightson, P
A case of pituitary gigantism occurring in a 31 month old female child is reported. Growth records indicate that the disorder began early in the second yr of life. Apart from her size and history of excessive sweating, there were no characteristic clinical features of endocrinopathy. Elevated and autonomous secretion of GH (60-109 microgram/l) and prolactin were corrected by the removal of an eosinophilic pituitary adenoma. In the subsequent 6 yr, despite the presence of immunoreactive GH (4.6-17.3 microgram/l), plasma somatomedin was subnormal and the patient showed growth failure which responded normally to exogenous GH therapy. This case, which appears to be the youngest example of verified pituitary gigantism on record, illustrates that a successful outcome can be achieved by surgical ablative therapy.
Mangupli, Ruth; Rostomyan, Liliya; Castermans, Emilie; Caberg, Jean-Hubert; Camperos, Paul; Krivoy, Jaime; Cuauro, Elvia; Bours, Vincent; Daly, Adrian F; Beckers, Albert
Pituitary gigantism is a rare condition caused by growth hormone secreting hypersecretion, usually by a pituitary tumor. Acromegaly and gigantism cases that have a genetic cause are challenging to treat, due to large tumor size and poor responses to some medical therapies (e.g. AIP mutation affected cases and those with X-linked acrogigantism syndrome). We performed a retrospective study to identify gigantism cases among 160 somatotropinoma patients treated between 1985 and 2015 at the University Hospital of Caracas, Venezuela. We studied clinical details at diagnosis, hormonal responses to therapy and undertook targeted genetic testing. Among the 160 cases, eight patients (six males; 75 %) were diagnosed with pituitary gigantism and underwent genetic analysis that included array comparative genome hybridization for Xq26.3 duplications. All patients had GH secreting pituitary macroadenomas that were difficult to control with conventional treatment options, such as surgery or primary somatostatin receptor ligand (SRL) therapy. Combined therapy (long-acting SRL and pegvisomant) as primary treatment or after pituitary surgery and radiotherapy permitted the normalization of IGF-1 levels and clinical improvement. Novel AIP mutations were the found in three patients. None of the patients had Xq26.3 microduplications. Treatment of pituitary gigantism is frequently challenging; delayed control increases the harmful effects of GH excess, such as, excessive stature and symptom burden, so early diagnosis and effective treatment are particularly important in these cases.
Ritzén, E M; Wettrell, G; Davies, G; Grant, D B
True gigantism with overproduction of growth hormone (GH) and prolactin (PRL) was diagnosed in two boys, aged 13 years (case I) and 7 1/2 years (case II). Both had shown increased growth rates since early childhood (from 4 years and 1 1/2 years, respectively), but no skeletal acromegalic features were noted. However, both showed increased sweating and both had advanced pubic hair relative to testis volume. No other pituitary dysfunction was recorded. Case I underwent transsphenoidal surgery with only incomplete and temporary suppression of GH and PRL levels. However, in both patients bromocriptine administration promptly suppressed PRL levels. Following combined irradiation and bromocriptine treatment, GH also gradually normalized over a period of 2 years. Both boys are still on treatment, and both showed an increase in plasma GH concentrations when the dose of bromocriptine was reduced or discontinued, indicating that even 3 1/2-5 years after irradiation therapy (and during continuous treatment with bromocriptine) the disease was controlled but not cured. However, in these two boys bromocriptine has proved effective in controlling the PRL/GH oversecretion.
Matsuno, A; Teramoto, A; Yamada, S; Kitanaka, S; Tanaka, T; Sanno, N; Osamura, R Y; Kirino, T
The cases of gigantism sisters with somatotroph adenomas unrelated to multiple endocrine neoplasia (MEN) Type 1 are reported. The sisters grew rapidly since they were 5 or 6 years old and were diagnosed to have gigantism with pituitary adenoma by computed tomographic scan and magnetic resonance imaging. A serum endocrinological examination showed the elevated growth hormone values. After thyroxine-releasing hormone stimulation, growth hormone values exhibited a paradoxical rise. They were supposed to be unrelated to MEN Type 1, because analysis of the 11th chromosomes and the other endocrine functions were normal. They were operated on by the transphenoidal method. Immunohistochemical staining of both tumor specimens confirmed somatotroph adenomas. Pituitary adenoma associated with MEN Type 1 is a well-recognized entity. However, the sporadic occurrence of pituitary adenoma unrelated to MEN Type 1, especially in siblings, is extremely rare. Fifteen cases of pituitary adenomas in siblings were described in the literature. As for gigantism, only two brothers were reported. Our case of gigantism sisters is the second sporadic case. In our review of the isolated cases of pituitary adenoma in siblings described in the literature, 12 (70%) of 17 cases including ours are acromegaly or gigantism. This incidence is much higher than that of MEN Type 1 patients with pituitary adenomas. The cause of the familial occurrence of pituitary adenomas is still unclear, although autosomal recessive inheritance has been suggested. It has been stated that point mutations in codon 201 or 227 of the Gs alpha gene located in chromosome 20 were found in about 35 to 40% of somatotroph adenomas.(ABSTRACT TRUNCATED AT 250 WORDS)
Araki, Y; Sakai, N; Andoh, T; Yoshimura, S; Yamada, H
We report a case of central neurocytoma presenting with gigantism. The patient was a 19-year-old man with a 2-year history of rapid growth. Computed tomography revealed a round, slightly enhancing calcified tumor in the septal region. This lesion was resected, and postoperative radiotherapy was given. The preoperative serum growth hormone level was 20.7 ng/mL, and postoperatively this fell to 0.9 ng/mL. Pituitary dysfunction was not noted either before or after the operation. A low level of production of growth hormone releasing factor was detected when tumor cells obtained during surgery were cultured.
Lodish, Maya B.; Trivellin, Giampaolo; Stratakis, Constantine A.
Purpose of review To provide an update on the mechanisms leading to pituitary gigantism, as well as to familiarize the practitioner with the implication of these genetic findings on treatment decisions. Recent findings Prior studies have identified gigantism as a feature of a number of monogenic disorders, including mutations in the aryl hydrocarbon receptor interacting protein (AIP) gene, multiple endocrine neoplasia types 1 and 4, McCune Albright Syndrome, Carney Complex, and the paraganglioma, pheochromocytoma and pituitary adenoma association (3PA) due to succinate dehydrogenase defects. We recently described a previously uncharacterized form of early-onset pediatric gigantism caused by microduplications on chromosome Xq26.3 and we termed it X-LAG (X-linked acrogigantism). The age of onset of increased growth in X-LAG is significantly younger than other pituitary gigantism cases, and control of growth hormone excess is particularly challenging. Summary Knowledge of the molecular defects that underlie pituitary tumorigenesis is crucial for patient care as they guide early intervention, screening for associated conditions, genetic counseling, surgical approach (partial or total hypophysectomy), and choice of medical management. Recently described microduplications of Xq26.3 account for more than 80% of the cases of early-onset pediatric gigantism. Early recognition of X-LAG may improve outcomes, as successful control of growth hormone excess requires extensive anterior pituitary resection and are difficult to manage with medical therapy alone. PMID:26574647
Rostomyan, Liliya; Daly, Adrian F; Petrossians, Patrick; Nachev, Emil; Lila, Anurag R; Lecoq, Anne-Lise; Lecumberri, Beatriz; Trivellin, Giampaolo; Salvatori, Roberto; Moraitis, Andreas G; Holdaway, Ian; Kranenburg-van Klaveren, Dianne J; Chiara Zatelli, Maria; Palacios, Nuria; Nozieres, Cecile; Zacharin, Margaret; Ebeling, Tapani; Ojaniemi, Marja; Rozhinskaya, Liudmila; Verrua, Elisa; Jaffrain-Rea, Marie-Lise; Filipponi, Silvia; Gusakova, Daria; Pronin, Vyacheslav; Bertherat, Jerome; Belaya, Zhanna; Ilovayskaya, Irena; Sahnoun-Fathallah, Mona; Sievers, Caroline; Stalla, Gunter K; Castermans, Emilie; Caberg, Jean-Hubert; Sorkina, Ekaterina; Auriemma, Renata Simona; Mittal, Sachin; Kareva, Maria; Lysy, Philippe A; Emy, Philippe; De Menis, Ernesto; Choong, Catherine S; Mantovani, Giovanna; Bours, Vincent; De Herder, Wouter; Brue, Thierry; Barlier, Anne; Neggers, Sebastian J C M M; Zacharieva, Sabina; Chanson, Philippe; Shah, Nalini Samir; Stratakis, Constantine A; Naves, Luciana A; Beckers, Albert
Despite being a classical growth disorder, pituitary gigantism has not been studied previously in a standardized way. We performed a retrospective, multicenter, international study to characterize a large series of pituitary gigantism patients. We included 208 patients (163 males; 78.4%) with growth hormone excess and a current/previous abnormal growth velocity for age or final height >2 s.d. above country normal means. The median onset of rapid growth was 13 years and occurred significantly earlier in females than in males; pituitary adenomas were diagnosed earlier in females than males (15.8 vs 21.5 years respectively). Adenomas were ≥10 mm (i.e., macroadenomas) in 84%, of which extrasellar extension occurred in 77% and invasion in 54%. GH/IGF1 control was achieved in 39% during long-term follow-up. Final height was greater in younger onset patients, with larger tumors and higher GH levels. Later disease control was associated with a greater difference from mid-parental height (r=0.23, P=0.02). AIP mutations occurred in 29%; microduplication at Xq26.3 - X-linked acrogigantism (X-LAG) - occurred in two familial isolated pituitary adenoma kindreds and in ten sporadic patients. Tumor size was not different in X-LAG, AIP mutated and genetically negative patient groups. AIP-mutated and X-LAG patients were significantly younger at onset and diagnosis, but disease control was worse in genetically negative cases. Pituitary gigantism patients are characterized by male predominance and large tumors that are difficult to control. Treatment delay increases final height and symptom burden. AIP mutations and X-LAG explain many cases, but no genetic etiology is seen in >50% of cases. © 2015 Society for Endocrinology.
Otsuka, Fumio; Mizobuchi, Satoshi; Ogura, Toshio; Sato, Kenji; Yokoyama, Masataka; Makino, Hirofumi
We report the case of 19-year-old man with pituitary gigantism due to growth hormone-producing pituitary macroadenoma. The patient complained of recurrent headache and excessive growth spurt since age 15. Octreotide administration was initiated following transsphenoidal pituitary adenomectomy. Octreotide injection for 4 years efficaciously reduced the size of remnant adenoma as well as serum growth hormone levels. Notably, octreotide exhibited a potent analgesic effect on his intractable cluster headache that has continued even after reduction of the adenoma volume. The analgesic effect lasted 2 to 6 hours after each injection and no tachyphylaxis to octreotide appeared during 4-year treatment. To characterize the headache and the pain intensity, analgesic drugs including octreotide, lidocaine, morphine and thiopental were tested using a visual analogue scale (VAS) evaluation, with the result that octreotide exhibited a prompt and complete disappearance of the headache. Headache relief was in part reproduced by morphine injection (56% reduction) but not by lidocaine or thiopental. The present case suggests that the intractable headache associated with pituitary gigantism is possibly related to the endogenous opioid system. Thus, the headache control by octreotide is clinically helpful for continuation of the self-injection regimen.
Müssig, K; Gallwitz, B; Honegger, J; Strasburger, C J; Bidlingmaier, M; Machicao, F; Bornemann, A; Ranke, M B; Häring, H-U; Petersenn, S
Gigantism is rare with the majority of cases caused by a growth hormone (GH)-secreting pituitary adenoma. Treatment options for GH-secreting pituitary adenomas have been widened with the availability of long-acting dopamine agonists, depot preparations of somatostatin analogues, and recently the GH receptor antagonist pegvisomant. A 23-year-old male patient presented with continuous increase in height during the past 6 years due to a GH-secreting giant pituitary adenoma. Because of major intracranial extension and failure of octreotide treatment to shrink the tumour, the tumour was partially resected by a trans-frontal surgical approach. At immunohistochemistry, the tumour showed a marked expression of GH and a sparsely focal expression of prolactin. Somatostatin receptors (sst) 1-5 were not detected. Tumour tissue weakly expressed dopamine receptor type 2. The Gs alpha subunit was intact. Conversion from somatostatin analogue to pegvisomant normalized insulin-like-growth-factor-I (IGF-I) levels and markedly improved glucose tolerance. Pegvisomant is a potent treatment option in patients with pituitary gigantism. In patients who do not respond to somatostatin analogues, knowledge of the SST receptor status may shorten the time to initiation of pegvisomant treatment.
Kuo, Sheng-Fong; Chuang, Wen-Yu; Ng, Sohching; Chen, Chih-Hung; Chang, Chen-Nen; Chou, Chi-Hsiang; Weng, Wei-Chieh; Yeh, Chih-Hua; Lin, Jen-Der
Hyperglycemia is seldom described in young patients with pituitary gigantism. Here, we describe the case of a 17-year-old Taiwanese boy who developed depressive mood disorder and diabetic ketoacidosis (DKA) at the presentation of pituitary gigantism. The boy complained of lethargy and dysphoric mood in June 2008. He presented at the emergency department with epigastralgia and dyspnea in January 2009. Results of laboratory tests suggested type 1 diabetes mellitus with DKA. However, serum C-peptide level was normal on follow-up. Although he had no obvious features of acral enlargement, a high level of insulin-like growth factor 1 was detected, and a 75 g oral glucose suppression test showed no suppression of serum growth hormone levels. A pituitary macroadenoma was found on subsequent magnetic resonance imaging. The pituitary adenoma was surgically removed, followed by gamma-knife radiosurgery, and Sandostatin long-acting release treatment. He was then administered metformin, 500 mg twice daily, and to date, his serum glycohemoglobin has been <7%.
Tajima, Toshihiro; Tsubaki, Junko; Ishizu, Katsura; Jo, Wakako; Ishi, Nobuaki; Fujieda, Kenji
The use of octreotide-LAR and cabergoline therapy has shown great promise in adults with acromegaly; however, the experience in pediatric patients has rarely been reported. We described a clinical course of a 15-year-old boy of McCune-Albright syndrome (MAS) with pituitary gigantism. At the age of 8 years, a growth hormone (GH) and prolactin (PRL) producing pituitary adenoma was diagnosed at our hospital. He also had multiple fibrous dysplasia, so that he was diagnosed as having MAS. The tumor was partially resected, and GNAS1 gene mutation (R201C) was identified in affected tissues. We introduced octreotide to suppress GH secretion (100 mug 2/day s.c). During therapy with octreotide, IGF-1 and GH levels could not be suppressed and the patient frequently complained of nausea from octreotide treatment. Therefore, the therapy was changed to monthly injections of octreotide-LAR at the age of 12.3 years and was partially effective. However, as defect of left visual field worsened due to progressive left optic canal stenosis, he underwent second neurological decompression of the left optic nerve at 13.4 years of age. After surgery, in addition to octreotide-LAR, cabergoline (0.25 mg twice a month) was started. This regimen normalized serum levels of GH and IGF-1; however, he showed impaired glucose tolerance and gallstones at 15.7 years of age. Therefore, the dose of octreotide-LAR was reduced to 10 mg and the dose of cabergoline increased. This case demonstrated the difficulty of treating pituitary gigantism due to MAS. The use of octreotide-LAR and cabergoline should be considered even in pediatric patients; however, adverse events due to octreotide-LAR must be carefully examined.
Anniko, M; Ritzén, E M
True gigantism with overproduction of growth hormone (GH) and prolactin (PRL) was diagnosed in a 13-year-old boy. The clinical history indicated that the tumour had caused an oversecretion of GH since the age of 4-5 years. At diagnosis, the sella turcica was markedly enlarged. No infiltrative growth was noted at surgery. Endocrine investigations showed elevated GH and PRL secretion. Light and electron microscopy of tumour tissue revealed densely packed pleomorphic cells of both GH and PRL type. In addition, oncocyte-like cells were observed. Organ culture of pieces of tumour tissue demonstrated continued secretion of GH and PRL into the medium for more than 5 days in vitro. Addition of bromocriptine to the medium caused a rapid decline in PRL secretion while GH secretion remained the same. X-ray irradiation in vitro also caused a decrease in PRL secretion. These effects of bromocriptine and X-ray on hormone secretion in vitro mirrored the corresponding effect of treatment, when the patient showed signs of tumour recurrence after pituitary surgery. It is concluded that also in childhood, the in vitro response of tumour tissue to various treatments may be explored as a possible way to predict the efficacy of pharmacological or irradiation treatment of pituitary tumours.
Rix, M; Laurberg, P; Hoejberg, A S; Brock-Jacobsen, B
The use of a growth hormone (GH) receptor antagonist, pegvisomant has shown great promise in adults with acromegaly, but experience in paediatric patients is lacking. We aimed to describe the results of pegvisomant therapy in a 12-year-old girl with an aggressive GH-secreting pituitary tumour. To evaluate the ability of pegvisomant therapy to control the effects of peripheral GH excess in a case of pituitary gigantism. Pegvisomant was introduced at 10 mg/day, given subcutaneously, and gradually increased to 20 mg/day until serum IGF-I was normal for age. A large pituitary adenoma with suprasellar extension was diagnosed in a 12-year-old girl with progressive tall stature (178 cm), GH hypersecretion without suppression during oral glucose loading (nadir serum GH, 90 mU/l), high serum IGF-I and serum prolactin levels. Surgical extirpation was not possible because tumour tissue was fibrous and adherent to the optical nerves. Histological examination showed a mixed GH- and prolactin-secreting adenoma with lymphocytic infiltration of B and T cells. Treatment with a dopamine agonist, cabergoline, normalized serum prolactin, but GH secretion was resistant to both somatostatin analogue, octreotide and cabergoline. Radiation followed by pegvisomant therapy titrated up in dose to 20 mg/day led to a marked reduction in GH secretion and normalization of IGF-I, and to growth arrest and improvement of well-being. We suggest that treatment in pituitary gigantism with pegvisomant is safe and may normalize IGF-I levels and effectively stop growing.
Patt, Hiren P; Bothra, Nikita; Goel, Atul H; Kasaliwal, Rajeev; Lila, Anurag R; Bandgar, Tushar R; Shah, Nalini S
Limited data are available on pituitary gigantism, as it is a rare disorder. This study was carried out to assess the clinical, hormonal, and radiologic profiles and management outcomes of patients with pituitary gigantism. We conduced a retrospective analysis of 14 patients with pituitary gigantism who presented to a single tertiary care institute from 1990 to 2014. Thirteen patients were male, and 1 was female. The mean age at diagnosis was 21.9 ± 6.1 years, with a mean lag period of 6.5 ± 5.6 years. The mean height SD score at the time of diagnosis was 3.2 ± 0.6. Symptoms of tumor mass effect were the chief presenting complaint in the majority (50%) of patients, while 2 patients were asymptomatic. Six patients had hyperprolactinemia. At presentation, the nadir PGGH (postglucose GH) and insulin-like growth factor (IGF 1)-ULN (× upper limit of normal) were 63.2 ± 94.9 ng/mL and 1.98 ± 0.5, respectively. All (except 1 with mild pituitary hyperplasia) had pituitary macroadenoma. Six patients had invasive pituitary adenoma. Transsphenoidal surgery (TSS) was the primary modality of treatment in 13/14 patients, and it achieved remission in 4/13 (30.76%) patients without recurrence over a median follow-up of 7 years. Post-TSS radiotherapy (RT) achieved remission in 3/5 (60%) patients over a median follow-up of 3.5 years. None of the patients received medical management at any point of time. Gigantism is more common in males, and remission can be achieved in the majority of the patients with the help of multimodality treatment (TSS and RT).
Arisaka, O; Hall, R; Hughes, I A
An 11 year old, tall boy presented with symptoms typical of pituitary apoplexy. A large necrotic and haemorrhagic tumour was removed, which was shown to be an adenoma secreting growth hormone and prolactin. Subsequent treatment comprised cranial irradiation and hormone replacement. Eighteen months after operation growth was static and plasma growth hormone and prolactin concentrations were undetectable. Treatment of pituitary apoplexy should comprise excision of the tumour and postoperative i...
Full Text Available We present the first case study of idiopathic gigantic suprapubic lymphedema and buried penis treated with puboscrotal reconstruction in a patient with initial extreme obesity after an extensive weight reduction (120 kg. Massive localized lymphedema of the suprapubic region should be differentiated from the scrotal type. Severe lymphedema could not resolve on its own and weight reduction does not seem to be helpful in such cases.
Rostomyan, Liliya; Potorac, Iulia; Beckers, Pablo; Daly, Adrian F; Beckers, Albert
AIP mutations are rare in sporadic acromegaly but they are seen at a higher frequency among certain specific populations of pituitary adenoma patients (pituitary gigantism cases, familial isolated pituitary adenoma (FIPA) kindreds, and patients with macroadenomas who are diagnosed ≤30 years). AIP mutations are most prevalent in patients with pituitary gigantism (29% of this group were found to have mutations in AIP gene). These data support targeted genetic screening for AIP mutations/deletions in these groups of pituitary adenoma patients. Earlier diagnosis of AIP-related acromegaly-gigantism cases enables timely clinical evaluation and treatment, thereby improving outcomes in terms of excessive linear growth and acromegaly comorbidities. Copyright © 2017 Elsevier Masson SAS. All rights reserved.
Flitsch, J; Lüdecke, D K; Stahnke, N; Wiebel, J; Saeger, W
The management of pituitary macroadenomas which lead to gigantism may require multiple therapeutical approaches, including medical treatment, surgery, and radiation therapy. Transsphenoidal surgery (TSS) during early childhood that achieves total removal of a growth hormone (GH) secreting tumor is rarely reported. The surgeon is confronted with special problems regarding the infantile anatomy. In this case, a 3.5 year old child, the youngest successfully treated by TSS so far, suffered from a GH- and prolactin (PRL) secreting macroadenoma of the pituitary gland. The girl initially presented with an increasing growth rate, later with breast development, and finally, at the age of 2.8 years, with galactorrhea and secretion of blood from the nipples. Increased levels of GH [122 micrograms/l], insulin-like growth factor (IGF-1) [830 micrograms/l], insulin-like growth factor binding protein 3 (IGFBP-3) [8.6 mg/l] and PRL [590 micrograms/l] were found. MRI scans revealed a macroadenoma of 2.7 cm diameter. An eight-week trial of relatively low dose dopamine agonists led to a reduction of PRL, while the GH- and IGF-1 levels remained unchanged; the tumor showed only little shrinkage. Since there was chiasma compression, we opted for early TSS. A complete tumor removal was achieved despite the difficulties of a narrow approach. After TSS, low levels of GH, IGF-1, and PRL documented a complete tumor removal, but persistent diabetes insipidus and anterior lobe deficits resulted from surgery. In summary, if primary medical therapy alone is unable to adequately reduce hormone hypersecretion and tumor size in early childhood, TSS is recommended. Thus, radiation therapy may be reserved for surgical failure.
Tourtelot, John B; Vesely, David L
A 7'3" basketball player was noted to have 2 to 3 times thicker tissue in his hands than 6'10" players by an endocrinologist sitting 10 rows above the player in a basketball arena. This led to the diagnosis of pituitary gigantism where the history revealed that he was 7'3" at 15 years of age. At age 19 when the acryl enlargement was noted, a diagnostic workup revealed elevated growth hormones and insulin-like growth factor 1 (IGF-1) with a 2 × 1.3 cm pituitary tumor. His history suggested that his epiphyseal plates had closed at age 15, and because he continued to produce IGF-1, he now has acromegaly. His elevated adrenocorticotropic hormone (ACTH) before surgery suggests that he also had preclinical Cushing's disease. After pituitary transsphenoidal surgery, all acryl enlargement in hands and ligaments disappeared. His growth hormone, IGF-1 and ACTH returned to normal 2 weeks after surgery.
Shimatsu, Akira; Teramoto, Akira; Hizuka, Naomi; Kitai, Kazuo; Ramis, Joaquim; Chihara, Kazuo
The somatostatin analog lanreotide Autogel has proven to be efficacious for treating acromegaly in international studies and in clinical practices around the world. However, its efficacy in Japanese patients has not been extensively evaluated. We examined the dose-response relationship and long-term efficacy and safety in Japanese patients with acromegaly or pituitary gigantism. In an open-label, parallel-group, dose-response study, 32 patients (29 with acromegaly, 3 with pituitary gigantism) received 5 injections of 60, 90, or 120 mg of lanreotide Autogel over 24 weeks. Four weeks after the first injection, 41% of patients achieved serum GH level of gigantism) received lanreotide Autogel once every 4 weeks for a total of 13 injections. Dosing was initiated with 90 mg and adjusted according to clinical responses at Weeks 16 and/or 32. At Week 52, 47% of patients had serum GH levels of gigantism.
Christian Lyngsaa Lang
Full Text Available Lipomas are the most common benign mesenchymal tumour. The solitary subcutaneous lipoma accounts for approximately one-quarter to one-half of all soft tissue tumours. The preferred locations include the trunk, shoulder, upper arm, and the neck. In this case report, we present a gigantic axillary lipoma weighing 23.35 kg. Due to the tumour mass and its long-term presence, the lipoma caused anatomical changes in the axilla making its removal challenging and difficult.
Snarska, J.; Hady, H. R.; Kaminski, F.; Szajda, S. D.; Werel, D.; Zimnoch, L.; Siemiatkowski, A.
Retroperitoneal leiomyosarcomas are very rare malignant neoplasms. They are built up of smooth muscle cells. They can be found beyond the retroperitoneal space in the uterus, the stomach, the intestines, the blood vessel walls and the skin. This type of tumour grows slowly and gives no characteristic clinical symptoms. This results in late diagnosis, when they grow to a huge size. Retroperitoneal leiomyosarcomas are most frequently described in the literature in the fifth or sixth decade of life. There are no biochemical markers which could suggest the development of leiomyosarcoma. The basic diagnostic examination includes: ultrasound and computer tomography of the abdomen. The fundamental treatment of retroperitoneal leiomyosarcomas consists in aggressive surgical treatment - complete tumour resection. Large size of the tumour can make it difficult to perform radical surgery. In the case presented in this report, our team managed to remove a retroperitoneal leiomyosarcoma completely despite its huge mass and size (300 x 200 mm). Data from the literature indicate that a complete resection of the tumour is the most significant prognostic factor in the case of patients with retroperitoneal leiomyosarcoma. The efficacy of radiotherapy and chemotherapy has not been proved. The other unfavourable prognostic factor in the course of sarcomas is the age of the patient, below 50 years old. The patient presented in the report is a 31-year-old woman, which is associated with a worse prognosis. According to some authors, the size of the tumour is not significant for the fast recurrence of the disease, whereas others state that it is. The case was presented because of such a huge leiomyosarcoma being rarely found in the retroperitoneal space, the age of the patient and the asymptomatic course of the disease. (authors)
SA JOURNAL OF RADIOLOGY • December 2010. CASE REPORT. Acute pituitary apoplexy complicating a pituitary macroadenoma. Abstract. Pituitary apoplexy is a rare but potentially life-threatening condition caused by either haemorrhage or infarction of the pituitary gland. In most cases, a pre-existing pituitary ...
Full Text Available Introduction. Turner syndrome presents with one of the most frequent chromosomal aberrations in female, typically presented with growth retardation, ovarian insufficiency, facial dysmorphism, and numerous other somatic stigmata. Gigantism is an extremely rare condition resulting from an excessive growth hormone (GH secretion that occurs during childhood before the fusion of epiphyseal growth plates. The major clinical feature of gigantism is growth acceleration, although these patients also suffer from hypogonadism and soft tissue hypertrophy. Case report. We presented a girl with mosaic Turner syndrome, delayed puberty and normal linear growth for the sex and age, due to the simultaneous GH hypersecretion by pituitary tumor. In the presented case all the typical phenotypic stigmata related to Turner syndrome were missing. Due to excessive pituitary GH secretion during the period while the epiphyseal growth plates of the long bones are still open, characteristic stagnation in longitudinal growth has not been demonstrated. The patient presented with delayed puberty and primary amenorrhea along with a sudden appearance of clinical signs of hypersomatotropinism, which were the reasons for seeking medical help at the age of 16. Conclusion. Physical examination of children presenting with delayed puberty but without growth arrest must include an overall hormonal and genetic testing even in the cases when typical clinical presentations of genetic disorder are absent. To the best of our knowledge, this is the first reported case of simultaneous presence of Turner syndrome and gigantism in the literature.
Dragović, Tamara; Đuran, Zorana; Jelić, Svetlana; Marinković, Dejan; Kiković, Saša; Kuzmić-Janković, Snežana; Hajduković, Zoran
Turner syndrome presents with one of the most frequent chromosomal aberrations in female, typically presented with growth retardation, ovarian insufficiency, facial dysmorphism, and numerous other somatic stigmata. Gigantism is an extremely rare condition resulting from an excessive growth hormone (GH) secretion that occurs during childhood before the fusion of epiphyseal growth plates. The major clinical feature of gigantism is growth acceleration, although these patients also suffer from hypogonadism and soft tissue hypertrophy. We presented a girl with mosaic Turner syndrome, delayed puberty and normal linear growth for the sex and age, due to the simultaneous GH hypersecretion by pituitary tumor. In the presented case all the typical phenotypic stigmata related to Turner syndrome were missing. Due to excessive pituitary GH secretion during the period while the epiphyseal growth plates of the long bones are still open, characteristic stagnation in longitudinal growth has not been demonstrated. The patient presented with delayed puberty and primary amenorrhea along with a sudden appearance of clinical signs of hypersomatotropinism, which were the reasons for seeking medical help at the age of 16. Physical examination of children presenting with delayed puberty but without growth arrest must include an overall hormonal and genetic testing even in the cases when typical clinical presentations of genetic disorder are absent. To the best of our knowledge, this is the first reported case of simultaneous presence of Turner syndrome and gigantism in the literature.
Hannah-Shmouni, Fady; Trivellin, Giampaolo; Stratakis, Constantine A.
Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing unders...
de Herder, Wouter W
In 1886 Pierre Marie used the term "acromegaly" for the first time and gave a full description of the characteristic clinical picture. However several others had already given clear clinical descriptions before him and sometimes had given the disease other names. After 1886, it gradually became clear that pituitary enlargement (caused by a pituitary adenoma) was the cause and not the consequence of acromegaly, as initially thought. Pituitary adenomas could be found in the great majority of cases. It also became clear that acromegaly and gigantism were the same disease but occurring at different stages of life and not different diseases as initially thought. At the end of the 19th and beginning of the 20th century most information was derived from case descriptions and post-mortem examinations of patients with acromegaly or (famous) patients with gigantism. The stage was set for further research into the pathogenesis, diagnosis and therapy of acromegaly and gigantism.
Maes, B; Caron, J; Couchot, J; Gross, A; Leutenegger, M
The authors report a case of cerebral gigantism and review 83 cases found in the world literature. The diagnosis depends on the association of several factors, none of which alone are specific but which, on the whole, form a fairly characteristic picture. Large size at birth, leading only rarely to gigantism at adult age. Facial dysmorphia of acromegaloid type. Ecephalopathy with mental deficiency without any neuroradiological abnormality. Finally, endocrine investigations and somatotropic function were normal. No etiology has been found up to now. The most satisfactory theory is that of a hypothalamopituitary disturbance. Treatment may simple avoid excess height at adult age and is based on sex hormone therapy to accelerate bony maturation.
Hanel Ricardo Alexandre
Full Text Available Pituitary abscesses are potentially life-threatening lesions if not appropriately diagnosed and treated. The authors have operated on more than five hundred cases of pituitary tumors and only one represented a case of pituitary abscess. A 35-year-old woman was investigated for chronic frontal headache. CT scan showed a cystic sellar lesion with ring enhancement after contrast injection leading to an initial diagnosis of pituitary adenoma. She underwent a sublabial transsphenoidal approach to the pituitary gland. After dural opening, purulent material was obtained and no tumor or other associated lesion was detected. There was no evidence of current or previous septicemic illness, meningitis, cavernous sinus thrombosis or sinus infection. Cultures were negative. She was put on antibiotics and discharged after 4 weeks. Nowadays, 10 years after treatment, she is doing well, with no anterior pituitary hormone deficit. MRI shows a partially empty sella without residual lesion and the pituitary stalck is in the midline. The early diagnosis and adequate treatment of this life-threatening lesion may result in excellent prognosis.
Schoof, Ellen; Dörr, Helmuth G; Kiess, Wieland; Lüdecke, Dieter K; Freitag, Eduard; Zindel, Volker; Rascher, Wolfgang; Dötsch, Jörg
In children, there is little experience with octreotide therapy for pituitary tumors, especially growth hormone (GH) producing adenomas. We report on a 13-year-old boy with gigantism due to a GH-producing pituitary adenoma caused by a Gsalpha mutation on the basis of McCune-Albright syndrome. At the age of 6.5 years a GH- and prolactin-producing pituitary adenoma was diagnosed. The adenoma was surgically removed. Immediately thereafter, the small adenoma residuum was treated with octreotide (2 x 100 microg/day s.c.). During therapy with octreotide, the growth rate dropped to normal values; however, rose again after 2 years of treatment. The insulin-like growth factor I (IGF-I) levels remained above the 95th percentile, the GH level mostly >2 microg/l. After 5 years of octreotide therapy, GH (6.9 microg/l), IGF-I (620 microg/l), IGF-binding protein 3 (5.4 mg/l), and prolactin (17.0 ng/ml) levels were still elevated. The growth velocity was +2.4 SDS (standard deviation score), the pubertal status was mature, and the bone age was 14.3 years (prospective final height 208 cm). A magnetic resonance imaging scan showed an unchanged residual 4-mm rim of adenoma at the pituitary site. Side effects from octreotide therapy were not reported by the patient or his family. The therapy was changed to the long-acting release octreotide analog octreotide-LAR. After 1 year of treatment with octreotide-LAR, the GH level was 1.0 microg/l, and the prospective final height dropped by 10 cm. This case demonstrates that combined surgical and medical treatment can influence the prognosis of childhood gigantism; however, the prognosis of this rare condition remains uncertain. Copyright 2004 S. Karger AG, Basel
Hannah-Shmouni, Fady; Trivellin, Giampaolo; Stratakis, Constantine A.
Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing understanding of the molecular and genetic etiologies of pituitary gigantism and acromegaly yielded several genetic causes, including multiple endocrine neoplasia type 1 and 4, McCune-Albright syndrome, Carney complex, familial isolated pituitary adenoma, pituitary adenoma association due to defects in familial succinate dehydrogenase genes, and the recently identified X-linked acrogigantism. The early diagnosis of these conditions helps guide early intervention, screening, and genetic counseling of patients and their family members. In this review, we provide a concise and up-to-date discussion on the genetics of gigantism and acromegaly. PMID:27657986
Hannah-Shmouni, Fady; Trivellin, Giampaolo; Stratakis, Constantine A
Gigantism and acromegaly are rare disorders that are caused by excessive GH secretion and/or high levels of its mediator, IGF-1. Gigantism occurs when excess GH or IGF-1 lead to increased linear growth, before the end of puberty and epiphyseal closure. The majority of cases arise from a benign GH-secreting pituitary adenoma, with an incidence of pituitary gigantism and acromegaly of approximately 8 and 11 per million person-years, respectively. Over the past two decades, our increasing understanding of the molecular and genetic etiologies of pituitary gigantism and acromegaly yielded several genetic causes, including multiple endocrine neoplasia type 1 and 4, McCune-Albright syndrome, Carney complex, familial isolated pituitary adenoma, pituitary adenoma association due to defects in familial succinate dehydrogenase genes, and the recently identified X-linked acrogigantism. The early diagnosis of these conditions helps guide early intervention, screening, and genetic counseling of patients and their family members. In this review, we provide a concise and up-to-date discussion on the genetics of gigantism and acromegaly. Published by Elsevier Ltd.
Bondanelli, Marta; Bonadonna, Stefania; Ambrosio, Maria Rosaria; Doga, Mauro; Gola, Monica; Onofri, Alessandro; Zatelli, Maria Chiara; Giustina, Andrea; degli Uberti, Ettore C
Chronic growth hormone (GH)/insulin-like growth factor I (IGF-I) excess is associated with considerable mortality in acromegaly, but no data are available in pituitary gigantism. The aim of the study was to evaluate the long-term effects of early exposure to GH and IGF-I excess on cardiovascular and metabolic parameters in adult patients with pituitary gigantism. Six adult male patients with newly diagnosed gigantism due to GH secreting pituitary adenoma were studied and compared with 6 age- and sex-matched patients with acromegaly and 10 healthy subjects. Morphologic and functional cardiac parameters were evaluated by Doppler echocardiography. Glucose metabolism was assessed by evaluating glucose tolerance and homeostasis model assessment index. Disease duration was significantly longer (Pgigantism than in patients with acromegaly, whereas GH and IGF-I concentrations were comparable. Left ventricular mass was increased both in patients with gigantism and in patients with acromegaly, as compared with controls. Left ventricular hypertrophy was detected in 2 of 6 of both patients with gigantism and patients with acromegaly, and isolated intraventricular septum thickening in 1 patient with gigantism. Inadequate diastolic filling (ratio between early and late transmitral flow velocitygigantism and 1 of 6 patients with acromegaly. Impaired glucose metabolism occurrence was higher in patients with acromegaly (66%) compared with patients with gigantism (16%). Concentrations of IGF-I were significantly (Pgigantism who have cardiac abnormalities than in those without cardiac abnormalities. In conclusion, our data suggest that GH/IGF-I excess in young adult patients is associated with morphologic and functional cardiac abnormalities that are similar in patients with gigantism and in patients with acromegaly, whereas occurrence of impaired glucose metabolism appears to be higher in patients with acromegaly, although patients with gigantism are exposed to GH excess for a
Tahara, Shigeyuki; Murakami, Mami; Kaneko, Tomomi; Shimatsu, Akira
A multicenter, open-label, phase 2 study was conducted to investigate the efficacy and safety of long-acting pasireotide formulation in Japanese patients with acromegaly or pituitary gigantism. Medically naïve or inadequately controlled patients (on somatostatin analogues or dopamine agonists) were included. Primary end point was the proportion of all patients who achieved biochemical control (mean growth hormone [GH] levelsacromegaly, n=32; pituitary gigantism, n=1) were enrolled and randomized 1:1:1 to receive open-label pasireotide 20mg, 40mg, or 60mg. The median age was 52 years (range, 31-79) and 20 patients were males. At month 3, 18.2% of patients (6/33; 90% confidence interval: 8.2%, 32.8%) had biochemical control (21.2% [7/33] when including a patient with mean GHacromegaly or pituitary gigantism.
Cha, Ji Hyeon; Kim, Hak Hee; Kim, Sun Mi; Seo, Myung Hee; Yoon, Hoi Soo
D-penicillamine, a chelating agent of copper, is the drug of choice for the treatment of Wilson's disease. Breast enlargement is a rare complication arising from its use, and we report a case of breast gigantism which developed after it had been used for ten months to treat this condition. Mammography demonstrated bilaterally enlarged dense breasts; ultrasonography, similarly, demonstrated enlargement, revealing the presence of a mass, shown at biopsy to be benign, in the left one
Cha, Ji Hyeon; Kim, Hak Hee; Kim, Sun Mi; Seo, Myung Hee; Yoon, Hoi Soo [College of Medicine, Ulsan Univ., Seoul (Korea, Republic of)
D-penicillamine, a chelating agent of copper, is the drug of choice for the treatment of Wilson's disease. Breast enlargement is a rare complication arising from its use, and we report a case of breast gigantism which developed after it had been used for ten months to treat this condition. Mammography demonstrated bilaterally enlarged dense breasts; ultrasonography, similarly, demonstrated enlargement, revealing the presence of a mass, shown at biopsy to be benign, in the left one.
Here we commemorate the character and academic authority of Prof. Zabiholah Gorban (1903-2006), the founder of Shiraz medical school. No doubt, in the scope of history of contemporary medicine, he has been efficient and effective. With respect to this fact, his article on a rare case described in Acta anatomica published in Iran in 1966, entitled (Observations on a giant skeleton) is browsed and reviewed. A case named Siah Khan with combined acromegaly and gigantism that appears to have letters to say still after nearly half a century.
Figueiredo, Rossana Lopes de; Nobrega, Rosangela Figueiredo; Toscano, Carlos Alberto Regis
The authors report a case of primary retroperitoneal teratoma which has grown for seven years. the diagnosis was presumed through image diagnostic methods and it was confirmed after surgery and histopathology analysis. (author). 7 refs., 6 figs
Turra, S; Santini, S; Cagnoni, G; Jacopetti, T
We report our experience in seven patients with congenital gigantism of the foot with the following diagnoses: neurofibromatosis (two), fibrolipomatosis (two), Proteus syndrome (two), and idiopathic localized gigantism (one). Our purpose is to introduce a new classification of foot gigantism, based on the concept of "neuroinduction." In our experience, intraoperative examination and subsequent histologic examination show consistently pathologic findings in the plantar nerve and its terminal branches in the foot affected by gigantism. Limited surgical treatment was used in five patients. To prevent forefoot enlargement and recurrence of deformity, we suggest complete ray resection. We evaluated our results using radiographs, functional status, and cosmetic considerations.
Felix, I A; Horvath, E; Kovacs, K; Smyth, H S; Killinger, D W; Vale, J
A 29-year old giantess with growth hormone excess and hyperprolactinemia underwent transsphenoidal surgery to remove her pituitary tumor. Electron microscopy revealed a mammosomatotroph adenoma composed of one cell type. Immunoelectron microscopy, using the immunogold technique, demonstrated predominantly growth hormone or prolactin or a varying mixture of both growth hormone and prolactin in the adenoma cells. The presence of growth hormone and prolactin was found not only in the cytoplasm of the same adenoma cells but also in the same secretory granules. In the nontumorous adenohypophysis, somatotrophs and lactotrophs showed ultrastructural signs of hyperactivity. This finding is in contrast with the presence of suppressed somatotrophs and lactotrophs seen in nontumorous portions of adult pituitaries harboring growth hormone or prolactin-secreting adenomas. Our morphological study reinforces the view that growth hormone-producing pituitary tumors, originating in childhood, are different from those of the adult gland.
A collection of 893 historical picture postcards from 1900 to 1935, depicting dwarfs and giants, was analysed from medical and psychosocial viewpoints. In conditions such as 'bird headed dwarfism', achondroplasia, cretinism, so-called Aztecs or pinheads, Grebe chondrodysplasia, and acromegalic gigantism, the disorder could be diagnosed easily. In hypopituitary dwarfism, exact diagnosis was more difficult because of heterogeneity. The most common conditions depicted were pituitary dwarfism and achondroplasia. Most of those with gigantism had pituitary gigantism and acromegaly. Brothers and sisters or parents and their children provided evidence of mendelian inheritance of some of these disorders. The cards suggest that being put on show provided, at least in some cases, social benefits. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 PMID:9764085
Santos, Alair Augusto S.M.D. dos; Fontes, Cristina Asvolinsque P.; Magnago, Marcelo; Moreira, Denise Madeira; Andreiuolo, Pedro Angelo; Oliveira, Fernando Barros de; Teixeira, Ricardo Tostes D.; Correa, Saul Orlando C.
We reviewed 60 cases of patients with pituitary macroadenomas who were submitted to magnetic resonance imaging evaluations at private institutions in Niteroi and Rio de Janeiro, Brazil. The aim of this study was to demonstrate the advantages of magnetic resonance imaging in the diagnosis and follow-up of these patients. Macroprolactinomas were the most prevalent tumors. We also observed four growth-hormone (GH) secreting macroadenomas with clinical signs of acromegaly, and one GH- and prolactin-secreting macroadenoma. Seven patients presented pituitary apoplexy and hyperintense signal on T1-W images before contrast medium (gadolinium) administration suggestive of intratumoral bleeding. The follow-up of 15 cases demonstrated a reduction in the size of the tumor after surgery, clinical treatment or radiotherapy. Magnetic resonance imaging is important for the study of pituitary macroadenomas, particularly before surgery, as it shows the involvement of adjacent structures, specially the cavernous sinus, optic chiasm and pituitary stalk compression. (author)
Melo Débora Gusmão
Full Text Available Sotos syndrome or cerebral gigantism is characterized by macrocephaly, overgrowth, mental retardation and central nervous system abnormalities. Congenital heart defects may be present. We report 8 patients with this syndrome and relate their clinical features, neuroimaging and echocardiographic findings.
Full Text Available Only 4-9% of patients with Cushing’s disease (CD harbor pituitary macroadenomas. Clinical and biochemical features of macrocorticotropinomas are poorly understood. Some evidence exist that these tumors presents clinical features more similar to a non-functioning adenomas, being though defined silent corticotropinomas, rather than to ACTH-secreting adenomas. In this paper, we report a case of a 60-year old woman with a history of obesity, arterial hypertension and diabetes mellitus who presented with overt central hypothyroidism. Magnetic resonance imaging disclosed giant pituitary adenoma measuring 50 mm. Endocrinological evaluation confirmed CD: ACTH 50.3 pmol/L, urinary free-cortisol of 739 nmol/24h and cortisol of 639 nmol/L after 1 mg dexamethasone suppression test. Tumor mass was reduced by 50% using purely endoscopic transsphenoidal approach. Thirty-eight months after the partial resection, the patient had well controlled CD: ACTH 20.2 pmol/L, urinary free-cortisol of 238 nmol/24h, cortisol of 105 nmol/L after 1 mg dexamethasone suppression test. To the best of our knowledge, this is the largest ACTH-secreting adenoma ever reported. Our case suggests that tumor size does not necessarily correlate with aggressiveness of CD in patients with macrocorticotropinomas and that long-term control of CD may be achieved albeit incomplete surgical removal. Further studies are needed in order to determine the best treatment option for patients with macrocorticotropinomas.
Figueiredo, Rossana Lopes de [Paraiba Univ., Campina Grande, PB (Brazil). Faculdade de Medicina; Franca Costa, Hamilton Belo de [Hospital Geral de Campina Grande, PB (Brazil); [Clinica Pronto-Socorro Infantil, Campina Grande, PB (Brazil); Nobrega, Rosangela Figueiredo [Clinica Inside, Campina Grande, PB (Brazil); Toscano, Carlos Alberto Regis [Hospital Pedro I, Campina Grande, PB (Brazil)
The authors report a case of primary retroperitoneal teratoma which has grown for seven years. the diagnosis was presumed through image diagnostic methods and it was confirmed after surgery and histopathology analysis. (author). 7 refs., 6 figs.
Rekha, Arcot; Gopalan, T R
Gigantism of the lower limb can occur because of plexiform neurofibromas. This condition is seen with café au lait patches and multiple neurofibromatosis in this case of von Recklinghausen neurofibromatosis. We report our patient and review literature of this uncommon condition.
Lee, Tae Hee; Lee, Seoung Ro; Park, Dong Woo; Hahm, Chang Kok; Park, Choong Ki; Kim, Yong Su; Paik, Seung Sam; Hong, Eun Kyung [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)
A 34-year-old man who for two months had experienced headaches and blurred vision showed a 6 x 5 x 4 cm-sized sellar and suprasellar mass with mainly cystic and partially solid portions on both brain CT and MRI. Peripheral, rim-like calcification of the cystic mass was clearly visible on non-enhanced CT and pituitary acidophil stem cell adenoma was confirmed. This is an immature neoplasm which is shown by immunohistochemical study and electromicrocopy to be derived from the common progenitor of growth hormone and prolactin cells. We recently encountered one case, involving unusual radiographic findings, in which pituitary macroadenoma mimicked craniopharyngioma, and we report the CT and MR findings of this tumor.
Kim, Da Mi; Yu, In Kyu [Dept. of Radiology, Eulji University Hospital, Daejeon (Korea, Republic of); Kim, Han Kyu [Dept. of Neurosurgery, Eulji University Hospital, Daejeon (Korea, Republic of); Son Hyun Jin [Dept. of Pathology, Eulji University Hospital, Daejeon (Korea, Republic of)
A 38-year-old man was admitted to our hospital with diplopia. The patient had a relatively well-defined pituitary mass with high cellularity as well as weaker enhancement on imaging modalities including computed tomography (CT) and magnetic resonance imaging (MRI), than a typical pituitary adenoma. The distinction between a pseudotumor and an invasive neoplasm is very difficult before biopsy. In this case report, we discuss the characteristic imaging features of a fibrosing inflammatory pseudotumor of the pituitary gland
Chentli, Farida; Azzoug, Said; Amani, Mohammed El Amine; Haddam, Ali El Mahdi; Chaouki, Dalal; Meskine, Djamila; Chaouki, Mohamed Lamine
True gigantism is an exceptional and fascinating pediatric disease. Our aim in this study was to describe the different etiologies of a large group of children with gigantism and the natural history of their growth. In this multicenter study, we considered as giant children, adolescents and adults whose heights were ≥3 SD compared to their target stature or to our population average lengths. Isolated hypogonadism and Klinefelter syndrome were excluded from this series. All underwent clinical exam, and hormonal and neurological investigations. From 1980 to 2010, we observed 30 giants: 26 males (86.6%) and 4 females (mean age 19.8 ± 11 years). Among the 13 patients (40.3%) who consulted before the age of 16 years, 9 had acromegaly and 6 had mental retardation and body malformations. Based on growth hormone (GH) secretion evaluation, 2 groups were observed: pituitary gigantism (n = 16): GH = 150 ± 252 ng/ml (n ≤ 5), and other causes with normal GH (0.7 ± 0.6 ng/ml): 6 Sotos syndrome and 8 idiopathic cases. Only the first group had neurological, ophthalmological, metabolic and cardiovascular complications and received treatment. The result was not optimal as GH normalization was not observed. Reduction of tumor size and decreased GH plasma values were not observed. Gigantism predominates in males. The main cause is GH excess. The diagnosis was very late except for cerebral gigantism. Complications were observed in pituitary gigantism only. Copyright © 2012 S. Karger AG, Basel.
Wang, Hai-hua; Tian, Guang-lei; Zhu, Yin; Zhang, You-le; Zhao, Jun-hui; Tian, Wen
To summarize the clinical characteristic and outcome of digital gigantism of the foot. Retrospectively analyze the clinical documents of cases of digital gigantism of the foot. Twelve 12 cases with 13 feet in this study included 8 male and 4 female with an average 4.6-years-old. All the deformities were found at birth. Multiple toes involved were more than single toe, and tibial toe involved more than fibular. Forefoot was enlarged. All the phalanges involved and partial metatarsal bones were enlarged. Marked increase in subcutaneous fat was found in all cases in the operation which infiltrated interossei and articular capsules. The appearance of the nerves and its branches in the foot were normal and fat infiltrating was not discovered. The operation types included debulking, epiphyseal arrest, amputation, nerve stripping and anastomosis. Seven cases were followed up with mean periods 25.6 months. Functional evaluation according to a criterion formulated by author revealed a result of 2 excellent, 2 good and 3 fair. Digital gigantism of the foot is an uncommon congenital deformity of the foot characterized by overgrowth of both the soft-tissue and the osseous elements of the enlarged toe and forefoot. Surgical treatment is the unique method, and the goal is to reduce the size of the foot to allow fitting regular shoes and walking readily. There are several types of operations which to be chosen. The indication, the timing of operative intervention and the selection of operation type should be paid more attention.
Apostolos K A Karagiannis
Full Text Available Pituitary abscess is a rare life-threating entity that is usually misdiagnosed as a pituitary tumor with a definite diagnosis only made postoperatively. Over the last several decades, advances in healthcare have led to a significant decrease in morbidity and mortality due to pituitary abscess. We report a case of a 34-year-old woman who was admitted to our department for investigation of a pituitary mass and with symptoms of pituitary dysfunction, headaches and impaired vision. During her admission, she developed meningitis-like symptoms and was treated with antibiotics. She eventually underwent transsphenoidal surgery for excision of the pituitary mass. A significant amount of pus was evident intraoperatively; however, no pathogen was isolated. Six months later, the patient was well and had full recovery of the anterior pituitary function. Her menses returned, and she was only on treatment with desmopressin for diabetes insipidus that developed postoperatively.
Barth, P.G.; Vlasveld, L.; Valk, J.
A case of Sotos' syndrome (cerebral gigantism) is described. Pneumencephalography, performed at the age of 15 days, revealed abnormal separation of the opercula on the right. By comparing the contours with developmental anatomical features of this area it agreed with a foetal development of 24 weeks gestational age. Bilateral carotid angiography was normal. CT showed normal development of the Sylvian area at the age of 27 months.
Barth, P.G.; Vlasveld, L.; Valk, J.
A case of Sotos' syndrome (cerebral gigantism) is described. Pneumencephalography, performed at the age of 15 days, revealed abnormal separation of the opercula on the right. By comparing the contours with developmental anatomical features of this area it agreed with a foetal development of 24 weeks gestational age. Bilateral carotid angiography was normal. CT showed normal development of the Sylvian area at the age of 27 months. (orig.)
Uesaka, T.; Miyazono, M.; Nishio, S.; Iwaki, T.
A 34-year-old man presented with a 4-month history of visual obscuration. Magnetic resonance imaging showed a solid, discrete, contrast-enhancing pituitary mass with suprasellar extension. Surgery, which was performed via a transsphenoidal approach, disclosed the pituitary tumor to be a fibrillary astrocytoma (pituicytoma). This case report contains the clinical and neuroimaging features of this rare tumor of the neurohypophysis, which masqueraded as a pituitary adenoma. (orig.)
Full Text Available Pituitary abscess is a relatively uncommon cause of pituitary hormone deficiencies and/or a suprasellar mass. Risk factors for pituitary abscess include prior surgery, irradiation and/or pathology of the suprasellar region as well as underlying infections. We present the case of a 22-year-old female presenting with a spontaneous pituitary abscess in the absence of risk factors described previously. Her initial presentation included headache, bitemporal hemianopia, polyuria, polydipsia and amenorrhoea. Magnetic resonance imaging (MRI of her pituitary showed a suprasellar mass. As the patient did not have any risk factors for pituitary abscess or symptoms of infection, the diagnosis was not suspected preoperatively. She underwent transsphenoidal resection and purulent material was seen intraoperatively. Culture of the surgical specimen showed two species of alpha hemolytic Streptococcus, Staphylococcus capitis and Prevotella melaninogenica. Urine and blood cultures, dental radiographs and transthoracic echocardiogram failed to show any source of infection that could have caused the pituitary abscess. The patient was treated with 6 weeks of oral metronidazole and intravenous vancomycin. After 6 weeks of transsphenoidal resection and just after completion of antibiotic therapy, her headache and bitemporal hemianopsia resolved. However, nocturia and polydipsia from central diabetes insipidus and amenorrhoea from hypogonadotrophic hypogonadism persisted.
Koo, Hee Youn; Lee, Myung Jun; Lee, Chang Joon; Yoo, Jeong Hyun
Colloid cyst is a congenital lesion which is thought to be derived from the primitive neuro epithelium, and is most frequently located in the anterior half of the third ventricle. Colloid cysts rarely occur in the pituitary gland, and we describe a case of pituitary colloid cyst, including the CT, MRI and pathologic findings
Remagen, W.; Nidecker, A.; Prein, J.
In summary, a fascinating case is presented of an enormous 'blow-out' lesion in the left half of the mandible in a 17-year-old boy. The histological diagnosis was benign fibrous histiocytoma or non-ossifying fibroma. An extensive differential diagnosis was presented by the authors and although benign fibrous histiocytoma was their final diagnosis, they could not exclude an example of the rarely encountered entity called the Jaffe-Campanacci syndrome. This syndrome consists of multiple non-ossifying fibromas of the mandible, cafe-au-lait spots, various endocrine disorders, mental retardation, occular anomalies and cardiovascular malformations. (orig./SHA)
Full Text Available A 60-year-old male with a huge prostate cyst presented with obstruction symptom of urethra and intestinal tract. Complete excision of the cystic prostate failed as a result of the strong adherence and twice operations history, but we confirmed prostate adenocarcinoma and relieved his obstruction symptom. Case 2 was a 77-year-old male with an 8 cm cyst of which biopsy showed prostate cancer in local hospital. He was admitted 18 months later because of intestinal obstruction. Radical resection had a satisfied result of obstruction symptom and PSA. Here we summarized malignant characteristics of cystic lesions in prostate or surrounding structures and management.
Full Text Available Abstract Introduction Pituitary adenomas represent the most frequently observed type of sellar masses; however, the presence of a rapidly growing sellar tumor, diabetes insipidus, ophthalmoplegia and headaches in an older patient strongly suggests metastasis to the pituitary. Since the anterior pituitary has a great reserve capacity, metastasis to the pituitary and pituitary involvement in lymphoma are usually asymptomatic. Whereas diabetes insipidus is the most frequent symptom, patients can present with headaches, ophthalmoplegia and bilateral hemianopsia. Case presentation A 70-year-old woman with no previous history of malignancy presented with headaches, right oculomotor nerve palsy and diabetes insipidus. As magnetic resonance imaging revealed a sellar mass involving the pituitary gland and infundibular stalk, which also extended into the right cavernous sinus and sphenoid sinus, the patient underwent an immediate transsphenoidal decompression surgery. Her prolactin was 102.4 ng/ml, whereas her gonadotropic hormone levels were low. A low level of urine osmolality after overnight water deprivation, along with normal plasma osmolality suggested diabetes insipidus. Histological examination revealed that the mass had been the infiltration of a high grade B-cell non-Hodgkin's lymphoma involving respiratory system epithelial cells. Paranasal sinus computed tomography scanning and magnetic resonance imaging of the thorax and abdomen were performed. Since magnetic resonance imaging did not reveal any abnormality, after paranasal sinus computed tomography was performed, we concluded that the primary lymphoma originated from the sphenoid sinus and infiltrated the pituitary. Chemotherapy and radiotherapy to the sellar area were planned, but the patient died and her family did not permit an autopsy. Conclusion Lymphoma infiltration to the pituitary is difficult to differentiate from pituitary adenoma, meningioma and other sellar lesions. To plan the
Özçetin, Mustafa; Karacı, Mehmet; Toroslu, Ertuğ; Edebali, Nurullah
Pituitary adenomas usually arise from the anterior lobe of the pituitary gland and are manifested with hormonal disorders or mass effect. Mass effect usually occurs in nonfunctional tumors. Pituitary adenomas may be manifested with visual field defects or rarely in the form of total oculomotor palsy. Visual field defect is most frequently in the form of bitemporal hemianopsia and superior temporal defect. Sudden loss of vision, papilledema and ophthalmoplegia may be observed. Pituitary apople...
Full Text Available Ectopic neurohypophysis is an anomaly of the Pituitary gland whichmay be associated with short stature due to Growth hormone deficiency.MRI is the modality of choice in diagnosing this condition. We present acase of pituitary dwarfism and ectopic neurohypophysis with clinical andradiological findings. 21 year-old male admitted with short stature. Allhormones, except prolactin, of anterior hypophysis were low. Bright spotwas ectopically located at level of median eminence on enhanced MRI ofhypophysis and stalk of hypophysis was not observed. Ectopicneurohypophysis may be present with pituitary dwarfism. Cranial MRI maybe useful to investigate related pathologies in such cases.
Dragović Tamara; Đuran Zorana; Jelić Svetlana; Marinković Dejan; Kiković Saša; Kuzmić-Janković Snežana; Hajduković Zoran
Introduction. Turner syndrome presents with one of the most frequent chromosomal aberrations in female, typically presented with growth retardation, ovarian insufficiency, facial dysmorphism, and numerous other somatic stigmata. Gigantism is an extremely rare condition resulting from an excessive growth hormone (GH) secretion that occurs during childhood before the fusion of epiphyseal growth plates. The major clinical feature of gigantism is growth acceler...
Nishio, S; Morioka, T; Suzuki, S; Takeshita, I; Fukui, M; Iwaki, T
The clinicopathologic features of seven paediatric patients with pituitary adenomas (2 male, 5 female; mean age 14.3 years) were reviewed. There were three non-functioning adenomas, three prolactinomas, and one growth hormone producing adenoma. Five patients presented with visual field deficits, and six patients had endocrine symptoms, which included menstrual irregularities in all female patients, pubertal delay in two females, and growth delay and gigantism in one case each. On neuroimaging studies, five adenomas showed parasellar extension, while the remaining two prolactinomas were intrasellar microadenomas. While two patients with prolactinomas received good results with bromocriptine treatment alone, the remaining five patients underwent either craniotomy or transsphenoidal surgery. Postoperatively, visual disturbances improved markedly in all patients. Two patients also received replacement hormonal therapy. While six patients have been stable for 3.6 years on average, one non-functioning tumour recurred 2 years after the initial transcranial subtotal resection of the tumour. Although there are still many unknowns concerning the biology and optimal treatments for paediatric pituitary adenomas, many of them are assumed to be relatively rapidly growing tumours, while others merely have an earlier tumour genesis than in adults. Copyright 2001 Harcourt Publishers Ltd.
... children or adolescents can lead to increased height (gigantism), because the long bones of their arms and ... Information & Resources MedlinePlus (6 links) Encyclopedia: Acromegaly Encyclopedia: Gigantism Encyclopedia: Pituitary Tumor Encyclopedia: Prolactinoma Health Topic: Endocrine ...
Tiago Leal Ghezzi
Full Text Available Cordoma sacrococcígeo é uma neoplasia maligna rara que se origina de remanescentes da notocorda. A localização crítica, comportamento localmente agressivo, reconhecida resistência à radioterapia, significativa morbimortalidade cirúrgica e elevada taxa de recidiva tornam seu tratamento um desafio. Descrevemos um caso de cordoma sacrococcígeo gigante.Sacrococcygeal chordoma is a rare malignant neoplasm arised from the remmants of the notochord. The critical localization, locally aggressive behavior, well-known resistance to radiation therapy, meaningful surgical morbimortality and increased recurrence rate become its treatment a challenge. We describe a case of a giant unresectable sacrococcygeal chordoma.
Fukuta, Kaori; Hidaka, Takao; Ono, Yosuke; Kochi, Keiko; Yasoshima, Kuniaki; Arai, Takashi
Pituitary stalk transection syndrome (PSTS) is a rare complication that can accompany breech delivery. Early diagnosis of this syndrome is difficult, and it may cause a serious delay in the diagnosis. We present a case of PSTS ascertained after breech delivery. A 20-year-old woman presented with primary amenorrhea. The patient was born by breech delivery and had a history of treatment for pituitary dwarfism. Her laboratory findings showed pituitary hypothyroidism, and hormone replacement therapy was initiated. At 28 years old, she became pregnant and had a normal delivery at 38 weeks' gestation. One year after delivery, her thyroid hormone level changed. Laboratory test showed adrenocortical insufficiency, and magnetic resonance imaging of the pituitary gland showed transection of the pituitary stalk and development of an ectopic posterior lobe. These findings were compatible with PSTS. When a patient who has been born by breech delivery presents with symptoms of pituitary deficiency, PSTS should be considered in the differential diagnosis. © 2015 Japan Society of Obstetrics and Gynecology.
Sakayama, Kenshi; Sugawara, Yoshifumi; Kidani, Teruki; Fujibuchi, Taketsugu; Kito, Katsumi; Tanji, Nozomu; Nakamura, Atsushi
We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case.
Gołkowski, Filip; Buziak-Bereza, Monika; Stefańska, Agnieszka; Trofimiuk, Małgorzata; Pantofliński, Jacek; Huszno, Bohdan; Czepko, Ryszard; Adamek, Dariusz
A case of GH and TSH secreting pituitary macroadenoma is reported. A 45-year-old female presented clinical features of acromegaly (the abnormal growth of the hands and feet, with lower jaw protrusion), diabetes mellitus, hypertension, nodular goiter and hyperthyroidism of unclear origin. NMR pituitary imaging revealed intra and extrasellar tumor. The laboratory examinations showed very high plasma levels of GH and IGF-1 and normal level of TSH coexisting with high plasma levels of free thyroid hormones. Pharmacological pretreatment with somatostatin analogues caused the substantial reduction of GH and TSH plasma levels. Histological and immunohistochemical examination of the tissue obtained at transsphenoidal surgery showed GH and TSH secreting adenoma. The laboratory examinations after surgery showed normal GH and IGF-1 plasma levels and reduced insulin requirement, what indicates radical operation. The very low plasma levels of TSH and free thyroid hormones after surgery and immunohistochemical examination suggest central hyperthyroidism due to TSH secreting pituitary tumor (thyrotropinoma).
Cha, Seung Woo; Park, Dong Woo; Park, Choong Ki; Pyo, Ju Yeon; Lee, Young Jun; Lee, SAeung Ro
Most intrasellar meningiomas are located in the subdiaphragmatic and supraglandular region because they originate from the diaphragma sellae. Subglandular meningiomas located under the pituitary gland are extremely rare. Intrasellar meningiomas in the subdiaphragmatic and subglandular region probably originate from the dura in the sellar floor. We report a case of a subglandular meningioma along with a review of the literature.
Cha, Seung Woo; Park, Dong Woo; Park, Choong Ki; Pyo, Ju Yeon [College of Medicine, Hanyang University, Guri Hospital, Guri (Korea, Republic of); Lee, Young Jun; Lee, SAeung Ro [Dept. of Radiology, College of Medicine, Hanyang University, Seoul (Korea, Republic of)
Most intrasellar meningiomas are located in the subdiaphragmatic and supraglandular region because they originate from the diaphragma sellae. Subglandular meningiomas located under the pituitary gland are extremely rare. Intrasellar meningiomas in the subdiaphragmatic and subglandular region probably originate from the dura in the sellar floor. We report a case of a subglandular meningioma along with a review of the literature.
Bobeff, Ernest Jan; Wiśniewski, Karol; Papierz, Wielisław; Stefańczyk, Ludomir; Jaskólski, Dariusz Jan
Introduction: Ectopic sphenoid sinus pituitary adenoma is a rare tumour originating from embryologic remnants of Rathke's pouch. Although it is considered a clinically benign neoplasm, necrosis is encountered in 25% of cases and it can invade adjacent bone structures. Aims: To establish clinical, radiological and histopathological features of ectopic sphenoid sinus pituitary adenoma. Material and methods: Analysis of three cases: two females and one man, aged 61-70. Results: One patient presented with a unilateral hearing loss, the other two with headache and vertigo. They all suffered from type 2 diabetes mellitus. Neurological examination revealed no abnormality. Radiological imaging showed a sphenoid sinus space-occupying soft-tissue lesion with bone erosion in 2 cases and empty sella in 2 patients whereas one had a normal pituitary gland. All were operated on via the transnasal approach. Total resection was achieved in one patient and subtotal in two; in two cases we observed intact sellar dura and in one intact sellar floor. Histopathology showed immunoreactivity for synaptophysin in all cases and cytokeratin in two. The Ki-67 index was less than 2%. Immunohistochemical staining demonstrated growth hormone cells in all cases whereas prolactin and adrenocorticotropin in two. The patients were discharged home in good condition with no neurological deficits. Conclusions: Ectopic sphenoid sinus pituitary adenoma should always be considered in differential diagnosis of sphenoid sinus lesion in the elderly, especially in coexistence with empty sella or type 2 diabetes mellitus. Since ectopic sphenoid sinus pituitary adenoma is a benign lesion, surgical removal is an effective treatment. .
Ernest Jan Bobeff
Full Text Available Introduction : Ectopic sphenoid sinus pituitary adenoma is a rare tumour originating from embryologic remnants of Rathke’s pouch. Although it is considered a clinically benign neoplasm, necrosis is encountered in 25% of cases and it can invade adjacent bone structures. Aims : To establish clinical, radiological and histopathological features of ectopic sphenoid sinus pituitary adenoma. Material and methods: Analysis of three cases: two females and one man, aged 61-70. Results : One patient presented with a unilateral hearing loss, the other two with headache and vertigo. They all suffered from type 2 diabetes mellitus. Neurological examination revealed no abnormality. Radiological imaging showed a sphenoid sinus space-occupying soft-tissue lesion with bone erosion in 2 cases and empty sella in 2 patients whereas one had a normal pituitary gland. All were operated on via the transnasal approach. Total resection was achieved in one patient and subtotal in two; in two cases we observed intact sellar dura and in one intact sellar floor. Histopathology showed immunoreactivity for synaptophysin in all cases and cytokeratin in two. The Ki-67 index was less than 2%. Immunohistochemical staining demonstrated growth hormone cells in all cases whereas prolactin and adrenocorticotropin in two. The patients were discharged home in good condition with no neurological deficits. Conclusions : Ectopic sphenoid sinus pituitary adenoma should always be considered in differential diagnosis of sphenoid sinus lesion in the elderly, especially in coexistence with empty sella or type 2 diabetes mellitus. Since ectopic sphenoid sinus pituitary adenoma is a benign lesion, surgical removal is an effective treatment.
Viciano, Joan; De Luca, Stefano; López-Lázaro, Sandra; Botella, Daniel; Diéguez-Ramírez, Juan Pablo
Pituitary gigantism is a rare endocrine disorder caused by hypersecretion of growth hormone during growing period. Individuals with this disorder have an enormous growth in height and associated degenerative changes. The continued hypersecretion of growth hormone during adulthood leads to acromegaly, a condition related to the disproportionate bone growth of the skull, hands and feet. The skeletal remains studied belong to a young adult male from the Jewish necropolis of "Ronda Sur" in Lucena (Córdoba, Spain, VIII-XII centuries CE). The individual shows a very large and thick neurocranium, pronounced supraorbital ridges, an extremely prominent occipital protuberance, and an extremely large and massive mandible. Additional pathologies include enlargement of the vertebral bodies with degenerative changes, thickened ribs, and a slight increased length of the diaphysis with an increased cortical bone thickness of lower limbs. Comparative metric analysis of the mandible with other individuals from the same population and a contemporary Mediterranean population shows a trend toward acromegalic morphology. This case is an important contribution in paleopathological literature because it is a rare condition that has not been widely documented in ancient skeletal remains.
Carlos Augusto Real Martinez
Full Text Available BACKGROUND: Retroperitoneal lipoma is an extremely rare neoplasm. AIMS: The authors report a case of giant retroperitoneal lipoma in a 32-year-old white female, with a history of pain and an abdominal mass over a 2-year period. Total abdominal ultrasonography and barium enema showed a large mass located in the retroperitoneal space behind the ascending colon. Laparotomy showed a large encapsulated tumor measuring 20 x 13 x 10 cm and weighing 3.400 g. The histological study revealed a benign neoplasm of fatty cells. CONCLUSION: The patient remains well 17 years after surgery, without recurrentce of the disease.RACIONAL: O lipoma é a neoplasia mesenquimal mais freqüente, raramente localizada no retroperitônio. Na maioria das vezes, o diagnóstico diferencial pré-operatório com os lipossarcomas de baixo grau de malignidade é difícil de ser estabelecido. OBJETIVO: Apresentar um caso de lipoma gigante retroperitoneal em mulher de 32 anos que há 2 anos apresentava história de dor e tumor abdominal palpável. A ultra-sonografia abdominal e o enema opaco mostraram grande massa localizada no retroperitônio, que deslocava o ceco e o cólon ascendente. A laparotomia mostrou tumor encapsulado com 20 x 13 x 10 cm e 3.400 g de peso. O estudo histopatológico mostrou presença de lipoma retroperitonial. A paciente encontra-se bem, sem recidiva da doença, 17 anos após a cirurgia.
Vogl, T J; Nerlich, A; Dresel, S H; Bergman, C
We report the radiological findings in the unusual case of the Bavarian "Tegernsee Giant." With conventional radiography, CT, and histologic examination, we succeeded in diagnosing two disorders: The Tegernsee Giant suffered from (a) juvenile gigantism caused by a growth hormone-secreting tumor of the pituitary gland and (b) a polyostotic form of fibrous dysplasia of the skull and multiple bones particularly on the left side of the body.
Thawani, Jayesh P.; Bailey, Robert L.; Burns, Carrie M.; Lee, John Y. K.
Background: Examining the pathologic progression of a pituitary adenoma from the point of a prepubescent child to an adult with gigantism affords us an opportunity to consider why patients may develop secretory or functioning tumors and raises questions about whether therapeutic interventions and surveillance strategies could be made to avoid irreversible phenotypic changes. Case Description: A patient underwent a sublabial transsphenoidal resection for a clinically non-functioning macroa...
Full Text Available A girl with partial gigantism (the increased I and II fingers of the left foot is being examined. This condition is a rare and unresolved problem, as the definite reason of its development is not determined. Wait-and-see strategy is recommended, as well as correcting operations after closing of growth zones, and forming of data pool for generalization and development of schemes of drug and radial therapeutic methods.
W.W. de Herder (Wouter)
textabstractFamilial GH-secreting tumors are seen in association with three separate hereditary clinical syndromes: multiple endocrine neoplasia type 1, Carney complex, and familial isolated pituitary adenomas.
Wouter W. de Herder
Full Text Available Familial GH-secreting tumors are seen in association with three separate hereditary clinical syndromes: multiple endocrine neoplasia type 1, Carney complex, and familial isolated pituitary adenomas.
Sharifi, Guive; Jalessi, Maryam; Sarvghadi, Farzaneh; Farhadi, Mohammad
McCune-Albright syndrome (MAS) is an uncommon polyostotic manifestation of fibrous dysplasia in association with at least one endocrinopathy that is mostly associated with precocious puberty and hyperpigmented skin macules named café-au-lait spots. We present an atypical manifestation of McCune-Albright syndrome in a 19-year-old man with the uncommon association of polyostotic fibrous dysplasia and gigantism in the absence of café-au-lait spots and precocious puberty. He presented with a height increase to 202 cm in the previous 3 years, which had become more progressive in the few months prior. Physical examination revealed only a mild facial asymmetry; however, a computed tomography (CT) scan discovered vast areas of voluminous bones with ground-glass density and thickening involving the craniofacial bones and skull base. Magnetic resonance imaging (MRI) found a right stalk shift of the pituitary with a 20 mm pituitary adenoma. We describe the diagnostic and endoscopic endonasal transsphenoidal approach for excision of the tumor. Georg Thieme Verlag KG Stuttgart · New York.
Wolansky, L.J. [Department of Radiology, New Jersey Medical School, UMDNJ, Newark, NJ (United States); Gallagher, J.D. [Department of Radiology, New Jersey Medical School, UMDNJ, Newark, NJ (United States); Heary, R.F. [Neurological Surgery, New Jersey Medical School, UMDNJ, Newark, NJ (United States); Malantic, G.P. [Department of Radiology, New Jersey Medical School, UMDNJ, Newark, NJ (United States); Dasmahapatra, A. [Section of Endocrinology, New Jersey Medical School, UMDNJ, Newark, NJ (United States); Shaderowfsky, P.D. [Department of Radiology, New Jersey Medical School, UMDNJ, Newark, NJ (United States); Budhwani, N. [Department of Radiology, New Jersey Medical School, UMDNJ, Newark, NJ (United States)
Pituitary abscesses, rare lesions, may be divided into primary and secondary types. Primary pituitary abscesses occur within a previously healthy gland, while secondary abscesses arise within an existing lesion, such as an adenoma, craniopharyngioma, or Rathke`s cleft cyst. Secondary abscesses share radiologic characteristics with the lesions from which they arise. There has been no review of the MRI characteristics of primary pituitary abscesses. We report two cases and review the literature. The typical primary pituitary abscess gives the same or slightly lower signal than brain on T1-weighted images, and could be mistaken for a solid mass or presumed to represent a pituitary adenoma. Contrast-enhanced images are useful, demonstrating absence of central enhancement, suggesting a fluid or necrotic center. In one of our cases, meningeal enhancement was obvious; this has not been reported previously and may be diagnostic, when associated with a rim-enhancing pituitary mass. (orig.). With 3 figs., 1 tab.
Roerink, S.H.P.P.; Lindert, E.J. van; Ven, A.C. van de
In this double case report, we present two special cases of pituitary apoplexy. First, we describe a patient with growth hormone deficiency despite clinical suspicion of acromegaly. Imaging showed evidence of a recent pituitary apoplexy, which might have caused spontaneous remission of the
Full Text Available The article describes a one-year follow-up study of the clinical case of organic hallucinosis that we have described in the previous article in the patient with pituitary macroadenoma. At primary admission to the hospital (one year earlier the likelihood of permanent or recurrent nature of hallucinosis and absolutely poor prognosis for recovery and life without removal of the tumor was pointed out to the patient and his family. However, the patient and his relatives flatly refused to undergo neurosurgical intervention. The article describes the dynamics of psychopathological and somatic statuses of the patient during his readmission to a psychiatric hospital. The exciting cause of death on the 25th day of hospital stay was phenomenon of cerebral edema. By results of post-mortem studies the underlying cause of death was small cell chromophobe pituitary adenoma. There was a complete accuracy between clinical and post-mortem diagnosis. Absolutely unfavorable prognosis made before has been confirmed. The conclusion about the need of psychoeducational interventions with this contingent of patients and their relatives for prevention of an adverse disease outcome is made.
Kim, Young Tong; Bae, Won Kyung; Cho, Won Soo; Lee, Hye Kyoung
We report a case of reversible pituitary enlargement due to hypothyroidism in a 3-year-old male. On T1-weighted images, the mass was located in the anterior lobe of the pituitary gland, and the signal intensity of the mass was equal to that of brain cortex. Gd-DTPA enhanced T1-weighted images showed homogeneous enhancement of the mass. After supplemental therapy with thyroid hormone for 8 months, MRI showed that the pituitary gland was markedly smaller
Ranke, M B; Bierich, J R
In five cases of Sotos Syndrome serum somatomedin activities were measured. In two of these cases elevated levels and an increased secretion of growth hormone was observed. In one case (index case) a suspected hypothalamic tumor mass could be excluded, but hydrocephalus with increased intracranial pressure was present. The pathogenesis of gigantism in this syndrome is discussed.
Mahta, Ali; Haghpanah, Vahid; Lashkari, Anahita; Heshmat, Ramin; Larijani, Bagher; Tavangar, Seyed Mohammad
Pituitary adenomas without clinically active hypersecretion are summarized under the term non-functioning pituitary adenoma (NFPA). Since there are no specific serum markers, the differential diagnosis and treatment imply special difficulties. By using immunohistochemical methods we will have new insight into the nature and pathogenesis of these tumours. Ki-67 is a nuclear antigen detected by the monoclonal antibody MIB-1 and its labelling index (LI) is considered a marker of normal and abnormal cell proliferation. The aim of this study was to investigate the possible role of immunohistochemistry and MIB1-LI determination in NFPAs to predict tumoural behaviour and better management. In this clinicopathological study, 85 cases of NFPAs were analysed immunohistochemically. MIB1-LI was also determined in studied cases. Clinical presentation, treatment and follow-up data were also reviewed and the correlation between clinical and pathologic findings was established. Eighteen adenomas (21.2%) were immunoreactive to one or two adenohypophysial hormones of which 4 GH positive adenomas had aggressive behaviour (2 significant juxtasellar extensions and 2 recurrences). MIB-1 LI was more than 5% in only 5 cases including 2 invasive adenomas but with no evidence of recurrence. No significant statistical difference between clinical presentations in immunoreactive and non-immunoreactive NFPAs was observed except for unilateral temporal hemianopia which was more common in immunoreactive adenomas (P=0.022). NFPAs comprise several pathologically different types of tumours, some of which are potentially hormone producing, but some defects in hormone secretion or production of biologically inactive or insufficient amount of hormone may be the culprit in the lack of evidence of rising serum hormone levels. MIB-1 LI may be indicative of invasiveness but not a predictor of recurrence. Silent somatotropinomas may have more aggressive behaviour in comparison with other NFPAs.
Tanaka, Tomoko; Hiramatsu, Katsushi; Nosaka, Takuto; Saito, Yasushi; Naito, Tatsushi; Takahashi, Kazuto; Ofuji, Kazuya; Matsuda, Hidetaka; Ohtani, Masahiro; Nemoto, Tomoyuki; Suto, Hiroyuki; Yamamoto, Tatsuya; Kimura, Hirohiko; Nakamoto, Yasunari
Metastasis to the pituitary gland is extremely rare and is often detected incidentally by symptoms associated with endocrine dysfunction. Breast and lung cancer are the most common primary metastasizing to pituitary gland. Metastasis from hepatocellular carcinoma to the pituitary gland is extremely rare, with only 10 cases having been previously reported. We present here the first case of pituitary metastasis of hepatocellular carcinoma presenting with panhypopituitarism diagnosed by magnetic resonance imaging. We report the case of an 80-year-old Japanese woman who presented with the sudden onset of hypotension and bradycardia after having previously been diagnosed with hepatocellular carcinoma. Based on low levels of pituitary hormones, she was diagnosed with panhypopituitarism caused by metastasis of the hepatocellular carcinoma to the pituitary gland. Magnetic resonance imaging with arterial spin-labeling was effective in the differential diagnosis of the intrasellar tumor. The patient died despite hormone replacement therapy because of hypovolemic shock. Metastasis to the pituitary gland causes various non-specific symptoms, so it is difficult to diagnose. The present case emphasizes the importance of diagnostic imaging in identifying these metastases. Clinicians should consider the possibility of pituitary metastasis in patients with malignant tumors who demonstrate hypopituitarism.
Tanaka, Tomoko; Hiramatsu, Katsushi; Nosaka, Takuto; Saito, Yasushi; Naito, Tatsushi; Takahashi, Kazuto; Ofuji, Kazuya; Matsuda, Hidetaka; Ohtani, Masahiro; Nemoto, Tomoyuki; Suto, Hiroyuki; Yamamoto, Tatsuya; Kimura, Hirohiko; Nakamoto, Yasunari
Metastasis to the pituitary gland is extremely rare and is often detected incidentally by symptoms associated with endocrine dysfunction. Breast and lung cancer are the most common primary metastasizing to pituitary gland. Metastasis from hepatocellular carcinoma to the pituitary gland is extremely rare, with only 10 cases having been previously reported. We present here the first case of pituitary metastasis of hepatocellular carcinoma presenting with panhypopituitarism diagnosed by magnetic resonance imaging. We report the case of an 80-year-old Japanese woman who presented with the sudden onset of hypotension and bradycardia after having previously been diagnosed with hepatocellular carcinoma. Based on low levels of pituitary hormones, she was diagnosed with panhypopituitarism caused by metastasis of the hepatocellular carcinoma to the pituitary gland. Magnetic resonance imaging with arterial spin-labeling was effective in the differential diagnosis of the intrasellar tumor. The patient died despite hormone replacement therapy because of hypovolemic shock. Metastasis to the pituitary gland causes various non-specific symptoms, so it is difficult to diagnose. The present case emphasizes the importance of diagnostic imaging in identifying these metastases. Clinicians should consider the possibility of pituitary metastasis in patients with malignant tumors who demonstrate hypopituitarism
Amaral, L.L.F. do; Mendonca, R.A.; Natal, M.R.C.
The authors present two cases of pituitary dwarfism where the study by high resolution computed tomography and by magnetic resonance imaging show transection of the pituitary stalk as the cause of the endocrine disturb. The importance of these methods on the investigation of disturbs of the hypothalamus-hypophyseal axis is stressed. (author)
Asymptomatic pituitary abnormalities occur in about 10% of cranial magnetic resonance imaging scans, but metastatic carcinoma of the pituitary gland is rare: 133 cases have been reported. Two thirds secreted either prolactin or adrenocorticotropic hormone, and another 24% were non-secreting.
Steenbrugge, F; Poffyn, B; Uyttendaele, D; Verdonk, R; Verstraete, K
The authors report the case of a 13-year-old patient with neurofibromatosis (NF-I), who suffered blunt trauma to the left tibia in 1993. The diagnosis of subperiosteal hematoma was made. Treatment consisted of temporary rest. There was a recurrence in 1996, and the subperiosteal hematoma was drained. In 1997, a shortening osteotomy of the left tibia was performed. However, massive gigantism with elephantiasis of the left leg remained, causing a serious functional and cosmetic problem. In 1999, the leg was amputated above the knee. The literature is reviewed and 7 case reports are compared. The pathogenesis of subperiosteal hematoma is discussed.
Yoshida, Masanori; Murakami, Miho; Ueda, Harumi; Miyata, Misaki; Takahashi, Norio; Oiso, Yutaka
Although pituitary function is often impaired in pituitary apoplexy, the development of thyrotoxicosis is rare. We describe an unusual case of hypopituitarism due to pituitary apoplexy coexisting with transient hyperthyroidism. A 74-year-old woman presented with severe fatigue, palpitation, appetite loss, hypotension, and hyponatremia. Endocrine studies showed hyperthyroidism and anterior pituitary hormone deficiencies. A magnetic resonance imaging suggested recent-onset pituitary apoplexy in a pituitary tumor, although the patient had no apoplectic symptoms such as headache and visual disturbance. Thyrotoxicosis and adrenal insufficiency worsened her general condition. Glucocorticoid supplementation improved her clinical symptoms and hyponatremia. Serum anti-thyrotropin receptor and thyroid-stimulating antibody titers were negative, and her thyroid function was spontaneously normalized without antithyroid medication, suggesting painless thyroiditis. Thereafter, her thyroid function decreased because of central hypothyroidism and 75 µg of levothyroxine was needed to maintain thyroid function at the euthyroid stage. The pituitary mass was surgically removed and an old hematoma was detected in the specimen. Considering that painless thyroiditis develops as a result of an autoimmune process, an immune rebound mechanism due to adrenal insufficiency probably caused painless thyroiditis. Although the most common type of thyroid disorder in pituitary apoplexy is central hypothyroidism, thyrotoxicosis caused by painless thyroiditis should be considered even if the patient has pituitary deficiencies. Because thyrotoxicosis with adrenal insufficiency poses a high risk for a life-threatening adrenal crisis, prompt diagnosis and treatment are critical.
Chao, Chen-Cheng; Lin, Chun-Ju
Pituitary apoplexy is a rare clinical emergency which results from hemorrhage or infarction in the pituitary gland. We present a 14-year-old girl with pituitary apoplexy and review the literature. Our patient experienced blurred vision, nausea, and headache. Her best-corrected visual acuity was 20/200 and 20/20. Confrontation test visual field testing revealed bitemporal hemianopsia. Brain imaging demonstrated a suprasellar mass. The microscopic endonasal transsphenoidal approach only found 5-10 mL brownish fluid-like material. Pathology confirmed no malignancy. Pituitary apoplexy was diagnosed. Her nausea and headache gradually improved. Six months after operation, her best-corrected visual acuity had improved to 20/30 and 20/20. Although pituitary apoplexy is rare in pediatric patients, prompt evaluation including detailed ophthalmic examination, biochemical evaluation, endocrine workup, and image study are very important. Copyright © 2014 Elsevier Inc. All rights reserved.
Domenico Billeci, M.D.
Full Text Available Pituitary apoplexy is a dramatic condition that can occur spontaneously or triggered by various precipitating factors. Head trauma is a rare but well-recognized cause of apoplectics events. We present the case of an 81-year-old woman, with negative past medical history and under antiplatelet agents, who experienced an isolated VI cranial nerve palsy 24 h after a mild head trauma. Early brain CT revealed an unknown pituitary lesion without signs of intralesional bleeding. Only late brain MRI imaging revealed pituitary apoplexy together with a subarachnoid hemorrhage. After aggravation of neurological condition the patient, undergo endoscopic transsphenoidal decompression of cranial nerves with rapid deficits improvement. Our aim is to share our experience and to propose the first critical review of all cases of post-traumatic pituitary apoplexy described in literature. We also try to suggest some management advice for post traumatic pituitary apoplexy.
Full Text Available Background. Hypophysitis is an inflammatory disease of the pituitary gland that may mimic pituitary tumors clinically and radiologically. Case Description. We report a case of a xanthomatous hypophysitis initially diagnosed as pituitary adenoma. A 31-year-old woman presented with headache, diabetes insipidus, and amenorrhea. A head CT scan showed no intrasellar changes, while an MRI scan showed a sellar cystic mass. An endocrinological work up revealed mild hypocortisolism and diabetes insipidus (DI. Transsphenoidal surgery was performed. The intraoperative histological examination suggested a pituitary adenoma. The removed tissue showed central necrosis surrounded by accumulation of foamy cells and xanthomatous epithelioid cells. The patient made an uneventful postoperative recovery, Nevertheless, DI persisted and the adenohypophysis hypofunction did not recover. Conclusion. We describe an unusual inflammatory lesion of the pituitary gland mimicking an adenoma. A high level of clinical suspicion of inflammatory disorders is necessary for correct diagnosis and optimal management.
Ray, Munni; Malhi, P; Bhalla, A K; Singhi, P D
A case of cerebral gigantism (Sotos syndrome) with West syndrome in a one-year-old male child is reported. The case had a large stature, typical facies and neurodevelopmental delay along with infantile spasms, which were refractory to treatment with valproate and clonazepam.
Full Text Available Yu Liu,1 Feng Liu,2 Qi Liang,3 Yexin Li,4 Zhifei Wang5 1Department of Neurosurgery, The Third Xiangya Hospital, Central South University, Changsha, 2Department of Neurosurgery, The Third Xiangya Hospital, Central South University, Changsha, 3Department of Radiology, The Third Xiangya Hospital, Central South University, Changsha, 4Department of Neurosurgery, The Central Hospital of Shaoyang, Shaoyang, 5Department of Neurosurgery, The Third Xiangya Hospital, Central South University, Changsha, Hunan, People’s Republic of China Abstract: Pituitary abscess is a rare but critical disorder caused by an infectious process where purulent material accumulates inside the sella turcica. Since symptoms, signs and radiographic characteristics of pituitary abscess are similar to several other pituitary lesions, correct diagnosis before surgery is challenging. In this article, two cases of pituitary abscess treated in our department are reported, followed by a literature review. In these two cases, both patients presented with intermittent fever. Magnetic resonance imaging revealed a suprasellar lesion with rim enhancement after contrast injection. After transsphenoidal surgery, the diagnosis of pituitary abscess was confirmed. The patients were then given antibiotic treatment and recovered fully in less than 2 months. Findings of this article support timely diagnosis and proper treatment including transsphenoidal surgery and antibiotic therapy for pituitary abscess, leading to lower mortality rates and higher probability of pituitary hormone function recovery. Keywords: pituitary abscess, transsphenoidal resection, antibiotic therapy, diagnosis
Full Text Available Pituitary carcinoma (PC is a very rare entity (0.2% of all pituitary tumors, with only about 140 cases reported in English literature. There are no reliable histological, immunohistochemical or ultrastructural features distinguishing pituitary adenoma (PA from PC. By definition, a diagnosis of PC is made after a patient with PA develops non-contiguous central nervous system (CNS or systemic metastases. To date, only three cases of PC have been reportedly diagnosed on fine needle aspiration (FNA. Two of the reported cases were diagnosed on FNA of the cervical lymph nodes and one on FNA of the vertebral bone lesion. Herein, we present a case of PC, diagnosed on FNA of the liver lesion. In this case, we describe cytologic features of PC and compare them to histologic features of the tumor in the pituitary. Clinical behavior of tumor, pathogenesis of metastasis and immunochemical and prognostic markers will also be described.
Finer, N; Emery, P; Hicks, B H
Mammary gigantism is a rare complication of D-penicillamine treatment. We report a further case with pathological and endocrine details together with a review of the seven cases previously reported and possible mechanisms.
Trivellin, Giampaolo; Daly, Adrian F; Faucz, Fabio R; Yuan, Bo; Rostomyan, Liliya; Larco, Darwin O; Schernthaner-Reiter, Marie Helene; Szarek, Eva; Leal, Letícia F; Caberg, Jean-Hubert; Castermans, Emilie; Villa, Chiara; Dimopoulos, Aggeliki; Chittiboina, Prashant; Xekouki, Paraskevi; Shah, Nalini; Metzger, Daniel; Lysy, Philippe A; Ferrante, Emanuele; Strebkova, Natalia; Mazerkina, Nadia; Zatelli, Maria Chiara; Lodish, Maya; Horvath, Anelia; de Alexandre, Rodrigo Bertollo; Manning, Allison D; Levy, Isaac; Keil, Margaret F; Sierra, Maria de la Luz; Palmeira, Leonor; Coppieters, Wouter; Georges, Michel; Naves, Luciana A; Jamar, Mauricette; Bours, Vincent; Wu, T John; Choong, Catherine S; Bertherat, Jerome; Chanson, Philippe; Kamenický, Peter; Farrell, William E; Barlier, Anne; Quezado, Martha; Bjelobaba, Ivana; Stojilkovic, Stanko S; Wess, Jurgen; Costanzi, Stefano; Liu, Pengfei; Lupski, James R; Beckers, Albert; Stratakis, Constantine A
Increased secretion of growth hormone leads to gigantism in children and acromegaly in adults; the genetic causes of gigantism and acromegaly are poorly understood. We performed clinical and genetic studies of samples obtained from 43 patients with gigantism and then sequenced an implicated gene in samples from 248 patients with acromegaly. We observed microduplication on chromosome Xq26.3 in samples from 13 patients with gigantism; of these samples, 4 were obtained from members of two unrelated kindreds, and 9 were from patients with sporadic cases. All the patients had disease onset during early childhood. Of the patients with gigantism who did not carry an Xq26.3 microduplication, none presented before the age of 5 years. Genomic characterization of the Xq26.3 region suggests that the microduplications are generated during chromosome replication and that they contain four protein-coding genes. Only one of these genes, GPR101, which encodes a G-protein-coupled receptor, was overexpressed in patients' pituitary lesions. We identified a recurrent GPR101 mutation (p.E308D) in 11 of 248 patients with acromegaly, with the mutation found mostly in tumors. When the mutation was transfected into rat GH3 cells, it led to increased release of growth hormone and proliferation of growth hormone-producing cells. We describe a pediatric disorder (which we have termed X-linked acrogigantism [X-LAG]) that is caused by an Xq26.3 genomic duplication and is characterized by early-onset gigantism resulting from an excess of growth hormone. Duplication of GPR101 probably causes X-LAG. We also found a recurrent mutation in GPR101 in some adults with acromegaly. (Funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development and others.).
Prerana N Shah
Full Text Available Successful anaesthetic management for caesarean section in a case with previous pituitary tumour resection, with residual tumour, is reported. The pituitary gland undergoes global hyperplasia during pregnancy. Functional pituitary tumours may exhibit symptomatic enlargement during pregnancy. Growth hormone secreting tumour is associated with acromegaly which has associated anaesthetic implications of difficult airway, systemic hypertension, and diabetes and electrolyte imbalance. Intracranial space occupying lesions can increase intra cranial pressure and compromise cerebral perfusion or cause herniation. We report management of this case.
İlhan Kılınç; Deniz Gökalp; Cihan Akgül Özmen
Ectopic neurohypophysis is an anomaly of the Pituitary gland whichmay be associated with short stature due to Growth hormone deficiency.MRI is the modality of choice in diagnosing this condition. We present acase of pituitary dwarfism and ectopic neurohypophysis with clinical andradiological findings. 21 year-old male admitted with short stature. Allhormones, except prolactin, of anterior hypophysis were low. Bright spotwas ectopically located at level of median eminence on enhanced MRI ofhyp...
Zarghooni, Kourosh; Sobotrke, Rolf; Schmidt, Heinrich; Rollinghoff, Marc; Siewe, Jan; Eysel, Peer
The authors present what appears to be the first case of congenital kyphosis due to a T12 hemivertebra in a four-year-old boy with endochondral gigantism syndrome of unknown origin. Because of his overgrowth, the patient had severe medical and orthopaedic problems and was almost immobile. Prior to surgery, he experienced a rapidly progressive thoracolumbar kyphosis to 600 (T10-L2). MRI of the brain and spine showed critical protraction of the spinal cord and myelopathy from compression at T12. Single-stage posterior resection of the hemivertebra with spinal shortening and dorsal transpedicular instrumentation of T10-L2 was performed. Although the bone tissue was cartilaginous and dysplastic, 420 (30%) correction was achieved along with decompression of the spinal canal. The patient experienced no neurological impairment post-operatively. At follow-up examination 1.5 year after surgery, the patient's movement disorder had improved markedly and he was able to stand and walk. This very rare case demonstrates that single-stage posterior hemivertebra resection and transpedicular instrumentation for correction of congenital kyphosis can be a safe and effective procedure even in a very challenging case.
A collection of 893 historical picture postcards from 1900 to 1935, depicting dwarfs and giants, was analysed from medical and psychosocial viewpoints. In conditions such as 'bird headed dwarfism', achondroplasia, cretinism, so-called Aztecs or pinheads, Grebe chondrodysplasia, and acromegalic gigantism, the disorder could be diagnosed easily. In hypopituitary dwarfism, exact diagnosis was more difficult because of heterogeneity. The most common conditions depicted were pituitary dwarfism and...
Full Text Available OBJECTIVE: Pituitary stalk interruption syndrome (PSIS is characterized by the absence of pituitary stalk, pituitary hypoplasia, and ectopic posterior pituitary. Due to the rarity of PSIS, clinical data are limited, especially in Chinese people. Herein, we analyzed the clinical characteristics of patients diagnosed with PSIS from our center over 10 years. PATIENTS AND METHODS: We retrospectively analyzed the clinical manifestations and laboratory and MRI findings in 55 patients with PSIS. RESULTS: Of the 55 patients with PSIS, 48 (87.3% were male. The average age was 19.7±6.7 years and there was no familial case. A history of breech delivery was documented in 40 of 45 patients (88.9% and 19 of 55 patients (34.5% had a history of dystocia. Short stature was found in 47 of 55 patients (85.5% and bone age delayed 7.26±5.37 years. Secondary sex characteristics were poor or undeveloped in most patients. The prevalence of deficiencies in growth hormone, gonadotropins, corticotropin, and thyrotropin were 100%, 95.8%, 81.8%, 76.3%, respectively. Hyperprolactinemia was found in 36.4% of patients. Three or more pituitary hormone deficiencies were found in 92.7% of the patients. All patients had normal posterior pituitary function and absent pituitary stalk on imaging. The average height of anterior pituitary was 28 mm, documented anterior pituitary hypoplasia. Midline abnormalities were presented in 9.1% of patients. CONCLUSIONS: The clinical features of our Chinese PSIS patients seem to be different from other reported patients in regarding to the higher degree of hypopituitarism and lower prevalence of midline defects. In addition, our patients were older at the time of case detection and the bone age was markedly delayed. We also had no cases of familial PSIS.
Arai, Nobuhiko; Inaba, Makoto; Ichijyo, Takamasa; Kagami, Hiroshi; Mine, Yutaka
Thyrotropin-producing pituitary tumor is relatively rare. In particular, concurrent cases associated with Graves' disease are extremely rare and only nine cases have been reported so far. We describe a case of a thyrotropin-producing pituitary adenoma concomitant with Graves' disease, which was successfully treated. A 40-year-old Japanese woman presented with mild signs of hyperthyroidism. She had positive anti-thyroid-stimulating hormone receptor antibody, anti-thyroglobulin antibody, and anti-thyroid peroxidase antibody. Her levels of serum thyroid-stimulating hormone, which ranged from low to normal in the presence of high levels of serum free thyroid hormones, were considered to be close to a state of syndrome of inappropriate secretion of thyroid-stimulating hormone. Magnetic resonance imaging showed a macropituitary tumor. The coexistence of thyrotropin-producing pituitary adenoma and Graves' disease was suspected. Initial therapy included anti-thyroid medication, which was immediately discontinued due to worsening symptoms. Subsequently, surgical therapy for the pituitary tumor was conducted, and her levels of free thyroid hormones, including the thyroid-stimulating hormone, became normal. On postoperative examination, her anti-thyroid-stimulating hormone receptor antibody levels decreased, and the anti-thyroglobulin antibody became negative. The coexistence of thyrotropin-producing pituitary adenoma and Graves' disease is rarely reported. The diagnosis of this condition is complicated, and the appropriate treatment strategy has not been clearly established. This case suggests that physicians should consider the coexistence of thyrotropin-producing pituitary adenoma with Graves' disease in cases in which thyroid-stimulating hormone values range from low to normal in the presence of thyrotoxicosis, and the surgical treatment of thyrotropin-producing pituitary adenoma could be the first-line therapy in patients with both thyrotropin-producing pituitary adenoma
Santos, Alair Augusto S.M.D. dos; Fontes, Cristina Asvolinsque P.; Magnago, Marcelo [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Inst. de Radiologia; Moreira, Denise Madeira [Universidade Federal, Rio de Janeiro, RJ (Brazil). Inst. de Radiologia; Andreiuolo, Pedro Angelo [Hospital Santa Cruz/Beneficencia Portuguesa, Niteroi, RJ (Brazil). Servico de Radiologia; Oliveira, Fernando Barros de; Teixeira, Ricardo Tostes D.; Correa, Saul Orlando C. [Universidade Federal, Rio de Janeiro, RJ (Brazil). Inst. de Biofisica Carlos Chagas Filho. Curso de Pos-graduacao Medica
We reviewed 60 cases of patients with pituitary macroadenomas who were submitted to magnetic resonance imaging evaluations at private institutions in Niteroi and Rio de Janeiro, Brazil. The aim of this study was to demonstrate the advantages of magnetic resonance imaging in the diagnosis and follow-up of these patients. Macroprolactinomas were the most prevalent tumors. We also observed four growth-hormone (GH) secreting macroadenomas with clinical signs of acromegaly, and one GH- and prolactin-secreting macroadenoma. Seven patients presented pituitary apoplexy and hyperintense signal on T1-W images before contrast medium (gadolinium) administration suggestive of intratumoral bleeding. The follow-up of 15 cases demonstrated a reduction in the size of the tumor after surgery, clinical treatment or radiotherapy. Magnetic resonance imaging is important for the study of pituitary macroadenomas, particularly before surgery, as it shows the involvement of adjacent structures, specially the cavernous sinus, optic chiasm and pituitary stalk compression. (author)
Ferrante, Franco; Blasi, Sergio; Crippa, Rolando; Angiero, Francesca
Hypopituitarism is a disorder caused by a reduced level of trophic hormones that may be consequent on different destructive processes. The clinical manifestations depend on the type of hormone involved. A deficiency of growth hormone (GH) in children causes the lack of growth known as pituitary dwarfism. The case is reported of a patient with pituitary dwarfism, multiple dental anomalies, functional prosthetic problems, and a revision of the literature. She was subjected to prosthetic rehabil...
Urbani, C; Russo, D; Raggi, F; Lombardi, M; Sardella, C; Scattina, I; Lupi, I; Manetti, L; Tomisti, L; Marcocci, C; Martino, E; Bogazzi, F
Acromegaly usually occurs as a sporadic disease, but it may be a part of familial pituitary tumor syndromes in rare cases. Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene have been associated with a predisposition to familial isolated pituitary adenoma. The aim of the present study was to evaluate the AIP gene in a patient with gigantism and in her relatives. Direct sequencing of AIP gene was performed in fourteen members of the family, spanning among three generations. The index case was an 18-year-old woman with gigantism due to an invasive GH-secreting pituitary adenoma and a concomitant tall-cell variant of papillary thyroid carcinoma. A novel germline mutation in the AIP gene (c.685C>T, p.Q229X) was identified in the proband and in two members of her family, who did not present clinical features of acromegaly or other pituitary disorders. Eleven subjects had no mutation in the AIP gene. Two members of the family with clinical features of acromegaly refused either the genetic or the biochemical evaluation. The Q229X mutation was predicted to generate a truncated AIP protein, lacking the last two tetratricopeptide repeat domains and the final C-terminal α-7 helix. We identified a new AIP germline mutation predicted to produce a truncated AIP protein, lacking its biological properties due to the disruption of the C-terminus binding sites for both the chaperones and the client proteins of AIP.
Aaltonen, Lauri A.; Daly, Adrian F.
Pituitary adenomas are one of the most frequent intracranial tumors and occur with a prevalence of approximately 1:1000 in the developed world. Pituitary adenomas have a serious disease burden, and their management involves neurosurgery, biological therapies, and radiotherapy. Early diagnosis of pituitary tumors while they are smaller may help increase cure rates. Few genetic predictors of pituitary adenoma development exist. Recent years have seen two separate, complimentary advances in inherited pituitary tumor research. The clinical condition of familial isolated pituitary adenomas (FIPA) has been described, which encompasses the familial occurrence of isolated pituitary adenomas outside of the setting of syndromic conditions like multiple endocrine neoplasia type 1 and Carney complex. FIPA families comprise approximately 2% of pituitary adenomas and represent a clinical entity with homogeneous or heterogeneous pituitary adenoma types occurring within the same kindred. The aryl hydrocarbon receptor interacting protein (AIP) gene has been identified as causing a pituitary adenoma predisposition of variable penetrance that accounts for 20% of FIPA families. Germline AIP mutations have been shown to associate with the occurrence of large pituitary adenomas that occur at a young age, predominantly in children/adolescents and young adults. AIP mutations are usually associated with somatotropinomas, but prolactinomas, nonfunctioning pituitary adenomas, Cushing disease, and other infrequent clinical adenoma types can also occur. Gigantism is a particular feature of AIP mutations and occurs in more than one third of affected somatotropinoma patients. Study of pituitary adenoma patients with AIP mutations has demonstrated that these cases raise clinical challenges to successful treatment. Extensive research on the biology of AIP and new advances in mouse Aip knockout models demonstrate multiple pathways by which AIP may contribute to tumorigenesis. This review assesses
Mukhida, Karim; Asa, Sylvia; Gentili, Fred; Shannon, Patrick
The authors describe a case of pituitary fossa ependymoma and discuss its immunohistochemical and ultrastructural characteristics. A 43-year-old man presented with decreased libido and panhypopituitarism. Magnetic resonance imaging demonstrated a well-demarcated enhancing lesion of the pituitary fossa that was completely resected via a transsphenoidal approach. Ependymomas rarely occur in the pituitary fossa, and have been reported in this location only three times in humans and once in a horse. This is the first study in which investigators examined the appearance of a pituitary ependymoma by using electron microscopy. Theories of the origin and treatment of these rare tumors are discussed as they relate to other articles on intracranial ependymomas.
Full Text Available The detailed surgical procedure of the transsphenoidal surgery for pituitary abscess has scarcely been described previously because it is a very rare clinical entity. The authors reported two cases of primary pituitary abscess. In case 1, the anterior wall of the sella turcica was reconstructed with the vomer bone after irrigating the abscess cavity, but the sella was not packed by fat for fear of the persistent infection by devascularized tissues. This led to the postoperative meningocele, the cerebrospinal fluid leak, and bacterial meningitis despite the successful abscess drainage. In case 2, tight sellar packing and reconstruction of the sellar wall were performed to avoid these postoperative complications, which resulted in complete drainage and uneventful postoperative course. Although accumulation of more cases is obviously needed to establish the definitive surgical technique in pituitary abscess surgery, our experience might suggest that packing of the sella is not impeditive for postoperative sufficient drainage.
Jin, Sung Chan; Lee, Seoung Ro; Kwon, Bae Ju; Moon, Won Jin; Jeon, Eui Yong [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)
A 27-year-old man presented with complaints of headache and visual disturbance, first noted six months earlier. Simple radiographs of skull sellar widening and calcification. Brian CT revealed a 3 x 3 x 4 cm-sized sellar suprasellar mass with heavy calcification. T1-weighted MR images showed that the signal intencity of the mass was slightly lower than that of the gray matter, while T2-weighted images showed heterogeneous high signal intensity with centrl low-signal-intensity foci, suggesting calcification After contrast infusion, enancement was irregular. Surgery revealed a 4 x 5 cm sized, well-demarcated, lobulated mass adhering to the meninges. Papillary-type pituitary adenoma was histologically confirmed. We report the CT and MR findings of atypical pituitary adenoma with extensive internal calcification mimicking craniopharyngioma.
Anterior Gray Matter Pituicytic Heterotopia with Monomorphic Anterior Pituitary Cells: A Variant of Nonsecretory Pituitary Adenoma Neuronal Choristoma? Report of a Rare Case and Review of the Literature.
Yowtak, June; Sharma, Suash; Forseen, Scott E; Alleyne, Cargill H
Mixed tumors of adenomatous and neuronal cells in the sellar region are an uncommon finding. The origins of these heterogeneous tumors are unknown, and management remains unsettled. We report a very rare case of anterior gray matter pituicytic heterotopia with monomorphic anterior pituitary cells that likely represents a variant of nonsecreting pituitary adenoma neuronal choristoma (PANCH) with no ganglion cells. We also review the current literature for the various clinical presentations of PANCH. A 49-year-old female complaining of headache, blurred vision, and hair loss was found to have a nonsecretory sellar mass with compression of the optic chiasm on magnetic resonance imaging (MRI). The mass was excised via a transsphenoidal procedure. Histological analysis of tissue sections revealed heterotopic gray matter with reactive gliosis without ganglion cells or Herring bodies. Only 1 smear exhibited characteristics of a pituitary adenoma. The overall findings were most consistent with a variant of PANCH. At a postoperative follow-up of 4.5 years, there was resolution of visual symptoms, and the residual sellar mass was stable on MRI. Neuronal choristoma is hypothesized to originate from embryonal pituitary or hypothalamus, or by differentiation from pituitary adenoma cells. Surgery is the cornerstone of management, and the clinical course appears to be similar to that of nonfunctioning pituitary adenoma in reported cases. Copyright © 2016 Elsevier Inc. All rights reserved.
Full Text Available Abstract Introduction Asymptomatic pituitary abnormalities occur in about 10% of cranial magnetic resonance imaging scans, but metastatic carcinoma of the pituitary gland is rare: 133 cases have been reported. Two thirds secreted either prolactin or adrenocorticotropic hormone, and another 24% were non-secreting. Case presentation A 42-year-old Caucasian man lived for 30 years after the diagnosis of a pituitary tumor whose clinical and biochemical features were those of acromegaly and hypogonadism. Radiotherapy, totaling 7300 rad, was administered to the sella over two courses. Growth hormone levels normalized, but he developed both thyroid and adrenal insufficiency, and replacement therapy was commenced. Fourteen years later, growth hormone levels again became elevated, and bromocriptine was commenced but led to side effects that could not be tolerated. An attempted surgical intervention failed, and octreotide and pergolide were used in succession. Twenty-seven years after the diagnosis, a mass from an excisional biopsy of below the angle of the mandible proved to be metastatic pituitary carcinoma. Immunohistochemical staining was positive for synaptophysin, growth hormone, and prolactin. One year later, an octreotide scan showed uptake at the sella, neck, and spleen. Our patient declined further active oncology treatment. Conclusions Metastatic pituitary carcinoma associated with acromegaly is particularly rare. To the best of our knowledge, this is the eighth such case and is the first report of growth hormone and prolactin present in the metastatic mass.
Hameed, Aisha; Mehta, Vaishali; Sinha, Prabha
We are reporting a rare case of de novo ovarian abscess in an endometrioma. Ovarian abscess within an endometrioma is a rare gynecological problem, but de novo abscess in the endometrioma is even rarer. Most of the ovarian abscesses develop in the endometriomas following interventions, e.g., aspiration, pelvic surgery, and oocyte retrieval. We are presenting a case of a spontaneous giant abscess in a large ovarian cyst in a nulliparous woman who presented with acute abdomen. Patient was treated in a district general hospital with multidisciplinary approach. Thirteen liters of the pus were drained. She has had a sub total (supra cervical) hysterectomy and bilateral salpingo-oophorectomy (BSO) performed. Histology of the abscess wall confirmed endometriotic nature of the cyst. Patient made an uneventful recovery and was discharged home on the 14th postoperative day. This case highlights that endometrioma and its complication can present as a surgical emergency and should be dealt as one.
Wang Hongsheng; Chen Huaqun; Dong Congsong; Li Wenhui; Dai Zhenyu; Chen Guozhi
Objective: To evaluate the clinical efficacy of preoperative embolization in treatment of patients with gigantic meningioma. Methods: Fourteen cases of gigantic meningioma diameter from 6 to 11 cm were measured by CT and MRI scan. DSA manifested that they are vascularizd meningioma and showed the mainly feeding arteries. We used getation sponge to superselectively embilized the feeding arteries. All tumors were performed surgical excision 3-7 days after the embolization. Results: DSA showed the blood supplies in the tumors in 9 cases were completely blocked, and that in 5 cases were dramatically eliminated. All patients were operated 3-7 days after the embolization. During the operations the bleeding were dramatically decreased and the operation time was shortened compared with those in unembolized cases. It helps us remove the tumors easy and quickly from the attachments. No complication occurred during and after the operations. Conclusion: Preoperative embolization of gigantic meningioma is a useful and relatively safe method in helping surgicaly and completely excised of tumor with significant reduction of blood loss and operation time. (authors)
Kim, Dong Gun; Heo, Young Jin; Kim, Eun Kyoung; Baek, Jin Wook; Jeong, Hae Woong; Jung, Hyun Seok [Busan Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)
Intracranial neuroblastomas are uncommon malignant tumors that usually arise in the supratentorial parenchymal or paraventricular location. A primary neuroblastoma arising in the sella turcica is extremely rare. We report a case of a 76-year-old man who presented with progressive bitemporal hemianopsia. His pituitary hormone levels were within the normal range, except for slightly increased prolactin. Pituitary magnetic resonance imaging revealed a solitary sellar mass with supra- and parasellar extension that mimicked a non-functioning pituitary adenoma or meningioma. The tumor was excised by transsphenoidal resection. Histopathologic analysis revealed small cells surrounded by a dense fibrillary stroma as well as strong expression of neural markers. Hence, the patient was diagnosed with sellar neuroblastoma. Prolactin levels normalized in the immediate postoperative period, although visual disturbances persisted. Herein, we describe the clinical manifestations, MRI characteristics, and histopathologic findings of this case.
Bladowska, J.; Biel, A.; Bednarek-Tupikowska, G.; Sasiadek, M.
Background: The sellar and parasellar region is an area where many heterogenous neoplastic, inflammatory, developmental and vascular pathologies can occur. Differentiation among various diseases may be not easy, because many of these lesions could mimic the clinical, endocrinologic and radiologic features of pituitary adenomas, which can be the cause of possible misdiagnosis. Case Report: We report a case of a 52-year-old man who presented with a persistent headache for the last 5 - 6 years and visual disturbances. Endocrine system examinations disclosed only insignificant hyperprolactinaemia. The MRI revealed an hypointense area - its presentation was similar to that of pituitary adenoma. The correct diagnosis, i.e. a colloid cyst of the pituitary gland, was made intraoperatively. Conclusions: Colloid cyst of the pituitary gland is a very rare pathology but it must be considered if there is an hypointense area between the anterior and posterior pituitary lobe in MR imaging without contrast enhancement and if the patient presents with headaches, hypopituitarism and hyperprolactinaemia. (authors)
Full Text Available Soft tissue sarcomas represent less than 1% of all human neoplasms. One-third of malignant tumors that arise in the retroperitoneum are sarcomas and liposarcoma is the most common retroperitoneal sarcoma and is known to grow to giant sizes, slow progress and few late symptoms. We report the case of a 40 year old woman with a giant retroperitoneal liposarcoma. A laparotomy was performed and a multilobulated tumor of 20 centimeters of diameter arising from retroperitoneum. The histological features were suggestive of pleomorphic liposarcoma weighing 8.5 Kilograms
Omar Lopez Arbolay
Full Text Available Intranasal meningoencephaloceles have historically been managed by neurosurgeons, although their main clinical manifestations are rhinological. Recent advances in endoscopic skull base surgery has significantly improved the treatment of these lesions and consequently diminished appreciable surgical morbidity. We report an ethmoidal meningoencephalocele case operated on by endonasal endoscopic approach for removal of the lesion and reconstructing the associated skull base. From this experience, we conclude that removal of the lesion and watertight closure of the skull base irrespective of the size of the mass and anterior skull base defect are the operation’s most important aspects.
Ohara, Nobumasa; Yoneoka, Yuichiro; Seki, Yasuhiro; Akiyama, Katsuhiko; Arita, Masataka; Ohashi, Kazumasa; Suzuki, Kazuo; Takada, Toshinori
Pituitary tumor apoplexy is a rare clinical syndrome caused by acute hemorrhage or infarction in a preexisting pituitary adenoma. It typically manifests as an acute episode of headache, visual disturbance, mental status changes, cranial nerve palsy, and endocrine pituitary dysfunction. However, not all patients present with classical symptoms, so it is pertinent to appreciate the clinical spectrum of pituitary tumor apoplexy presentation. We report an unusual case of a patient with pituitary tumor apoplexy who presented with periorbital edema associated with hypopituitarism. An 83-year-old Japanese man developed acute anterior hypopituitarism; he showed anorexia, fatigue, lethargy, severe bilateral periorbital edema, and mild cardiac dysfunction in the absence of headache, visual disturbance, altered mental status, and cranial nerve palsy. Magnetic resonance imaging showed a 2.5-cm pituitary tumor containing a mixed pattern of solid and liquid components indicating pituitary tumor apoplexy due to hemorrhage in a preexisting pituitary adenoma. Replacement therapy with oral hydrocortisone and levothyroxine relieved his symptoms of central adrenal insufficiency, central hypothyroidism, periorbital edema, and cardiac dysfunction. Common causes of periorbital edema include infections, inflammation, trauma, allergy, kidney or cardiac dysfunction, and endocrine disorders such as primary hypothyroidism. In the present case, the patient's acute central hypothyroidism was probably involved in the development of both periorbital edema and cardiac dysfunction. The present case highlights the need for physicians to consider periorbital edema as an unusual predominant manifestation of pituitary tumor apoplexy.
al Rashed, A A; al-Jarallah, A A; Salih, M A; Kolawole, T; al-Jarallah, J
Fourteen children (of Arab ethnic origin) with Sotos syndrome are described. They were referred to King Khalid University Hospital, Riyadh between July 1992 and June 1997. Their phenotypic characteristics were compared with established diagnostic criteria. There was a male:female ratio of 1.3:1 and a high rate of consanguinity (36%) among parents. At birth, 54% were large and about one-third showed increased height and occipitofrontal head circumference (OFHC). The neonatal histories revealed respiratory and feeding problems in 21%, followed later by delayed motor milestones and speech development in 57%. During childhood, weight, height and OFHC increased further to > 97th centile in 71%, 71% and 93%, respectively. A seizure disorder affected 43%, and 75% had mental retardation (IQ < 70). A non-specific EEG abnormality was found in half of those with seizures. Cranial CT/MRI showed ventricular dilatation in 15% and one patient had corpus callosum dysgenesis. Abdominal ultrasound revealed hydronephrosis in two patients. Radiological cephalometric measurements showed relative prognathism in cases of Sotos syndrome compared with controls (p = 0.003). The study highlights the importance of considering Sotos syndrome in children who present with psychomotor delay.
Full Text Available Kallmann syndrome (KS is a form of hypogonadotropic hypogonadism in combination with a defect in sense of smell, due to abnormal migration of gonadotropin-releasing hormone-producing neurons. We report a case of a 17-year-old Tunisian male who presented with eunuchoid body proportions, absence of facial, axillary and pubic hair, micropenis and surgically corrected cryptorchidism. Associated findings included anosmia. Karyotype was 46XY and hormonal measurement hypogonadotropic hypogonadism. MRI of the brain showed bilateral agenesis of the olfactory bulbs and 3.5 mm pituitary microadenoma. Hormonal assays showed no evidence of pituitary hypersecretion.
Full Text Available Hypopituitarism is a disorder caused by a reduced level of trophic hormones that may be consequent on different destructive processes. The clinical manifestations depend on the type of hormone involved. A deficiency of growth hormone (GH in children causes the lack of growth known as pituitary dwarfism. The case is reported of a patient with pituitary dwarfism, multiple dental anomalies, functional prosthetic problems, and a revision of the literature. She was subjected to prosthetic rehabilitation without surgical intervention, using zirconium substructures, thus eliminating the potential complications that may require trauma surgery. The therapeutic approach adopted led to excellent results and restored an aesthetic smile.
Liao, D Y; Liu, Z Y; Zhang, J; Ren, Q Q; Liu, X Y; Xu, J G
Objective: To investigate the effect of the second-stage transcranial and transsphenoidal approach for giant pituitary tumors. Methods: A retrospective review of 21 patients, who had undergone the transcranial surgery and then transsphenoidal surgery for giant pituitary adenomas from 2012 to 2015 in the neurosurgery department of West China Hospital, was performed. Visual findings, endocrine presentation, complications, and tumor types were collected. All data were based on clinical feature, MRI, and follow-up. Results: Among the 21 cases, gross total resection of tumor was achieved in 7 of all patients, subtotal in 11, and partial in 3. No intracranial hemorrhage or death occurred postoperatively. Postoperative infectionoccurred in one patient and cerebrospinal fluid leakage occurred in 3 patients. Four patients recovered after treatment. Conclusion: According to the clinical feature and MRI, it is safe and effective to choose the transcranial surgery and then transsphenoidal surgery for specific giant pituitary adenomas, which can improve treatment effects and reduce postoperative complications.
Rafael Pinilla González
Full Text Available El hipernefroma constituye el 3 % de todos los tumores malignos y es el tumor renal más frecuente del adulto. Se sospechaba la existencia de un carcinoma renal ante la presencia de hematuria, lumbalgia y tumor abdominal palpable. En la actualidad, el empleo sistemático de la ultrasonografía y la tomografía axial computarizada ha posibilitado la detección de masas renales incidentales y mejorado el pronóstico de estos pacientes. Se describe el caso de un paciente del sexo masculino, de 45 años de edad, con dolor en el lado izquierdo del abdomen y tumoración de crecimiento rápido, que se hizo muy dolorosa en los dos días previos a la operación. Por incremento notable del dolor y por no tener la posibilidad de realizar estudios imaginológicos, se decide operar de urgencia. Se diagnosticó un hipernefroma izquierdo con sangrado intratumoral y se realizó nefrectomía izquierda. El informe histológico confirmó un carcinoma de células renales del tipo de células claras. El paciente fue valorado antes del alta por el oncólogo, quien propuso seguimiento solamenteThe hypernephroma accounts for 3 % of all the malignant tumors and it is the most frequent renal tumor in adults. It was suspected the existence of a renal carcinoma in the presence of haematuria, low back pain and palpable abdominal tumor. Nowadays, the systematic use of ultrasonography and computerized axial tomography has made possible the detection of incidental renal masses, and has improved the prognosis of these patients. The case of a 45-year-old patient with pain on the left side of the abdomen and tumor of fast growth that became very painful 2 days before the operation was described. Due to a marked increase of pain and to the impossibility of carrying out imaging studies, it was decided to perform emergency surgery. A left hypernephroma with intratumoral bleeding was diagnosed and left nephrectomy was performed. The histological report confirmed a clear-cell renal
van Breda, E.; Keizer, H.A.; Kuipers, H.; Wolffenbuttel, B.H.R.
The data of the present case demonstrate that the abuse of androgenic anabolic steroids (AAS) may lead to serious health effects. Although most clinical attention is usually directed towards peripheral side effects, the most serious central side effect, hypothalamic-pituitary-dysfunction, is often
Pressman, Barry D
Modern pituitary imaging is MRI. However, computed tomography (CT) still has limited usefulness. In addition, because CT offers much better bone detail and calcium detection, there are some cases in which such additional information is necessary. Before the advent of CT, plain radiography, pneumoencephalography, and angiography were used to diagnose pituitary masses. More recently, CT, and then especially MRI, made it possible to primarily delineate lesions within and around the pituitary gland rather than depend on secondary information that could only suggest their presence. Copyright © 2017 Elsevier Inc. All rights reserved.
Full Text Available We present an unusual case of a metastatic mantle cell lymphoma (MCL to the pituitary gland. The patient had a known history of MCL for which she previously received chemotherapy. She presented with new-onset diplopia and confusion, and reported a history of progressive vision blurriness associated with headache, nausea, and vomiting. MRI of the brain showed an enhancing lesion within the sella turcica involving the cavernous sinuses bilaterally, extending into Meckel's cave on the left, and abutting the optic nerves bilaterally. Following surgical excision, histopathology revealed the tumor to be a MCL. Metastatic pituitary tumors are rare and have been estimated to make up 1% of tumors discovered in the sellar region. The two most common secondary metastatic lesions to the sella are breast and lung carcinoma followed by prostate, renal cell, and gastrointestinal carcinoma. Metastatic lymphoma to the pituitary gland is especially rare and is estimated to constitute 0.5% of all metastatic tumors to the sella turcica. To our knowledge, this is the first reported case of MCL metastasizing to the pituitary gland.
Full Text Available Pituitary adenomas are rare tumours of pediatric population. In etiology, genetic factors are more common than they are in adults. Because of the rarity of the cases, there are only a few large case studies in the literature. Pituitary tumours in children are often related with syndromes like MEN type 1, Carney Complex and Mc Cune Albright, but there is no case in the literature associated with Cri Du Chat syndrome. Statisticlly, it has been reported that, pediatric tumours occur more often in twins, in the pediatric population. Main treatment for prolactinomas is medical intervention with dopamine agonists, as in adults. Surgery is prefered when the tumour is resistant to medical treatment or shows mass effects around sellae. In that situation, as in adults, both transcranial and transsphenoidal approach is possible.
Olyaei, Yasamin; Sarmiento, J Manuel; Bannykh, Serguei I; Drazin, Doniel; Naruse, Robert T; King, Wesley
Rubinstein-Taybi Syndrome (RSTS) is an autosomal dominant disorder that is classically characterized by prenatal and postnatal growth restriction, microcephaly, dysmorphic craniofacial features, broad thumbs and toes, and intellectual disability. We describe the first reported case of a pituitary macroadenoma associated with RSTS. A 39-year-old Caucasian female with a past medical history of RSTS diagnosed at age two was found to have a gadolinium-enhancing pituitary mass on magnetic resonance imaging (MRI) of the brain three years ago during workup for migraine-like headaches. Subsequent serial imaging showed radiographic evidence of growth up to 11.5 x 14.0 x 10.0 mm in size. The pituitary sellar lesion was resected through an endoscopic transnasal transsphenoidal approach and was found to be a thyrotroph adenoma. RSTS is a rare, neurodevelopmental genetic disease where most patients with disabilities survive into adulthood. The disorder is associated with an increased predisposition for development of nervous system tumors, including pituitary adenomas.
Fang Wei; Zheng Zhaohua; Liao Zuyuan; Hu Yinsong
Objective: To analyze CT manifestation of retroperitoneal gigantic liposaxcoma and to improve the image understanding. Methods: Five cases of retroperitoneal gigantic liposarcoma confirmed by surgery and pathology in our hospital were collected. Plain and enhanced CT scan were performed. Results: Of five cases, one was substantive, two was pseudocyst and two was mixed tumor. Several patterns of enhancement such as strip, floccule, irregular patchy or nodular enhancement were revealed inside the lesions on enhanced CT scan. And strip-form of high density enhancement was a typical type. Conclusion: CT examination could determine the tumor's location, size and density, together with the relationship to adjacent organizations CT scan is an important method in diagnosing retroperitoneal gigantic liposarcoma. (authors)
Ram, Nanik; Ali, Syed Ahsan; Hussain, Syed Zubair
Pituitary stalk interruption syndrome is a rare congenital abnormality of the pituitary that is responsible for anterior pituitary deficiency. It is characterized by a classic triad of interrupted pituitary stalk, absent or ectopic posterior pituitary, and anterior pituitary hypoplasia or aplasia. Clinical presentation varies according to age. In adults it presents as short stature and anterior pituitary deficiency. Without early diagnosis and treatment, mortality and morbidity in these patients is high. Early diagnosis and treatment of this rare disease can prevent permanent short statue of the patient. We report the first case of pituitary stalk interruption syndrome from Pakistan. A 17-year-old Pakistani young man presented with short stature and underdeveloped secondary sexual characters. His siblings and parents were healthy, with normal height. An examination showed his blood pressure was 90/60 mmHg, and his height, weight, and body mass index were 142 cm, 34.5 kg, and 17.10 kg/m2, respectively. He had no hair growth on his face, axilla, or pubis. His testes were between 1 and 2 mL in size, with a 4 cm-at-stretch micropenis. His lab investigations showed that his thyroid stimulating hormone (TSH) was 8.58 uIU/mL (0.4 to 4.2), his free thyroid hormone level FT4 was 0.46 ng/dL (0.89 to 1.76), his prolactin was 21.1 ng/mL (3.0 to 14.7), and his baseline cortisol was 0.30 ug/dL (4.3 to 22.4). His cortisol level after 60 minutes of cosyntropin injection was 3.5 ug/dL (4.3 to 22.4), his insulin like growth factor IGF-1 was 31.56 ng/mL (247.3 to 481.7), his testosterone level was under 2.5 ng/dL (2 to 800), his follicle stimulating hormone FSH was 0.41 uIU/mL (0.0 to 10.0), and his leutinizing hormone LH was under 0.1 uIU/mL (1.2 to 7.8). His bone age was 10 years according to the Greulich and Pyle method, as shown by X-rays. The results from his pituitary magnetic resonance imaging scan were consistent with pituitary stalk interruption syndrome. We describe a young
Vermeij, Geerat J
Gigantism-very large body size-is an ecologically important trait associated with competitive superiority. Although it has been studied in particular cases, the general conditions for the evolution and maintenance of gigantism remain obscure. I compiled sizes and dates for the largest species in 3 terrestrial and 7 marine trophic and habitat categories of animals from throughout the Phanerozoic. The largest species (global giants) in all categories are of post-Paleozoic age. Gigantism at this level appeared tens to hundreds of millions of years after mass extinctions and long after the origins of clades in which it evolved. Marine gigantism correlates with high planktic or seafloor productivity, but on land the correspondence between productivity and gigantism is weak at best. All global giants are aerobically active animals, not gentle giants with low metabolic demands. Oxygen concentration in the atmosphere correlates with gigantism in the Paleozoic but not thereafter, likely because of the elaboration of efficient gas-exchange systems in clades containing giants. Although temperature and habitat size are important in the evolution of very large size in some cases, the most important (and rare) enabling circumstance is a highly developed ecological infrastructure in which essential resources are abundant and effectively recycled and reused, permitting activity levels to increase and setting the stage for gigantic animals to evolve. Gigantism as a hallmark of competitive superiority appears to have lost its luster on land after the Mesozoic in favor of alternative means of achieving dominance, especially including social organization and coordinated food-gathering.
Full Text Available Post-traumatic pituitary apoplexy is uncommon, most of which present with a sudden onset of severe headache and visual impairments associated with a dumbbell-shaped pituitary tumor. We experienced an unusual case of post-traumatic pituitary apoplexy with atypical clinical features. A 66-year-old man presented with mild cerebral contusion and an incidentally diagnosed intrasellar tumor after a fall accident with no loss of consciousness. The patients denied any symptoms before the accident. After 4 days, the left oculomotor nerve palsy developed and deteriorated associated with no severe headache. Repeated neuroimages suggested that pituitary apoplexy had occurred at admission and showed that the tumor compressed the left cavernous sinus. The patient underwent endonasal transsphenoidal surgery at 6 days after head injury, and the mass reduction improved the oculomotor nerve palsy completely within the following 14 days. The pathologic diagnosis was nonfunctioning pituitary adenoma with hemorrhage and necrosis.
Hosoda, Atsuhide; Mizunoya, Satoshi; Abe, Hideki; Kanai, Hidehito; Ikeda, Kazutoshi; Kidahashi, Hisaharu; Suzuki, Masanobu
The purpose of this study was to report a case of optic neuritis after gamma knife radiosurgery for pituitary adenoma. A 41-year-old woman presented with impaired vision in both eyes since 5 days before. She had received surgery for pituitary tumor 5 years before. She was treated by gamma knife radiosurgery for relapse of tumor 50 days before. Her corrected visual acuity was 0.5 right and 0.6 left. She had abnormal color sense. Flicker fusion frequency was decreased in both eyes. Both eyes showed enlarged blind spot and relative scotoma in the superior sector. Magnetic resonance imaging (MRI) showed enhanced signal in the optic nerve sheath. These findings led to the diagnosis of optic neuritis. Pulsed corticosteroid therapy was followed by improved vision of 1.2 in either eye. She has been doing well for 18 months until present. This case illustrates that optic neuritis may develop after gamma knife radiosurgery. (author)
Nishino, Kimihiro; Niwa, Yuri; Mizutani, Teruyuki; Shimizu, Ken; Hayashi, Kazumasa; Chaya, Jyunya; Kato, Noriko; Yamamuro, Osamu
We present the case of a 44-year-old nulliparous woman who experienced irregular menstrual cycles for about 10 years and developed both pituitary prolactinoma and endometrioid endometrial carcinoma. In premenopausal women, hyperprolactinemia causes hypogonadism by inhibiting secretion of gonadotropin-releasing hormone and thus suppressing luteinizing hormone levels, which can cause menstrual disorders ranging from amenorrhea, oligomenorrhea and chronic anovulatory cycle to short luteal phase of the menstrual cycle. A chronic anovulatory menstrual cycle is the most common cause of long-term exposure of the endometrium to endogenous estrogen without adequate opposition from progestins, which can lead to endometrioid endometrial carcinoma. In this case, pituitary prolactinoma may have caused the chronic anovulatory cycle and indirectly led to the endometrioid endometrial carcinoma. In patients for whom the cause of irregular menstruation and chronic anovulatory cycle is suspected to be hyperprolactinemia, explorations of both the hypophysis and endometrium are essential.
Full Text Available We present the case of a 44-year-old nulliparous woman who experienced irregular menstrual cycles for about 10 years and developed both pituitary prolactinoma and endometrioid endometrial carcinoma. In premenopausal women, hyperprolactinemia causes hypogonadism by inhibiting secretion of gonadotropin-releasing hormone and thus suppressing luteinizing hormone levels, which can cause menstrual disorders ranging from amenorrhea, oligomenorrhea and chronic anovulatory cycle to short luteal phase of the menstrual cycle. A chronic anovulatory menstrual cycle is the most common cause of long-term exposure of the endometrium to endogenous estrogen without adequate opposition from progestins, which can lead to endometrioid endometrial carcinoma. In this case, pituitary prolactinoma may have caused the chronic anovulatory cycle and indirectly led to the endometrioid endometrial carcinoma. In patients for whom the cause of irregular menstruation and chronic anovulatory cycle is suspected to be hyperprolactinemia, explorations of both the hypophysis and endometrium are essential.
Rajendran, Rajesh; Naik, Sarita; Sandeman, Derek D; Nasruddin, Azraai B
We report the use of pasireotide in a rare and unusual case of pituitary macroadenoma co-secreting GH, prolactin and ACTH. A 62-year-old Caucasian man presented with impotence. Clinically, he appeared acromegalic and subsequent investigations confirmed GH excess and hyperprolactinaemia. Magnetic resonance imaging (MRI) of pituitary revealed a large pituitary macroadenoma. He underwent trans-sphenoidal surgery and histology confirmed an adenoma with immunohistochemistry positive for ACTH, GH and prolactin. Acromegaly was not cured following surgery and inadequately controlled despite subsequent octreotide therapy. He underwent further debulking pituitary surgery, following which IGF1 levels improved but still high. This time adenoma cells showed immunohistochemistry positivity for ACTH only, following which subsequent investigations confirmed intermittent hypercortisolaemia compatible with pituitary Cushing's disease. We recommended radiotherapy, but in view of the pluripotential nature of the tumour, we proceeded with a trial of s.c. pasireotide therapy on the basis that it may control both his acromegaly and Cushing's disease. After 3 months of pasireotide therapy, his mean GH and IGF1 levels improved significantly, with improvement in his symptoms but intermittent hypercortisolaemia persists. His glycaemic control deteriorated requiring addition of new anti-diabetic medication. MRI imaging showed loss of contrast uptake within the tumour following pasireotide therapy but no change in size. We conclude that our patient has had a partial response to pasireotide therapy. Long-term follow-up studies are needed to establish its safety and efficacy in patients with acromegaly and/or Cushing's disease. Plurihormonal pituitary adenomas are rare and unusual.Patients with pituitary adenomas co-secreting ACTH and GH are more likely to present with acromegaly because GH excess can mask hypercortisolaemia.Pasireotide holds potential where conventional somatostatin analogues
Mahdieh Rezaei; Sara Rostami; Mehdi Saberi; Dariush Vosugh
We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old, female, terrier dog. The animal was admitted due to polyphagia, weight gain, polyuria, polydipsia, hair loss, exercise intolerance and panting at rest. On physical examination, abdominal distention, truncal and bilaterally symmetric alopecia, thin hypotonic skin, comedones, bruising, hyperpigmentation and calcinosis cutis on the dorsal midline were observed. Hematologic investigations showed stress l...
Zonana, J; Sotos, J F; Romshe, C A; Fisher, D A; Elders, M J; Rimoin, D L
Cerebral gigantism is a syndrome consisting of characteristic dysmorphic features, accelerated growth in early childhood, and variable degrees of mental retardation. Its etiology and pathogenesis have not been defined. Three families are presented with multiple affected members. The vertical transmission of the trait and equal expression in both sexes in these families indicates a genetic etiology with a dominant pattern of inheritance, probably autosomal. As in previously reported cases, extensive endocrine evaluation failed to define the pathogenesis of the accelerated growth present in this disorder.
Ana Julia Balau
Full Text Available O objetivo do trabalho é descrever o uso de interferon alfa no tratamento de pacientes com hemangioma gigante. Os autores relatam e analisam dois casos de hemangioma gigante em tratamento com interferon alfa. IBS, 3 anos, em acompanhamento no Ambulatório de Hematologia desde um ano de idade com quadro de lesão angiomatosa em praticamente toda hemiface direita, acompanhada de sangramentos gengivais importantes. Após a realização de exames complementares (Angiorressonância magnética e feito o diagnóstico de hemangioma gigante em face, foi iniciado tratamento com prednisona e, posteriormente, associação com interferon alfa e observada importante melhora do quadro, resultando na diminuição dos episódios de sangramento e no tamanho do tumor. C.N.P., 12 anos, apresentando nódulo em região lateral de joelho esquerdo há 2 anos, com aumento progressivo do tamanho e dor local. Fez uso de prednisona e, sem melhora do quadro, introduzido interferon alfa com regressão importante do tamanho do tumor. O tratamento com interferon alfa deve ser considerado no tratamento de hemangiomas, pois apresenta bons resultados em relação à diminuição do tamanho do tumor e, conseqüentemente, reduz as intercorrências clínicas associadas à sua presença, principalmente os sangramentos.The aim of this study is to describe the treatment using interferon-alpha of giant hemangiomas in children. The authors report two cases of children presenting with giant hemangiomas treated using interferon-alpha and analyze the results. IBS, 3 years-old, has been followed up in Famema Hemathology Service since she was 1 year-old with a tumor on the face and persistent bleeding. After clinical and radiologic evaluations and suggested the diagnosis of giant hemangioma, she started treatment with interferon-alpha. A great clinical improvement was observed a reducing of the number of episodes of bleedings and a decrease in of the tumor size. CNP, 12 years-old, came to
Full Text Available Introduction: Traumatic brain injury (TBI is one of the main causes of morbidity and mortality in young trauma patients with resultant multi-organ effects. Hypopituitarism following TBI can be debilitating and life threatening. TBI which causes hypopituitarism may be characterized by a single head injury, such as from a motor vehicle accident, or by chronic repetitive head trauma, as seen in combative supports including boxing, kick-boxing, and football. In the majority of cases, a diagnosis of hypopituitarism can be entirely missed resulting in severe neuro-endocrine dysfunction. We present a case series of two patients diagnosed with hypopituitarism after TBI and treated appropriately with favorable outcome. Case presentations: The first case is a 34 year-old male, who presented to the emergency department with blunt head trauma after a motor vehicle accident while riding his bicycle. He suffered from severe cranio-facial injuries, resulting in multifocal hemorrhagic contusions, epidural hematoma, and extensive cranio-facial fractures involving the sinuses. The patient developed persistent hypotension with a blood pressure as low as 60/40 mmHg on hospital day three.The second case is a 56 year-old male with a history of schizophrenia, who suffered traumatic brain injury after he was hit by a train. The patient sustained multiple facial fractures, pneumocephalus and C2/7 transverse processes fractures. He also had persistent hypotension, unresponsive to standard treatment. Investigation revealed a deficiency of anterior pituitary hormones resulting from pituitary axis disruption. Discussion: Hypopituitarism is becoming an increasingly recognized complication following TBI, ranging from total to isolated deficiencies. Traumatic Brain Injury is a major public health problem and is one of the leading causes of disability. Understanding and recognizing pituitary dysfunction after TBI can lead to better outcomes and improved quality of life
Full Text Available We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old, female, terrier dog. The animal was admitted due to polyphagia, weight gain, polyuria, polydipsia, hair loss, exercise intolerance and panting at rest. On physical examination, abdominal distention, truncal and bilaterally symmetric alopecia, thin hypotonic skin, comedones, bruising, hyperpigmentation and calcinosis cutis on the dorsal midline were observed. Hematologic investigations showed stress leukogram, high serum alkaline phosphatase activity, mild to moderate alanine aminotransferase activity, hypercholesterolemia, hypertriglyceridemia and hyperglycemia. Mild generalized interstitial lung patterns and hepatomegaly were detected in the radiographs. Bilaterally symmetric normal-sized adrenal glands were also diagnosed in ultrasonography. Diagnosis of pituitary-dependent hyperadrenocorticism was confirmed with low-dose dexamethasone suppression test. The dog was successfully treated with mitotane.
Fujio, Shingo; Ashari; Habu, Mika; Yamahata, Hitoshi; Moinuddin, F M; Bohara, Manoj; Arimura, Hiroshi; Nishijima, Yui; Arita, Kazunori
Thyroid stimulating hormone-secreting pituitary adenomas (TSHomas) are uncommon tumors of the anterior pituitary gland. Patients with TSHomas may present with hyperthyroidism, but the incidence of thyroid storm due to TSHomas has yet to be determined. We report a rare case of thyroid storm caused by TSHoma in a 54-year-old woman. Preoperatively she had symptoms of excessive sweating and palpitation. Blood tests showed inappropriate secretion of TSH with blood TSH 6.86 μ U/mL, fT3 19.8 pg/mL, and fT4 5.95 ng/dL. Magnetic resonance imaging (MRI) revealed a pituitary tumor with maximum diameter of 13 mm that was extirpated through transsphenoidal route. After operation the patient was stuporous and thyroid storm occurred presenting with hyperthermia, hypertension, and tachycardia. It was well managed with nicardipine, midazolam, steroids, and potassium iodide. Immunohistochemical staining of tumor specimen was positive for TSH and growth hormone (GH). One year after operation, fT3 and fT4 levels were still high. As her tumor was diagnosed to be GH- and TSH-producing adenoma, octreotide injection therapy was started, which normalized thyroid hormone levels. This is the second reported case with thyroid storm due to TSHoma and emphasizes the importance of strategies with interdisciplinary cooperation for prevention of such emergency conditions.
Sabbioni, Giacomo; Rani, Nicola; Devescovi, Valentina
The case of a 3-year-old child diagnosed with Type 1 neurofibromatosis is presented, showing pigmented birthmarks and gigantism of the left lower limb associated with the presence of multiple neurofibromas. Increased bone growth appears to be the direct or indirect consequence of a still undefined paracrine effect of nerve tumor cells.
Donnelly, Deirdre E; Morrison, Patrick J
The biblical giant Goliath has an identifiable family tree suggestive of autosomal dominant inheritance. We suggest that he had a hereditary pituitary disorder possibly due to the AIP gene, causing early onset and familial acromegaly or gigantism. We comment on the evidence within the scriptures for his other relatives including a relative with six digits and speculate on possible causes of the six digits. Recognition of a hereditary pituitary disorder in the biblical Goliath and his family sheds additional information on his and other family members' battles with David and his relatives.
Kumabe, Ayako; Kenzaka, Tsuneaki; Nishimura, Yoshioki; Aikawa, Masaki; Mori, Masaki; Matsumura, Masami
Background Anasarca in patients with lymphoma is a rare symptom. We report a patient with DLBCL associated with pituitary gland infiltration that was diagnosed based on significant anasarca. Case presentation A 72-year-old woman with a 10-year history of hypertension visited a local hospital presenting with anasarca and 15-kg weight gain in the past 3?months. we clinically diagnosed central hypothyroidism caused by pituitary gland infiltration of diffuse large B-cell lymphoma (DLBCL) (clinica...
Constantine L. Karras
Full Text Available Background. Purely ectopic pituitary adenomas are exceedingly rare. Here we report on a patient that presented with an incidental clival mass thought to be a chordoma. Endonasal resection, tumor pathology, and endocrinology workup revealed a prolactinoma. Case Presentation. A 41-year-old male presented with an incidental clival lesion presumed to be a chordoma. On MRI it involved the entire clivus, extended laterally to the petroclival junction, and invaded the cavernous sinuses bilaterally, encasing both internal carotid arteries, without direct extension into the sella. Intraoperatively, it was clear that the tumor originated from the clivus and that the sellar dura was completely intact. Frozen-section pathology was consistent with a pituitary adenoma. Immunostaining was positive for synaptophysin and prolactin with a low Ki-67 index, suggestive of a prolactinoma. Additional immunohistochemical stains seen in chordomas (EMA, S100, and Brachyury and other metastatic tumors were negative. A postoperative endocrine workup revealed an elevated serum prolactin of 881.3 ng/mL (normal < 20. Conclusions. In conclusion, it is crucial to maintain an extensive differential diagnosis when evaluating a patient with a clival lesion. Ectopic clival pituitary adenomas, although rare, may warrant an endocrinological workup preoperatively as the majority may respond to medical treatment.
Yoshii, Masaru; Okisaka, Shigekuni; Yanagawa, Youichi; Takiguchi, Hiroshi
A case of optic neuropathy after postoperative radiation therapy is reported. A 69-year-old woman had a partial resection of a pituitary adenoma in 1990 and was treated with 45 Gy of irradiation to the postoperative pituitary lesion for one month. Seven months later she had sudden right visual field loss. Goldmann perimetry examination revealed remarked visual field defect in her right eye with visual acuity of 1.0. The right relative afferent pupillary defect was positive. The value of critical flicker fusion for her right eye was reduced and the amplitude of steady-state pattern-reversal visually evoked cortical potential was significantly less for the right monocular stimulation than that for the fellow eye stimulation, but Ganzfeld electroretinograms were normal for both eyes. Magnetic resonance imaging using Gadolinium-diethylene triaminepenta-acetic acid revealed enhancement on the right optic nerve, which had not been recognized immediately after the radiation therapy, without any suggestion of right optic nerve compression by the residual pituitary adenoma. (author)
Yoshii, Masaru; Okisaka, Shigekuni; Yanagawa, Youichi; Takiguchi, Hiroshi [National Defense Medical Coll., Tokorozawa, Saitama (Japan)
A case of optic neuropathy after postoperative radiation therapy is reported. A 69-year-old woman had a partial resection of a pituitary adenoma in 1990 and was treated with 45 Gy of irradiation to the postoperative pituitary lesion for one month. Seven months later she had sudden right visual field loss. Goldmann perimetry examination revealed remarked visual field defect in her right eye with visual acuity of 1.0. The right relative afferent pupillary defect was positive. The value of critical flicker fusion for her right eye was reduced and the amplitude of steady-state pattern-reversal visually evoked cortical potential was significantly less for the right monocular stimulation than that for the fellow eye stimulation, but Ganzfeld electroretinograms were normal for both eyes. Magnetic resonance imaging using Gadolinium-diethylene triaminepenta-acetic acid revealed enhancement on the right optic nerve, which had not been recognized immediately after the radiation therapy, without any suggestion of right optic nerve compression by the residual pituitary adenoma. (author).
Li, Jiaqi; Li, Jianwei; Jiang, Shu; Yu, Ruichao; Yu, Yerong
Thyrotropin-secreting adenoma (TSHoma) is rare, diagnosis and treatment are often delayed if the condition coexists with Hashimoto thyroiditis. The enlarged pituitary adenoma may eventually induce panhypopituitarism, infertility, or the compression of optic nerves and optic chiasma. This patient was a 36-year-old man who had been referred to the pituitary disease multidisciplinary team (MDT) of the West China Hospital, due to infertility. Examinations revealed pituitary thyrotropin-secreting macroadenoma. We conducted trans-sphenoidal surgery. Human chorionic gonadotropin (HCG) and human menopausal gonadotropin (HMG) were used for reproductive reconstruction after surgery. This patient successfully fathered a child. To date, the multidisciplinary team treatment of TSHoma was rare, TSHomas are often misdiagnosed as macroadenomas, because the clinical features are varied and it often takes a long time to be diagnosed. So the purpose of this case report is to attract attention to the manifestation of increased thyroid stimulating hormone (TSH) concentration and discuss MDT treatment for TSH-secreting adenoma. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
Trivellin, Giampaolo; Hernández-Ramírez, Laura C; Swan, Jeremy; Stratakis, Constantine A
X-linked acrogigantism (X-LAG) is a recently described form of familial or sporadic pituitary gigantism characterized by very early onset GH and IGF-1 excess, accelerated growth velocity, gigantism and/or acromegaloid features. Germline or somatic microduplications of the Xq26.3 chromosomal region, invariably involving the GPR101 gene, constitute the genetic defect leading to X-LAG. GPR101 encodes a class A G protein-coupled receptor that activates the 3',5'-cyclic adenosine monophosphate signaling pathway. Highly expressed in the central nervous system, the main physiological function and ligand of GPR101 remain unknown, but it seems to play a role in the normal development of the GHRH-GH axis. Early recognition of X-LAG cases is imperative because these patients require clinical management that differs from that of other patients with acromegaly or gigantism. Medical treatment with pegvisomant seems to be the best approach, since X-LAG tumors are resistant to the treatment with somatostatin analogues and dopamine agonists; surgical cure requires near-total hypophysectomy. Currently, the efforts of our research focus on the identification of GPR101 ligands; in addition, the long-term follow-up of X-LAG patients is of extreme interest as this is expected to lead to better understanding of GPR101 effects on human pathophysiology. Copyright © 2018. Published by Elsevier Ltd.
Joo Hee Lim
Full Text Available A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma. To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS was performed by desmopressin use to stimulate ACTH. Finally, the patient was diagnosed with Cushing disease due to ACTH-secreting pituitary microadenoma, lateralized to the left side; subsequently underwent transsphenoidal surgery. Here we report a case of a 14-year-old girl diagnosed with Cushing disease with a pituitary tumor lateralized by IPSS using desmopressin, which is very rare in pediatric Cushing disease.
Lim, Joo Hee; Kim, Soo Jung; Jung, Mo Kyung; Kim, Ki Eun; Kwon, Ah Reum; Chae, Hyun Wook; Kim, Duk Hee; Kim, Ho-Seong
A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH) level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma. To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS) was performed by desmopressin use to stimulate ACTH. Finally, the patient was diagnosed with Cushing disease due to ACTH-secreting pituitary microadenoma, lateralized to the left side; subsequently underwent transsphenoidal surgery. Here we report a case of a 14-year-old girl diagnosed with Cushing disease with a pituitary tumor lateralized by IPSS using desmopressin, which is very rare in pediatric Cushing disease.
What is the real nature of pulsars? This is essentially a question of the fundamental strong interaction between quarks at low-energy scale and hence of the non-perturbative quantum chromo-dynamics, the solution of which would certainly be meaningful for us to understand one of the seven millennium prize problems (i.e., "Yang-Mills Theory") named by the Clay Mathematical Institute. After a historical note, it is argued here that a pulsar is very similar to an extremely big nucleus, but is a little bit different from the gigantic nucleus speculated 80 years ago by L. Landau. The paper demonstrates the similarity between pulsars and gigantic nuclei from both points of view: the different manifestations of compact stars and the general behavior of the strong interaction. (author)
Full Text Available Abstract Introduction Differentiation of cystic mass lesions of the sellar and parasellar regions may pose a diagnostic dilemma for physicians, neurosurgeons, radiologists and pathologists involved in treating patients with these entities. A considerable number of tumors previously identified as craniopharyngiomas may, in fact, have been xanthogranulomas. We report a case of pituitary dysfunction caused by xanthogranuloma of the intrasellar region. Case presentation A 47-year-old man of Japanese descent presented to our institution with a tumor located exclusively in the intrasellar region which manifested as severe hypopituitarism. MRI revealed a clearly defined intrasellar mass that was heterogeneously hyperintense on T1-weighted images and markedly hypointense on T2-weighted images. We preoperatively diagnosed the patient with Rathke's cleft cyst or non-functioning pituitary adenoma. Although the tumor was completely removed using a transsphenoidal approach, the improvement of the patient's endocrine function was marginal, and continued endocrine replacement therapy was needed. Postoperatively, a histological examination revealed the tumor to be a xanthogranuloma of the intrasellar region. His visual field defects and headache improved. Conclusion Because diagnosis depends on surgical intervention and xanthogranulomas of the intrasellar region are very rare, the natural history of xanthogranuloma is still unknown. Therefore, this entity is difficult to diagnose preoperatively. We suggest that xanthogranuloma should be included in the differential diagnosis, even in the case of sellar lesions, to formulate appropriate postoperative management and improve endocrine outcomes.
Nishi, Tohru; Matsukado, Yasuhiko; Kodama, Takafumi; Hiraki, Toshiro
A 57-year-old man was admitted because of visual disturbance due to a recurrent pituitary adenoma. Nine years ago craniotomy was performed for the chromophobe adenoma and postoperative radiation therapy was applied with tumor dose of 50 Gy. Digital subtraction angiography indicated existence of an aneurysm at the carotid bifurcation and the finding was confirmed by conventional angiography, which revealed a saccular aneurysm and irregularity of the carotid walls. In surgery there were not only the saccular aneurysm found in the angiogram, but also three other fusiform aneurysms and severe sclerotic change at the major arteries adjoining the sellar region. Azzarelli et al. reported a fatal case due to the development of arteriosclerotic intracranial fusiform aneurysms following radiation therapy for suprasellar germinoma. This case is the second case which indicates the development of intracranial aneurysm following radiation therapy. Emphasis is placed on careful follow-up examination for radiated pituitary adenoma with computed tomography, digital subtraction angiography, or occasionally conventional angiography, even though the postoperative condition of the primary lesion is stabilized.
Shrestha, Mithila; Raitanen, Jani; Salminen, Tiina; Lahkola, Anna; Auvinen, Anssi
The number of mobile phone users has grown rapidly, which has generated mounting public concern regarding possible health hazards. This study aims to assess pituitary tumor risk, as it has rarely been investigated. A case-control study was conducted with 80 eligible cases identified from all five university hospitals in Finland and frequency-matched 240 controls from the national population register. Controls were matched to cases by age, sex, region of residence and date of interview. A detailed history of mobile phone use was obtained using a structured interview. Several indicators of mobile phone use were assessed using conditional logistic regression. A reduced odds ratio was seen among regular mobile phone users [OR 0.39, 95% confidence interval (CI) 0.21, 0.72] relative to never/non-regular users, possibly reflecting methodological limitations. Pituitary tumor risk was not increased after 10 or more years since first use (OR 0.69, 95% CI 0.25, 1.89). The risk was not increased in relation to duration, cumulative hours of use, or cumulative number of calls. The results were similar for analog and digital phones. We found no excess risk associated with self-reported short- or medium-term use of mobile phones. This is consistent with most of the published studies. However, uncertainties remained for longer duration of use, as a very small proportion of study participants reported use beyond 10 years.
Full Text Available Cerebral Cavernous Malformations (CCMs are vascular lesions involving brain capillaries. They may occur sporadically or be inherited as autosomal dominant character. Due to incomplete penetrance, CCMs incidence is underestimated and, overall sporadic cases, are often accidentally diagnosed. Rarely CCMs are linked to other pathological conditions. Here we present the first case in literature of a young woman affected by sporadic CCM, pituitary adenoma, Hashimoto thyroiditis and mental illness of unknown etiology. Symptoms’ analysis suggests that she may suffer of Hashimoto encephalopathy (HE, a condition that in very few cases develops together with Hashimoto thyroiditis. Genetic bases of HE are still unknown and symptomatology is very heterogeneous. This paper is a preliminary report of the case and is focused on complexity of clinical manifestations that makes the diagnosis uncertain. If our hypothesis will be confirmed by further analysis, our aim will be to clarify genetic causes of HE.
Sasajima, Toshio; Mineura, Katsuyoshi; Kowada, Masayoshi; Sasaki, Junko; Sasajima, Hiroyasu; Sakamoto, Tetsuya
A case of pituitary apoplexy is presented in which a free niveau formation, a pathognomonic sign of this entity, was proved by means of computerized tomography (CT) by turning the head. A 48-year-old female had developed a sudden, excruciating, retroorbital headache, vomiting, and visual disturbance twice prior to admission. The visual acuity was 0.1 in the right eye and 0.6 in the left eye. The optic fundi were normal. There was a right temporal field loss and a left upper temporal quadrantanopsia. A plain skull film disclosed ballooning and a double floor of the turcic sella. A CT scan of the head, performed by means of a GE CT/T 8800 Scanner, showed an intrasellar low-density mass with a slightly enhanced rim. We were not convinced of the presence of a high-density area, though there seemed to be one adjacent to the posterior clinoid process and the turcic floor, because of the partial volume effect and the artifact related to the neighboring bones. Another high-resolution CT scan on the ensuing day, as the head was turned and then kept still at about 45 degrees to the right, while the patient was supine, for ten minutes, made it possible to visualize a free fluid level, comparable to a fluid-blood-density level. A transsphenoidal pituitary exploration identified blood fluid collection in the encapsulated tumor, a finding which was histologically consistent with the sinusoidal type of chromophobe adenoma. There was some microscopic evidence of necrosis and hemosiderin laden cells. The postoperative course was uneventful; visual acuity improved without delay, and the temporal field defect became significantly smaller. Two weeks later, visual acuity had recovered to 1.2 uncorrected in each eye. CT and the pertinent position of the head might be quite helpful for the visualization and confirmation of a subtle free fluid level in cases of pituitary apoplexy. (author)
Hubalewska-Hola, Alicja; Fröss, Katarzyna; Kostecka-Matyja, Marta; Sowa-Staszczak, Anna; Szybiński, Zbigniew; Huszno, Bohdan; Ptak, Marzena
Thyrotropin secreting adenoma, thyrotropinoma (TSH-oma), is a rare cause of hyperthyroidism--called secondary hyperthyroidism. The hormonal profile in pituitary hyperthyroidism is characterized by a nonsuppressed TSH in the presence of high levels of free thyroid hormones (fT4, fT3) reflecting an abnormal feedback. The diagnosis of TSH-oma is often made at the stage of macroadenoma because of the aggressive nature of the tumor and due to the fact that patients are mistakenly treated for more common primary hyperthyroidism for a long time. Two cases of TSH-secreting adenoma were detected in Chair and Department of Endocrinology, Collegium Medicum of the Jagiellonian University in Krakow for the last twenty years. Case 1: 49 year old woman was admitted to the Clinic of Endocrinology in 1999 with recurring hyperthyroidism treated with surgical thyroid ablation in 1992 and thyreostatics for the previous nine years. On admission to the Clinic her thyroid panel presented with elevated free hormone levels (mainly fT3-14.8 pmol/l) and not suppressed TSH-0.7 mIU/l suggesting central hyperthyroidism. MRI scan of the pituitary gland revealed microadenoma of 5 mm in diameter. She was qualified to transsphenoidal resection of the tumor. Histopathology revealed acidophilic adenoma with positive TSH staining. Thyroid hormones 8 days after the operation suggested full effectiveness of the surgery. Case 2: 65 year old man treated for one year with L-Thyroxin because of elevated TSH (60 mIU/l) and then with thyreostatics for elevated fT3 and fT4 was admitted to the Clinic of Endocrinology in 2000 with suspected thyrotropinoma. On admission to the Clinic thyroid panel suggested hyperthyroidism with fT4-40 pmol/l, FT3-11.2 pmol/l without suppression of TSH 2.2 mIU/l. MRI scan revealed a pituitary tumor 20 x 18 x 20 mm, compressing the optic chiasm. He was administered octreotide as a preparation for the operation. The patient underwent trans-sphenoidal resection of the adenoma
Tripathi, S.; Ammini, A.C.; Bhatia, R.; Gupta, R.; Berry, M.; Sarkar, C.; Mahajan, H.
Fourteen patients with adrenocorticotropic hormone (ACTH)-dependent hypercortisolism underwent pituitary scanning with computed axial tomography (CT) and magnetic resonance imaging (MRI). Computed tomography revealed pituitary macroadenomas in two patients, pituitary hyperplasia in one and suspicion of pituitary microadenoma in one. Thirteen patients underwent MRI. One with a macroadenoma diagnosed on CT did not undergo MRI. The MRI revealed a pituitary macroadenoma in one, microadenoma in three and hyperplasia in two cases. Magnetic resonance imaging following gadolinium diethylene triamine penta acetic acid (Gd-DTPA) enhancement revealed four more pituitary microadenomas. All patients who had pituitary adenomas (micro and macro) and hyperplasia underwent trans-sphenoidal pituitary surgery. One of the two patients, who had an enlarged pituitary on imaging but no demonstrable adenoma, was found to have a microadenoma at surgery. It is concluded that patients with ACTH dependent hypercortisolism should undergo MRI of the pituitary gland to identify/localize corticotroph pituitary ademonas. The study should include Gd-DTPA enhancement in cases where the scan is normal. 2 refs., 3 tabs., 3 figs
Mirto N. Prandini
Full Text Available São apresentados dois casos de mucocele gigante do seio frontal submetidos a tratamento cirúrgico. A manifestação clínica foi cefaléia de evolução prolongada, associada com protrusão unilateral do globo ocular de curta duração. Em ambos os casos foi realizada craniotomia frontal com remoção completa da lesão, reparação do soalho frontal com retalho pediculado de gálea e cranialização do seio frontal. No segundo caso, uma abordagem endoscópica intranasal foi combinada à abordagem externa no mesmo ato cirúrgico. Alguns aspectos abordando a etiologia, associação com outras afecções e tratamento cirúrgico são discutidos.Two patients harboring giant frontal mucoceles are reported. In both cases complaints of chronic headaches and progressive unilateral proptosis were preponderant. Surgical treatment included a frontal craniotomy with excision of the lesion, skull base reinforcement with pedicled galea and wide opening of the frontal sinuses. In the second case an intranasal endoscopic approach was combined with craniotomy at the same surgical operative time. Some aspects regarding etiology, association with other diseases and some surgical aspects are discussed.
Drimmie, F M; MacLennan, A C; Nicoll, J A; Simpson, E; McNeill, E; Donaldson, M D
True gigantism is rare in early childhood and is usually due to excess GH secretion from a pituitary adenoma. We report a case in which the endocrine abnormality is secondary to an optic glioma. Careful endocrine evaluation has shown that GH peak amplitude was not increased but rather there was failure of GH levels to suppress to baseline and a lack of pulsatility. There is no evidence of a direct secretory role for the tumour and we postulate that the tumour is affecting GH secretion through an effect on somatostatin tone. Specific tumour therapy is not indicated for this patient in the absence of mass effect or visual disturbance. The GH excess is being treated with somatostatin analogue (Octreotide) and as he has developed precocious puberty he is also receiving long acting GnRH analogue (Zoladex). This boy appears likely to have neurofibromatosis type 1 (NF1) which raises the question of subtle GH excess in NF1 patients with tall stature.
Full Text Available Abstract Introduction Hemangioblastomas are rare, benign tumors occurring in any part of the nervous system. Most are found as sporadic tumors in the cerebellum or spinal cord. However, these neoplasms are also associated with von Hippel-Lindau disease. We report a rare case of a sporadic sellar hemangioblastoma that became symptomatic due to pituitary apoplexy. Case presentation An 80-year-old, otherwise healthy Caucasian woman presented to our facility with severe headache attacks, hypocortisolism and blurred vision. A magnetic resonance imaging scan showed an acute hemorrhage of a known, stable and asymptomatic sellar mass lesion with chiasmatic compression accounting for our patient's acute visual impairment. The tumor was resected by a transnasal, transsphenoidal approach and histological examination revealed a capillary hemangioblastoma (World Health Organization grade I. Our patient recovered well and substitutional therapy was started for panhypopituitarism. A follow-up magnetic resonance imaging scan performed 16 months postoperatively showed good chiasmatic decompression with no tumor recurrence. Conclusions A review of the literature confirmed supratentorial locations of hemangioblastomas to be very unusual, especially within the sellar region. However, intrasellar hemangioblastoma must be considered in the differential diagnosis of pituitary apoplexy.
Slabu, Hannah; Arnason, Terra
Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis that can affect several organs, most commonly the respiratory tract and kidneys. Pituitary involvement is exceptionally rare. Most case reports of GPA of the pituitary gland have been described in middle-aged women who have concomitant ears, nose and throat involvement. The most frequent manifestation is diabetes insipidus due to a preponderance of posterior pituitary infiltration. The majority of cases sustain permanent dama...
A.A. Núñez Serrano
Full Text Available El condiloma gigante del pene o tumor de Buschke- Lowenstein, es un tumor epitelial benigno de origen viral y sexualmente transmisible, que en raros casos puede malignizar. Presentamos un paciente en el que el condiloma de localización peneana, creció rápidamente y destruyó estructuras. Su histología se caracteriza por papilomatosis y acantosis endo y exofítica. Existen diferentes tratamientos del tumor, pero el más efectivo es la extirpación quirúrgica radical para evitar recidivas y malignización.Buschke-Lowenstein tumour is an epithelial benign tumour sexually transmitted with a viral origin. We present a case of peneal localization with exofitic growth, compression and displacement of the deeper tissues, ulceration and urethral fistulae. Histology is characterized by papillomatosis and endo or exophytic acantosis. Local malignancy is still discussed. There are many possible treatments, but radical excision is the best to avoid malignant transformation and recurrences.
Kollias, S.S. [Dept. of Radiology, Section of Pediatric Neuroradiology, and Depts. of Radiology and Pediatrics, Children`s Hospital Medical Center, Cincinnati, OH (United States); Ball, W.S. [Dept. of Radiology, Section of Pediatric Neuroradiology, and Depts. of Radiology and Pediatrics, Children`s Hospital Medical Center, Cincinnati, OH (United States); Prenger, E.C. [Dept. of Radiology, Section of Pediatric Neuroradiology, and Depts. of Radiology and Pediatrics, Children`s Hospital Medical Center, Cincinnati, OH (United States)
We describe the clinical manifestations, associated abnormalities, MRI appearances and pathologic significance of a case of hypophyseal duplication. A 16-year-old girl presented with delayed sexual development and history of midline craniofacial anomalies. MRI revealed paired infundibula extending inferiorly to two small pituitary glands, a midline hypothalamic mass, and a midline cleft in the basisphenoid. Twelve cases of pituitary duplication have previously been described. The suggested pathogenesis is duplication of the prechordal plate and anterior end of the notochord during early embryologic development. (orig.)
Kiatpanabhikul, Phatharaporn; Shuangshoti, Shanop; Chantra, Kraisri; Navicharern, Patpong; Kingpetch, Kanaungnit; Houngngam, Natnicha; Snabboon, Thiti
Co-existence of thyrotropin/growth hormone-secreting pituitary adenoma with differentiated thyroid carcinoma is exceedingly rare, with less than 15 cases having been reported. Its clinical presentation and treatment strategy are challenging. We report a case of pituitary macroadenoma, with clinical syndromes of acromegaly and hyperthyroidism, and a thyroid nodule, with cytologically confirmed to be a papillary thyroid carcinoma. Clinical implications, focusing on the strategy for proper management, and possible pathogenesis were discussed. Copyright © 2017 Elsevier Ltd. All rights reserved.
Manjila, Sunil; Miller, Erin A; Vadera, Sumeet; Goel, Rishi K; Khan, Fahd R; Crowe, Carol; Geertman, Robert T
Duplication of the pituitary gland (DPG) is a rare craniofacial developmental anomaly occurring during blastogenesis with postulated etiology such as incomplete twinning, teratogens, median cleft face syndrome or splitting of the notochord. The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case. We report for the first time in the medical literature some unique associations with DPG, such as a clival encephalocele, third cerebral peduncle, duplicate odontoid process and a double tongue with independent volitional control. This patient also has the previously reported common associations such as duplicated sella, cleft palate, hypertelorism, callosal agenesis, hypothalamic enlargement, nasopharyngeal teratoma, fenestrated basilar artery and supernumerary teeth. This study also reviews 37 cases of DPG identified through MEDLINE literature search from 1880 to 2011. It provides a detailed analysis of the current case through physical examination and imaging. The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations. Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.
Ng C. S.
Full Text Available Central nervous system injury in particular cranial nerve palsy has been reported to be as high as 2%. Such prevalence of palsy generally attributed to surgical manipulation at the cavernous sinus, especially incurring the abducens nerve. We report the first case of acute oculomotor nerve sequel to the release of cystic fluid wrapping the nerve following a transsphenoidal excision of pituitary macroadenoma in a 57-year-old woman. She attended with the presentation of acute excruciating headache associated with partial drooping of right eye. The computed tomography and magnetic resonance imaging (MRI were consistent with pituitary apoplexy of an underlying pituitary macroadenoma. Urgent transsphenoidal hypophysectomy was done. Intra-operatively, cystic fluid was aspirated during pituitary tumour dissection. At the same time, curettage was employed to removal residual tumour after the tumour biopsy. Immediate post-operative assessment noted complete right eye ptosis, with clinical evidence of complete right third and fourth nerve palsies. MRI was repeated a week later in view of such palsy non-resolution. However, no local compression or edema noted. Observation and monitoring were opted versus surgical revision. Propitiously the aforementioned cranial nerve palsies persist for a month and subsequently subsided. In this case, we highlight the potential deleterious impact of aspirating cystic component and curettaging during pituitary surgery. Likely postulated accounts for such occurrence include sudden release of fluid pressure with resultant cystic traction on its enfolding cranial nerves and subsequent neuropraxia. We aim to invite comments that could enlighten us on this gray area.
Li, Chiao-Zhu; Li, Chiao-Ching; Hsieh, Chih-Chuan; Lin, Meng-Chi; Hueng, Dueng-Yuan; Liu, Feng-Chen; Chen, Yuan-Hao
The fatal type of antiphospholipid syndrome is a rare but life-threating condition. It may be triggered by surgery or infection. Endoscopic transnasal-transsphenoidal surgery is a common procedure for pituitary tumor. We report a catastrophic case of a young woman died of fatal antiphospholipid syndrome following endoscopic transnasal-transsphenoidal surgery. A 31-year-old woman of a history of stroke received endoscopic transnasal-transsphenoidal surgery for a pituitary tumor. The whole procedure was smooth. However, the patient suffered from acute delirium on postoperative day 4. Then, her consciousness became comatose state rapidly with dilatation of pupils. Urgent magnetic resonance imaging of brain demonstrated multiple acute lacunar infarcts. The positive antiphosphoipid antibody and severe thrombocytopenia were also noted. Fatal antiphospholipid syndrome was diagnosed. Plasma exchange, corticosteroids, anticoagulant agent were prescribed. The hemodynamic condition was gradually stable. However, the consciousness was still in deep coma. The patient died of organ donation 2 months later. If patients have a history of cerebral stroke in their early life, such as a young stroke, the APS and higher risk of developing fatal APS after major surgery should be considered. The optimal management of APS remains controversial. The best treatment strategies are only early diagnosis and aggressive therapies combing of anticoagulant, corticosteroid, and plasma exchange. The intravenous immunoglobulin is prescribed for patients with refractory APS.
Doberentz, Elke; Markwerth, Philipp; Wagner, Rebecca; Madea, Burkhard
Hypothermia causes systemic cellular stress. The pituitary gland is an endocrine gland and plays an important role in thermoregulation. When the core body temperature drops, the pituitary gland is activated by stimulation of hypothalamic hormones. In this study, we investigated morphological alterations of the pituitary gland in cases of fatal hypothermia. Several morphological alterations of the anterior lobe of the pituitary gland, such as hemorrhage, vacuolization, and hyperemia, have been previously described in fatal hypothermia. However, the diagnostic value of these findings is controversial. We compared 11 cases of fatal hypothermia with 10 cases lacking antemortem hypothermic influences. In the presence of thermal cellular stress, the expression of heat shock proteins increases to protect cellular structures. Therefore, we immunohistochemically analyzed Hsp27 and Hsp70. Hsp27 expression was detected in 27.3% of the cases of fatal hypothermia and in 10.0% of the control cases, whereas Hsp70 expression was not detected in any case. Additionally, Sudan staining was performed to quantify fatty degeneration. A positive reaction was found in 45.5% of the study group and in 10.0% of the control group. This indicates that fatty degeneration might be a valuable marker when other macroscopic signs of hypothermia are absent.
Anthony, Jeremy; Esper, Gregory J; Ioachimescu, Adriana
Hypothalamic-pituitary (HP) neurosarcoidosis (NS) accounts for 0.5 % cases of sarcoidosis and 1 % of HP masses. Correlative data on endocrine and neurological outcomes is lacking. Retrospective case series and literature review of presentation, treatment and outcome of HP NS. Our series includes 4 men, ages 34-59, followed for a median of 7.3 years (range 1.5-17). All had optic neuropathy, multiple pituitary hormone abnormalities (PHAs) and other organ involvement by sarcoidosis (lung, sino-nasal, brain/spine and facial nerve). Two patients had central diabetes insipidus and one impaired thirst with polydipsia. After treatment with high-dose glucocorticoids, optic neuropathy improved in one case and stabilized in the others. After treatment, HP lesions improved radiologically, but PHAs persisted in all cases. Review of four published series on HP NS in addition to ours yielded 46 patients, age 37 ± 11.8 years, 65 % male. PHAs consisted of anterior hypopituitarism (LH/FSH 88.8 %, TSH 67.4 %, GH 50.0 %, ACTH 48.8 %), hyperprolactinemia (48.8 %) and diabetes insipidus (65.2 %). PHAs were the first sign of disease in 54.3 % patients. Vision problems occurred in 28.3 % patients, but optic neuropathy was not well documented in previous series. Most patients (93.5 %) received high-dose glucocorticoids followed by taper; 50 % also received other immunomodulators, including methotrexate, mycophenolate mofetil, cyclosporine, azathioprine, infliximab and hydrochloroquine. Only 13 % patients showed improvement in PHAs. All-cause mortality was 8.7 %. HP NS is a serious disease requiring multidisciplinary treatment and lifelong follow-up. Prospective multicentric studies are needed to determine a more standardized approach to HP NS and outline predictors of disease outcome.
Geerat J Vermeij
Full Text Available Gigantism-very large body size-is an ecologically important trait associated with competitive superiority. Although it has been studied in particular cases, the general conditions for the evolution and maintenance of gigantism remain obscure. I compiled sizes and dates for the largest species in 3 terrestrial and 7 marine trophic and habitat categories of animals from throughout the Phanerozoic. The largest species (global giants in all categories are of post-Paleozoic age. Gigantism at this level appeared tens to hundreds of millions of years after mass extinctions and long after the origins of clades in which it evolved. Marine gigantism correlates with high planktic or seafloor productivity, but on land the correspondence between productivity and gigantism is weak at best. All global giants are aerobically active animals, not gentle giants with low metabolic demands. Oxygen concentration in the atmosphere correlates with gigantism in the Paleozoic but not thereafter, likely because of the elaboration of efficient gas-exchange systems in clades containing giants. Although temperature and habitat size are important in the evolution of very large size in some cases, the most important (and rare enabling circumstance is a highly developed ecological infrastructure in which essential resources are abundant and effectively recycled and reused, permitting activity levels to increase and setting the stage for gigantic animals to evolve. Gigantism as a hallmark of competitive superiority appears to have lost its luster on land after the Mesozoic in favor of alternative means of achieving dominance, especially including social organization and coordinated food-gathering.
Hansen, F J; Friis, B
Since the original description of cerebral gigantism, about 85 cases have been reported. Four papers comment on familial occurrence but never in parents and their children. This paper describes the syndrome in a mother and her child, which, together with facts pointing towards prenatal etiology, such as excessive birthweight, striking mutual resemblance and abnormal dermatoglyphics, points to a genetic defect. Previous endocrine studies are enlarged by the findings of normal serum somatomedin and serum prolactin.
Kumabe, Ayako; Kenzaka, Tsuneaki; Nishimura, Yoshioki; Aikawa, Masaki; Mori, Masaki; Matsumura, Masami
Anasarca in patients with lymphoma is a rare symptom. We report a patient with DLBCL associated with pituitary gland infiltration that was diagnosed based on significant anasarca. A 72-year-old woman with a 10-year history of hypertension visited a local hospital presenting with anasarca and 15-kg weight gain in the past 3 months. we clinically diagnosed central hypothyroidism caused by pituitary gland infiltration of diffuse large B-cell lymphoma (DLBCL) (clinical stage IV in the Ann Arbor staging classification). The first course of chemotherapy improved anasarca remarkably and the patient's body weight returned to what it was 3 months before. We experienced a patient with remarkable anasarca caused by DLBCL infiltration of the pituitary gland. A pituitary gland lesion with central hypothyroidism should be considered as one of the differential diagnoses of edema. This case was very valuable because we could assess it by following the time course of symptoms (edema and delayed relaxation time of the Achilles tendon reflex), laboratory data, and imaging findings (swelling anterior pituitary lobe).
Shinar, Shiri; Many, Ariel; Maslovitz, Sharon
Oxytocin, a nanopeptide secreted by the posterior pituitary gland, has well-established uterotonic activity. Its role in initiating the vigorous and regular contractions of the first stage of labor is still controversial. We report four cases of panhypopituitarism who had spontaneous onset of labor, undermining the role of maternal oxytocin in the first phase of labor. Four women with no residual pituitary function conceived through ovulation induction and were treated throughout pregnancy with thyroid replacement therapy, desmopressin and glucocorticoids. In all cases pituitary function was undetectable in repeated blood tests. We report their course of pregnancy and delivery. All four pregnancies progressed to term with hormonal replacement therapy. All cases went into spontaneous labor. Two women delivered vaginally unassisted by pharmacological intervention and two delivered by cesarean sections during active labor due to obstetrical indications. Three suffered postpartum hemorrhage. Lactation did not ensue in all four cases. Endogenous pituitary oxytocin is probably not obligatory for initiation of labor in the first phase of parturition. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.
Miyata, Samon; Demachi, Hiroshi; Terabayashi, Tadashi; Sugiyama, Yoshiaki; Miwa, Atsuo (Toyama Prefectural Central Hospital (Japan))
The authors report a case of a 37-year-old woman who was given a surgical resection, using a transsphenoidal approach, for a pituitary chromophobe adenoma (a prolactinoma) and postoperative radiotherapy with parallel-opposed lateral portals (a total dose of 50.8 Gy/26 fractions/37 days; TDF: 81). Seventeen months after this radiotherapy, however, she experienced vertigo and nausea, and a CT scan revealed decreased attenuation in the white matter of the bilateral temporal lobes, although cerebral angiograms showed no abnormalities. Delayed radiation necrosis of the temporal lobes was diagnosed and these clinical symptoms improved with the administrations of steroids and glyceol. Ten years after this radiation, the patient is alive and shows no neurological abnormalities. (author).
Olimpia Contreras Leal
Full Text Available Se trata de una adolescente de 13 años de edad con antecedentes de salud con menarquía a los 11 años y metrorragias frecuentes que acudió a consulta de Pediatría del Hospital Regional de Bata Litoral en la República de Guinea Ecuatorial por aumento de volumen del abdomen, específicamente del hemiabdomen derecho, es ínter consultada con Ginecología por sospecha de tumor de ovario, lo cual se corroboró por ultrasonografía. Se realiza intervención quirúrgica encontrándose quiste gigante de ovario derecho que pesó 6 kg del cual no tuvimos el diagnóstico histológico por no disponer de laboratorio de Anatomía Patológica, pero por las características macroscópicas se trató como un Quiste Seroso Simple, La paciente tuvo una evolución favorable.A 13- year-old girl is presented, having undergone a good health and menarche at 11 years old, she has had vaginal bleeding frequently, and came to Pediatrics Clinics at Bata Littoral Provincial Hospital in Equatorial Guinea with the Pediatrician. The main symptoms were abdominal volume increase, more specifically at right hemiabdomen and pain to deep palpation. This case was sent to the Gynecologist and an ovarian tumor was suspected, afterwards verified by the ultrasonographic study. The patients was referred to surgery, the final clinical diagnosis was Right Ovary Giant Cyst, weighing 6 kg. The histological diagnosis was not assessed because the lack of pathology lab in this country. Following the macroscopic features the tumor was treated as a Simple Serous Cyst. The patient had a favorable evolution.
Metyolkina, L; Peresedov, V
Since 1960 we have performed stereotactic transsphenoidal cryohypophysectomy in 70 patients with pituitary adenomas, 42 women and 28 men, aged 11-59 years. The dominant clinical syndrome was acromegaly in 50 patients, galactorrhea in 9, amenorrhea in 5, adiposogenital dystrophy in 4 and gigantism with mild endocrine symptomatology in 2 patients. In 67 patients the histological structure of the tumor was established by biopsy (50 patients with eosinophil adenoma, 10 with mixed-type adenoma, 4 with chromophobe adenoma and 3 with basophil adenoma). Somatotropic hormone, human growth hormone, prolactin, ACTH and 17-ketosteroid levels indicated active/inactive adenomas. In 42 cases the adenoma was only intrasellar, which was confirmed by contrast X-ray investigations, CT scanning, angiography and ophthalmological investigation. Transnasal stereotactic cryohypophysectomy was performed in all 70 cases using a stereotactic apparatus especially designed for operations on the pituitary. All patients (except 2) tolerated the operation well. No complications occurred. Vision deteriorated after operation in 1 patient. Thrombosis of the left middle cerebral artery developed in another patient. All the other patients noted improvement directly after operation - rapid diminution of signs of acromegaly and rapid restoration of normal values in hormonal tests. Six patients with continuing growth of the tumor underwent a second operation 1.5-6 years after the first operation. We conclude from our own clinical experience and information from the literature that transnasal stereotactic cryodestruction is highly effective and relatively safe in the management of pituitary adenoma.
Ectopic sphenoid sinus pituitary adenoma (ESSPA) with normal anterior pituitary gland: a clinicopathologic and immunophenotypic study of 32 cases with a comprehensive review of the english literature.
Thompson, Lester D R; Seethala, Raja R; Müller, Susan
33% reactive for a single hormone, with prolactin seen most frequently (59%); 19% of cases were non-reactive. The principle differential diagnosis includes olfactory neuroblastoma, neuroendocrine carcinoma, melanoma, and meningioma. All patients were treated with surgery. No patients died from disease, although one patient died with persistent disease (0.8 months). Surgery is curative in the majority of cases, although recurrence/persistence was seen in 4 patients (13.8%). In conclusion, ESSPAs are rare, affecting middle aged patients with non-specific symptoms, showing characteristic light microscopy and immunohistochemical features of their intrasellar counterparts. When encountering a tumor within the sphenoid sinus, ectopic pituitary adenoma must be considered, and pertinent imaging, clinical, and immunohistochemical evaluation undertaken to exclude tumors within the differential diagnosis. This will result in accurate classification, helping to prevent the potentially untoward side effects or complications of incorrect therapy.
Vermeij, Geerat J.
Gigantism—very large body size—is an ecologically important trait associated with competitive superiority. Although it has been studied in particular cases, the general conditions for the evolution and maintenance of gigantism remain obscure. I compiled sizes and dates for the largest species in 3 terrestrial and 7 marine trophic and habitat categories of animals from throughout the Phanerozoic. The largest species (global giants) in all categories are of post-Paleozoic age. Gigantism at this level appeared tens to hundreds of millions of years after mass extinctions and long after the origins of clades in which it evolved. Marine gigantism correlates with high planktic or seafloor productivity, but on land the correspondence between productivity and gigantism is weak at best. All global giants are aerobically active animals, not gentle giants with low metabolic demands. Oxygen concentration in the atmosphere correlates with gigantism in the Paleozoic but not thereafter, likely because of the elaboration of efficient gas-exchange systems in clades containing giants. Although temperature and habitat size are important in the evolution of very large size in some cases, the most important (and rare) enabling circumstance is a highly developed ecological infrastructure in which essential resources are abundant and effectively recycled and reused, permitting activity levels to increase and setting the stage for gigantic animals to evolve. Gigantism as a hallmark of competitive superiority appears to have lost its luster on land after the Mesozoic in favor of alternative means of achieving dominance, especially including social organization and coordinated food-gathering. PMID:26771527
Daliakopoulos Stavros I
Full Text Available Abstract Introduction Spontaneous retroperitoneal hemorrhage is a distinct clinical entity that can present as a rare life-threatening event characterized by sudden onset of bleeding into the retroperitoneal space, occurring in association with bleeding disorders, intratumoral bleeding, or ruptures of any retroperitoneal organ or aneurysm. The spontaneous form is the most infrequent retroperitoneal hemorrhage, causing significant morbidity and representing a diagnostic challenge. Case presentation We report the case of a patient with coronary artery disease who presented with transient ischemic attack, in whom anticoagulant therapy with heparin precipitated a massive spontaneous atraumatic retroperitoneal hemorrhage (with international normalized ratio 2.4, which was treated conservatively. Conclusion Delay in diagnosis is potentially fatal and high clinical suspicion remains crucial. Finally, it is a matter of controversy whether retroperitoneal hematomas should be surgically evacuated or conservatively treated and the final decision should be made after taking into consideration patient's general condition and the possibility of permanent femoral or sciatic neuropathy due to compression syndrome.
Nalini, A.; Biswas, Arundhati
We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient. PMID:19893668
Nalini, A; Biswas, Arundhati
We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient.
Full Text Available We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient.
Nevo, S.; Zeltzer, M.; Benderly, A.; Levy, J.
Three cases of cerebral gigantism, two sibs and their double first cousin, are described in a large inbred family from Israel. Two of the three were observed and diagnosed at birth and two were followed for two years. They all presented the signs and symptoms considered typical of this syndrome, as well as some of the less frequent findings. Generalized oedema and flexion contractures of the feet were observed in two of the three at birth. This has not hitherto been reported in cases of cerebral gigantism, of whom only a few have been observed and diagnosed at birth. Autosomal recessive inheritance is clearly implied in this family. Images PMID:4841084
Full Text Available Internal carotid artery (ICA supraclinoid segment aneurysms extending into the sellar region and leading to pituitary dysfunction are a rare occurrence. To date, long-term follow up of pituitary function 2 years post-treatment has never been reported. Herein, we present a case of pituitary dysfunction due to an unruptured ophthalmic segment internal carotid artery aneurysm and report improved 2-year follow-up results. A 76-year-old male presented with disturbed consciousness due to hyponatremia, which was caused by hypoadrenocorticism resulting from pituitary dysfunction complicated by hypogonadism and hypothyroidism. Computed tomography angiography revealed an intracranial aneurysm of the ophthalmic segment of the right ICA with an intrasellar extension. Thus, digital subtraction angiography and coil embolization were performed, followed by hormone replacement therapy. A 2-year follow-up revealed a partial improvement in the pituitary function, including complete restoration of thyroid-stimulating hormone level and other thyroid hormones levels, and partial restoration of testosterone levels, followed by discontinuation of thyroid hormone replacement therapy. However, the mechanisms of such pituitary dysfunction and the effects of various treatments, including clipping and coiling, on different hormones of pituitary function recovery remain unclear. A long-term follow-up of >2 years may elucidate the pituitary function recovery post-treatment and provide a medication adjustment for hormone replacement therapy.
Qi, Meng; Ye, Ming; Li, Meng; Zhang, Peng
Internal carotid artery (ICA) supraclinoid segment aneurysms extending into the sellar region and leading to pituitary dysfunction are a rare occurrence. To date, long-term follow up of pituitary function 2 years post-treatment has never been reported. Herein, we present a case of pituitary dysfunction due to an unruptured ophthalmic segment internal carotid artery aneurysm and report improved 2-year follow-up results. A 76-year-old male presented with disturbed consciousness due to hyponatremia, which was caused by hypoadrenocorticism resulting from pituitary dysfunction complicated by hypogonadism and hypothyroidism. Computed tomography angiography revealed an intracranial aneurysm of the ophthalmic segment of the right ICA with an intrasellar extension. Thus, digital subtraction angiography and coil embolization were performed, followed by hormone replacement therapy. A 2-year follow-up revealed a partial improvement in the pituitary function, including complete restoration of thyroid-stimulating hormone level and other thyroid hormones levels, and partial restoration of testosterone levels, followed by discontinuation of thyroid hormone replacement therapy. However, the mechanisms of such pituitary dysfunction and the effects of various treatments, including clipping and coiling, on different hormones of pituitary function recovery remain unclear. A long-term follow-up of >2 years may elucidate the pituitary function recovery post-treatment and provide a medication adjustment for hormone replacement therapy.
Arafah, B U; Brodkey, J S; Kaufman, B; Velasco, M; Manni, A; Pearson, O H
Twenty-five patients with acromegaly and 3 patients with gigantism underwent transsphenoidal microsurgery in an attempt to remove the tumor and preserve normal pituitary function whenever possible. An adenoma was identified and removed in 27 of 28 patients. Evaluation 3--6 months postoperatively revealed a GH level less than 5 ng/ml in 29 patients, 5--10 ng/ml in 4 patients and 11--29 ng/ml in 4 other patients. Dynamics of GH secretion were normal in 11 patients who had normal pituitary function and are considered cured. Two patients with low or undetectable GH levels are also considered cured at the expense of being hypopituitary. Three of 7 patients with normal basal GH levels but abnormal dynamics of GH secretion relapsed within 1 yr. Eleven of the 13 patients considered cured did not have extrasellar extension, while 14 of the 15 patients not cured had extrasellar extension. Five patients who were not cured with surgery received radiation therapy. Three patients were treated with an ergot derivative, Lergotrile mesylate, after surgery and radiation therapy failed to normalize GH levels. Transsphenoidal microsurgery is an optimal form of therapy for patients with acromegaly or gigantism, especially those with no extrasellar extension. Dynamics of GH secretion are very useful in evaluating the completeness of adenoma removal.
Full Text Available Objective To explore the operative method and therapeutic efficacy of surgical resection of large invasive pituitary adenomas with individualized approach under neuronavigator guidance.Methods Seventeen patients(10 males and 7 females,aged from 22 to 78 years with a mean of 39.2±9.2 years suffering from large invasive pituitary adenoma of higher than Hardy IV grade hospitalized from 2004 to 2009 were involved in the present study.All procedures were performed with the assistance of neuronavigator via individualized pterion approach,subfrontal extradural approach,trans-sphenoidal approach,or combined approach.The dispersedly invasive pituitary adenomas were resected under the guidance of neuronavigator by fully utilizing the natural anatomical cleavages.All the patients received follow-up CT scanning 3 days after operation,MRI scanning 1 to 3 months after operation,and clinical follow-up ranged from 6 to 72 months.The resection extent and outcome were assessed by imaging examination and clinical results.Results Total tumor removal was achieved in 15 cases,subtotal removal in 1 case,and extensive partial removal in 1 case.The visual impairment and headache were ameliorated in most cases,but in 1 patient they were worsened.Transient diabetes insipidus occurred in 8 cases,electrolyte disturbances were observed in 2 cases,leakage of cerebrospinal fluid appeared in 2 cases,hyposmia in 2 cases,visual impairment aggravated in 1 case,oculomotor nerve and abducens nerve paralysis on the operative side in 1 case,epidural hematoma in occipital and parietal regions in 1 case.No patient died during the follow-up period.Conclusions Individualized surgical approach designed according to the growth direction of tumor under neuronavigator guidance is helpful for the operators to identify the vessels and nerves in the operative field distinctly during the operation,thus the total removal rate is improved,safely of the operation to remove large invasive pituitary
Haeusler, G; Guchev, Z; Köhler, I; Schober, E; Haas, O; Frisch, H
Two boys are presented with the clinical features of cerebral gigantism and chromosomal variants which have not been described so far in this syndrome. In the first boy a de novo pericentric inversion of chromosome Y was found, the karyotypes of all other investigated family members were normal. The patient had an obstructive hypertrophic cardiomyopathy and atrial septal defect type II. The second boy had inherited pericentric inversion of the heterochromatic region of chromosome 9 from his mother. This chromosome 9 variant was also found in his sister who had a similar phenotype but without gigantism. Endocrine evaluation demonstrated normal results in both boys. The intellectual achievement in both cases was average.
Maheswari, S Uma; Sampath, V; Ramesh, A; Manoharan, K
Macrodystrophia lipomatosa (MDL) is a rare congenital form of localized gigantism characterized by progressive overgrowth of all mesenchymal elements with a disproportionate increase in fibro adipose tissue. Here we report a case of 20 years old male who presented with history of painless gradual enlargement of entire left upper limb since childhood. Magnetic resonance imaging and histopathology confirmed the diagnosis of macrodystrophia lipomatosa. This condition has to be differentiated from other causes of localized gigantism, since these conditions differ in their course, prognosis, complications and treatment.
S Uma Maheswari
Full Text Available Macrodystrophia lipomatosa (MDL is a rare congenital form of localized gigantism characterized by progressive overgrowth of all mesenchymal elements with a disproportionate increase in fibro adipose tissue. Here we report a case of 20 years old male who presented with history of painless gradual enlargement of entire left upper limb since childhood. Magnetic resonance imaging and histopathology confirmed the diagnosis of macrodystrophia lipomatosa. This condition has to be differentiated from other causes of localized gigantism, since these conditions differ in their course, prognosis, complications and treatment.
Wang, Lei; Yao, Yong; Feng, Feng; Deng, Kan; Lian, Wei; Li, Guilin; Wang, Renzhi; Xing, Bing
To explore possible reasons for the incidence of a pituitary abscess following transsphenoidal surgery and determine the most effective treatment. A series of 12 patients who had undergone transsphenoidal surgery in other hospitals before being treated at Peking Union Medical College Hospital were reviewed. The presence of a pituitary abscess was confirmed when pus was intraoperatively observed within the sella turcica. All patients were treated with debridement of the abscess, nine among whom through a transsphenoidal approach and the other three via a craniotomy, followed by antibiotic treatment and hormone replacement therapy. The mean follow-up time was 27.0 months (range from 3.0 to 79.0 months). Headache (92%), panhypopituitarism (58%) and visual disturbance (50%) were the most common clinical indicators of a pituitary abscess. Imaging tests demonstrated a pituitary mass in all patients, with seven (58%) manifested with typical magnetic resonance features of an abscess. Ten patients (83%) were correctly diagnosed preoperatively. During surgical exploration, six presented with severe inflammation or an abscess within the sphenoidal sinus. Causative organisms were identified in five patients (42%). After surgical and antibiotic therapies, all patients fully recovered except for two presenting with severe visual impairment. Six patients (50%) required hormone replacement therapy. Retrograde infection from the sphenoid sinus may be a vital mechanism underlying the formation of a pituitary abscess following transsphenoidal surgery. Debridement of the abscess through surgical approaches combined with antibiotic treatment has been found to yield positive outcomes. Copyright © 2014 Elsevier B.V. All rights reserved.
Raluca Maria Furnica
Full Text Available A 26-year-old woman presented with severe postpartum headaches. Magnetic resonance imaging (MRI revealed a symmetric, heterogeneous enlargement of the pituitary gland. Three months later, she developed central diabetes insipidus. A diagnosis of postpartum hypophysitis was suspected and corticosteroids were prescribed. Six months later, the pituitary mass showed further enlargement and characteristics of a necrotic abscess with a peripheral shell and infiltration of the hypothalamus. Transsphenoidal surgery was performed, disclosing a pus-filled cavity which was drained. No bacterial growth was observed, except a single positive blood culture for Staphylococcus aureus, considered at that time as a potential contaminant. A short antibiotic course was, however, administered together with hormonal substitution for panhypopituitarism. Four months after her discharge, severe headaches recurred. Pituitary MRI was suggestive of a persistent inflammatory mass of the sellar region. She underwent a new transsphenoidal resection of a residual abscess. At that time, the sellar aspiration fluid was positive for Staphylococcus aureus and she was treated with antibiotics for 6 weeks, after which she had complete resolution of her infection. The possibility of a pituitary abscess, although rare, should be kept in mind during evaluation for a necrotic inflammatory pituitary mass with severe headaches and hormonal deficiencies.
Naves, Luciana A; Daly, Adrian F; Dias, Luiz Augusto; Yuan, Bo; Zakir, Juliano Coelho Oliveira; Barra, Gustavo Barcellos; Palmeira, Leonor; Villa, Chiara; Trivellin, Giampaolo; Júnior, Armindo Jreige; Neto, Florêncio Figueiredo Cavalcante; Liu, Pengfei; Pellegata, Natalia S; Stratakis, Constantine A; Lupski, James R; Beckers, Albert
X-linked acro-gigantism (X-LAG) syndrome is a newly described disease caused by microduplications on chromosome Xq26.3 leading to copy number gain of GPR101. We describe the clinical progress of a sporadic male X-LAG syndrome patient with an Xq26.3 microduplication, highlighting the aggressive natural history of pituitary tumor growth in the absence of treatment. The patient first presented elsewhere aged 5 years 8 months with a history of excessive growth for >2 years. His height was 163 cm, his weight was 36 kg, and he had markedly elevated GH and IGF-1. MRI showed a non-invasive sellar mass measuring 32.5 × 23.9 × 29.1 mm. Treatment was declined and the family was lost to follow-up. At the age of 10 years and 7 months, he presented again with headaches, seizures, and visual disturbance. His height had increased to 197 cm. MRI showed an invasive mass measuring 56.2 × 58.1 × 45.0 mm, with compression of optic chiasma, bilateral cavernous sinus invasion, and hydrocephalus. His thyrotrope, corticotrope, and gonadotrope axes were deficient. Surgery, somatostatin analogs, and cabergoline did not control vertical growth and pegvisomant was added, although vertical growth continues (currently 207 cm at 11 years 7 months of age). X-LAG syndrome is a new genomic disorder in which early-onset pituitary tumorigenesis can lead to marked overgrowth and gigantism. This case illustrates the aggressive nature of tumor evolution and the challenging clinical management in X-LAG syndrome.
Full Text Available We present 2 patients with pituitary metastases from thyroid carcinoma—the first from anaplastic thyroid carcinoma and the second from follicular thyroid carcinoma. The first patient, a 50-year-old lady, presented with 2-week history of hoarseness of voice, dysphagia, dyspnoea, and neck swelling. Imaging revealed metastatic thyroid cancer to lymph nodes and bone. Histology from surgery confirmed anaplastic thyroid cancer. She was found to have pituitary metastases postoperatively when she presented with nonvertiginous dizziness. She subsequently underwent radiotherapy and radioiodine treatment but passed away from complications. The second patient, a 65-year-old lady, presented with loss of appetite and weight with increased goitre size and dyspnoea. Surgery was performed in view of compressive symptoms and histology confirmed follicular thyroid carcinoma. Imaging revealed metastases to bone, lung, and pituitary. She also had panhypopituitarism with hyperprolactinemia and diabetes insipidus. She received radioiodine therapy but eventually passed away from complications.
Lewandowski, Krzysztof C; Malicka, Katarzyna; Dąbrowska, Katarzyna; Lewiński, Andrzej
A 36-year-old woman was found to have a low morning ACTH concentration despite a history of Addison's disease. Past medical history: At the age of 23 years the subject developed Graves's disease, which was treated with radioiodine. At about the same time, she claimed to have two episodes of pancreatitis treated with cholecystectomy. About seven months later she was euthyroid on L-thyroxine (TSH 1.51 mIU/mL) but was admitted with hypotension, hyponatraemia (sodium 109 mmol/L), and low morning cortisol (119 nmol/L). Further investigations confirmed primary adrenal failure with ACTH concentration of 779 pg/mL (ref. range 0-60) prior to the dose of hydrocortisone. About nine years later she complained about tiredness. Clinically she was normotensive and not pigmented. BMI 22.3 kg/m². Periods were regular. ACTH concentration was surprisingly low (ACTH 8.53 pg/mL, ref. range 0-46), despite very low cortisol (3.37 nmol/L). She was admitted for further assessment. Pituitary MRI scan was unremarkable. An insulin tolerance test was performed and showed a clear increase of ACTH (from 15.2 to 165 pg/mL). There was, however, hardly any increase of ACTH after CRH stimulation (from 6.05 pg/mL to 10.2 pg/mL), thus demonstrating central CRH resistance. In summary, this patient developed secondary adrenal failure in the setting of previous Addison's disease. Interestingly, hypoglycaemia (but not CRH) provided a stimulus for ACTH release, thus demonstrating CRH resistance. The case confirms that besides CRH, other factors are responsible for stimulation of the ACTH-cortisol axis during insulin tolerance test.
Li, Jiaqi; Tan, Huiwen; Huang, Juan; Luo, Dan; Tang, Ying; Yu, Ruichao; Huang, Hui
Thyrotropin-secreting adenoma (TSHoma) is rare. Even though the thyrotoxicosis is mild in patients with TSHoma, it is still a rare cause of arrhythmia, ignore of mild disfunction of thyroid function of TSHoma can lead to the delayed diagnosis of pituitary tumor or leading to recurring of complications. Graves' disease is an auto-immue endocrinological disorder. Association of TSHoma and Graves's disease is extremely rare. Coexistence of these two diseases made the diagnosis and treatment complicated. This patient was a 55-year-old man who had been referred to the department of endocrinology and metabolism of the West China Hospital due to recurrent atrial fibrillation (AF) and thyroxicosis. Examinations revealed pituitary thyrotropin-secreting macroadenoma with Graves' disease. We conducted transsphenoidal surgery. Thyrozol was used to treat the recurrence of Graves' disease after pituitary surgery. The TSHoma was successfully cured, and recurrent Graves' disease was controlled very well. The association of TSHoma and Graves' disease is extremely rare. Even though the clinical features of thyrotoxicosis are milder in patients with TSHoma, thyroid function tests are still important clinical assessment of patients with AF, which is an arrhythmia associated with hyperthyroidism. TSHoma is a rare cause of thyrotoxicosis; however, ignoring of the mild disfunction caused by TSHoma can lead to the delayed diagnosis of pituitary tumors or to recurring of complications of TSHoma.
Gondim, Jackson A; Almeida, João Paulo; de Albuquerque, Lucas Alverne F; Gomes, Erika; Schops, Michele; Ferraz, Tania
Acromegaly is a chronic disease related to the excess of growth hormone (GH) and insulin-like growth factor–I secretion, usually by pituitary adenomas. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The introduction of endoscopy as an additional tool for surgical treatment of pituitary adenomas and, therefore, acromegaly represents an important advance of pituitary surgery in the recent years. The aim of this retrospective study is to evaluate the results of pure transsphenoidal endoscopic surgery in a series of patients with acromegaly who were operated on by a pituitary specialist surgeon. The authors discuss the advantages, outcome, complications, and factors related to the success of the endoscopic approach in cases of GHsecreting adenomas. The authors retrospectively analyzed data from cases involving patients with GH-secreting adenomas who underwent pure transsphenoidal endoscopic surgery at the Department of Neurosurgery of the General Hospital in Fortaleza, Brazil, between 2000 and 2009. Tumors were classified according to size as micro- or macroadenomas, and tumor extension was analyzed based on suprasellar/parasellar extension and sella floor destruction. All patients were followed up for at least 1 year. The criteria of disease control were GH levels transsphenoidal surgery for treatment of acromegaly. Disease control was obtained in 50 cases (74.6%). The rate of treatment success was higher in patients with microadenomas (disease control achieved in 12 [85.7%] of 14 cases) than in those with larger lesions. Suprasellar/parasellar extension and high levels of sella floor erosion were associated with lower rates of disease control (p = 0.01 and p = 0.02, respectively). Complications related to the endoscopic surgery included epistaxis (6.0%), transitory diabetes insipidus (4.5%), and 1 case of seizure (1.5%). Endoscopic transsphenoidal surgery represents an effective option for treatment of patients
J.F. Arango Ospina
Full Text Available Presentamos el caso de un paciente de 2 años de edad, conocido en su pueblo, como "el niño tortuga (testudines" debido a la gran similitud con dicho reptil. Desde su nacimiento presentó nevus melanocítico gigante localizado en región torácica posterior, región lumbosacra y lesiones satélites en extremidades y abdomen, de 50 por 40cm de diámetro y color café oscuro; la piel en las zonas afectadas es gruesa con grandes surcos de aspecto corrugado (cerebriforme. El nombre dado al niño se basa en la similitud de sus lesiones con la morfología de una tortuga, ya que el caparazón cubre la zona superior, inferior y lateral del cuerpo de este animal, de la misma forma que el nevus gigante lo hacía en el caso de nuestro paciente. Este caparazón está formado por placas óseas revestidas de placas corneas, que se asemejan a los surcos de aspecto corrugado que conforman el nevus melanocítico gigante. Los estudios clínicos practicados revelaron compromiso cardiovascular, hepático y esplénico, así como una desfavorable evolución y pronostico del cuadro clínico, por lo que esta patología, específicamente para este niño y de acuerdo a la junta Médico-Quirúrgica y valoraciones, fue considerada inoperable.We present the case of a 2 years-old patient, known in his town as "turtle boy"(testudines due to his great simility with this reptile because of his giant congenital melanocytic nevus, located in the posterior and lateral aspect of the chest and in smaller proportion in abdomen and extremities, of 50 by 40cm and dark brown colour. The skin in this zone is thick with great furrows and corrugated aspect. He was called "turtle boy" based on the morphology of the turtle, its shell covers the superior, inferior and lateral zone like in our patient's case. It is formed by bone layers covered by cornea layers, which resemble the furrows and corrugated aspect of the giant congenital melanocytic nevus. Medical investigation reveals
Gilis-Januszewska, Aleksandra; Wilusz, Małgorzata; Pantofliński, Jacek; Turek-Jabrocka, Renata; Sokołowski, Grzegorz; Sowa-Staszczak, Anna; Kluczyński, Łukasz; Pach, Dorota; Zieliński, Grzegorz; Hubalewska-Dydejczyk, Alicja
AbstractContext: Aggressive pituitary tumours causing Cushing's Disease are very rare, difficult to treat, and usually resistant to conventional therapy. There is growing evidence for the use of temozolomide (TZM), an alkylating chemotherapeutic agent, as first line chemotherapy in tumours resistant to repeated neurosurgery, radiotherapy and adrenalectomy. To present the response to TMZ in a rare case of an aggressive pituitary tumour in the course of Cushing's Disease and to review the literature referring to similar cases. In this report, we present the case of a 61 year old male patient who was diagnosed with Cushing's Disease in the course of a pituitary invasive macroadenoma in 2011. The patient underwent 4 transphenoidal non-radical neurosurgeries (2012,2013) with rapid tumour progression, repeated non-radical bilateral adrenalectomy (2012, 2013) and stereotactic radiotherapy, and gamma knife surgery (2013, 2015). Histopathological examination revealed macroadenoma with high cell polymorphism and the presence of Crooke's cells, Ki-<2%. Since 2015 the patient has been treated with 6 cycles of TMZ (320 mg per day for 5 consecutive days, 28-day cycle) with clinical and biochemical improvement and stabilized tumour size and no side effects. TMZ was continued for up to 9 cycles with a stable serum level of cortisol and ACTH being observed. However, clinical symptoms like headaches, visual field impairment, and finally hearing loss started to progress from the eighth cycle. After the ninth cycle of TMZ, there was a sudden increase in the size of the tumour, impairment of the cortisol and ACTH level, marked deterioration of the clinical status with the recurrence of severe headaches, narrowing of the visual field and hearing loss. At the beginning of 2016, a sudden clinical status and sight deterioration, strong headaches, drop of the right eyelid with widening of the pupil were observed. The patient died in February 2016. The case of our patient suggests that the
Full Text Available Diffuse and reactive pituitary gland enlargement secondary to primary hypothyroidism is an uncommon occurrence and that can masquerade many pituitary disorders. In present article, we report a case of 19 year female severe hypothyroidism presenting with diffuse enlargement of pituitary gland and hyperprolactinemia and review the clinical importance of this entity. Knowledge of this entity is very important to avoid unnecessary surgery and irreversible complications in this sub-group of patients.
Agrawal Amit; Reddy Amareesh P.; Malleswara Rao G.; Reddy V. Umamaheswara
Diffuse and reactive pituitary gland enlargement secondary to primary hypothyroidism is an uncommon occurrence and that can masquerade many pituitary disorders. In present article, we report a case of 19 year female severe hypothyroidism presenting with diffuse enlargement of pituitary gland and hyperprolactinemia and review the clinical importance of this entity. Knowledge of this entity is very important to avoid unnecessary surgery and irreversible complications in this sub-group of patients.
Mendola, Marco; Dolci, Alessia; Piscopello, Lanfranco; Tomei, Giustino; Bauer, Dario; Corbetta, Sabrina; Ambrosi, Bruno
We describe a patient affected by Cushing's disease due to the presence of double pituitary adenomas, one located within the anterior pituitary and the other in the infundibulum associated with a remnant of Rakthe's pouch. Cure was achieved only after the infundibulum lesion was surgically removed. A 38-year-old female presented with unexplained weight gain, hirsutism, amenorrhea, asthenia, recurrent cutaneous micotic infections and alopecia. Hormonal studies indicated Cushing's disease and MRI showed an enlarged pituitary gland with a marked and homogeneous enhancement after injection of gadolinium and an enlarged infundibulum with a maximum diameter of 8 mm. As a venous sampling of the inferior petrosal sinus after 10 μg iv desmopressin stimulation revealed a central to peripheral ACTH ratio consistent with a pituitary ACTH-secreting tumor, transphenoidal explorative surgery was performed and a 4-mm pituitary adenoma immunopositive for ACTH was disclosed and removed. Since postoperative hormonal evaluation showed persistent hypercortisolism, confirmed by dynamic tests, the patient again underwent surgery by transcranial access and the infundibulum mass was removed. Histology and immunochemistry were consistent with an ACTH-secreting adenoma. A few months after the second operation, cushingoid features were significantly reverted and symptoms improved. Although Cushing's patients bearing multiple adenomas have already been documented, the presence of two adenomas both immunohistochemically positive for ACTH is a very rare cause of Cushing's disease and this is the first report of a case of double ACTH-producing adenomas, one located in the pituitary gland and one attached to the stalk.
Full Text Available Temozolomide (TMZ is an alkylating chemotherapeutic agent that has recently been used in some cases as a new therapeutic tool for pituitary carcinomas and aggressive pituitary adenomas. In this report, we present the case of effective TMZ treatment in a 42-year-old man with ACTH-secreting carcinoma. The tumor grew progressively over 4 years, from 2.2 to 31.1 cm3, despite three surgical approaches and gamma-knife treatment. Ki-67 increased from 2 to 18%. An intradural metastasis at the foramen magnum was detected by MRI after the third operation. Thereafter, four cycles of 5-day TMZ administration (200 mg/m2/day during the first, and 150 mg/m2/day during the following cycles induced dramatic tumor size reduction (>90%. Clinical conditions improved progressively and, after 17 months from the beginning of TMZ administration, the patient is still alive. The treatment was well tolerated except for a transient thrombocytopenia (grade 4 WHO.
Ohta, Masahiro; Sasaki, Ushio; Shinohara, Nobuya; Takeda, Tetsuji; Chaki, Takanori; Nishigakiuchi, Keiji; Kusunoki, Katsusuke
A 60-year-old woman with radiation optic neuropathy 21 months after irradiation is reported. The patient received a total dose of 50 Gy in 25 fractions for 39 days for pituitary adenoma. She presented with bitemporal hemianopsia and loss of recent memory. Gadolinium-enhanced T1-weighted imaging was very useful for detecting lesions in the optic nerves and chiasm to the hypothalamus including mamillary bodies. Two-month steroid therapy was effective in preventing the disease progression, although visual loss and loss of recent memory were not improved. (N.K.)
Ohta, Masahiro; Sasaki, Ushio; Shinohara, Nobuya; Takeda, Tetsuji; Chaki, Takanori; Nishigakiuchi, Keiji; Kusunoki, Katsusuke (Ehime Prefectural Central Hospital, Matsuyama (Japan))
A 60-year-old woman with radiation optic neuropathy 21 months after irradiation is reported. The patient received a total dose of 50 Gy in 25 fractions for 39 days for pituitary adenoma. She presented with bitemporal hemianopsia and loss of recent memory. Gadolinium-enhanced T1-weighted imaging was very useful for detecting lesions in the optic nerves and chiasm to the hypothalamus including mamillary bodies. Two-month steroid therapy was effective in preventing the disease progression, although visual loss and loss of recent memory were not improved. (N.K.).
Full Text Available Nummular headache (NH is a newly categorized headache disorder characterized by a consistent clinicographics in each attack. Currently, it is considered as a primary headache disorder due to epicranial neuralgia but the pathomechanism is still unknown. We report a woman, whose recurrent NH subsided after trans-sphenoidal surgery for her pituitary oncocytoma. The recovery of NH in this patient encourages the central mechanism for NH occurrence. After a review of literature concerning, NH and intracranial secondaries we propose that central NH is a referral pain from pain-sensitive structures, such as meninges, superimposing by pre-existing lower pain threshold or pain modulation.
... Association (ABTA) International RadioSurgery Association National Brain Tumor Society National Institute of Child Health and Human Development ... Definition The pituitary is a small, bean-sized gland ...
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Romijn, Johannes A.
Patients with pituitary diseases have decreased quality of life. Sleep disorders are prevalent among patients with pituitary diseases and contribute to decreased quality of life. Patients previously treated for compression of the optic chiasm by surgery, and in some cases postoperative radiotherapy,
Jesser, J.; Schlamp, K.; Bendszus, M.
This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15 % of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65 % of pituitary gland adenomas secrete hormones whereby approximately 50 % secrete prolactin, 10 % secrete growth hormone (somatotropin) and 6 % secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10 % of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland. (orig.) [de
Jesser, J; Schlamp, K; Bendszus, M
This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15% of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65% of pituitary gland adenomas secrete hormones whereby approximately 50% secrete prolactin, 10% secrete growth hormone (somatotropin) and 6% secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10% of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland.
Yen, M T; Gedde, S J; Flynn, J T
To report a patient with unilateral glaucoma associated with Sotos syndrome. Sotos syndrome (cerebral gigantism) is a disorder of growth and development with characteristic facial changes and normal endocrine function. Ocular manifestations may also include megalocornea, iris hypoplasia, cataracts, megalophthalmos, strabismus, nystagmus, and retinal dystrophy. Case report. A 50 year-old man with the clinical features of Sotos syndrome presented with complaints of decreased vision in the left eye. Ophthalmologic examination revealed bilateral megalocornea, megalophthalmos, iris hypoplasia and transillumination defects, cataracts, and unilateral glaucoma. Intraocular pressure was lowered, and visual field loss was stabilized with topical medications. Sotos syndrome patients should be examined routinely to allow for early detection and treatment of potential ocular problems, including glaucoma.
Yeh, H; Price, R L; Lonsdale, D
A five-year-old girl with cerebral gigantism (Sotos' syndrome) and cataracts is described. Sotos' syndrome, characterized by generalized gigantism with normal endocrine studies has rarely been reported with ocular abnormalities and never with cataracts. It is important to study any child with cataracts for systemic disease.
Pendleton, Courtney; Adams, Hadie; Salvatori, Roberto; Wand, Gary; Quiñones-Hinojosa, Alfredo
A review of Dr. Cushing's surgical cases at Johns Hopkins Hospital revealed new information about his early operative experience with acromegaly. Although in 1912 Cushing published selective case studies regarding this work, a review of all his operations for acromegaly during his early years has never been reported. We uncovered 37 patients who Cushing treated with surgical intervention directed at the pituitary gland. Of these, nine patients who presented with symptoms of acromegaly, and one with symptoms of gigantism were selected for further review. Two patients underwent transfrontal 'omega incision' approaches, and the remaining eight underwent transsphenoidal approaches. Of the 10 patients, 6 were male. The mean age was 38.0 years. The mean hospital stay was 39.4 days. There was one inpatient death during primary interventions (10%) and three patients were deceased at the time of last follow-up (33%). The mean time to death, calculated from the date of the primary surgical intervention, and including inpatient and outpatient deaths, was 11.3 months. The mean time to last follow-up, calculated from the day of discharge, was 59.3 months. At the time of last follow-up, two patients reported resolution of headache; four patients reported continued visual deficits, and two patients reported ongoing changes in mental status. This review analyzes the outcomes for 10 patients who underwent surgical intervention for acromegaly or gigantism, and offers an explanation for Cushing's transition from the transfrontal "omega incision" to the transsphenoidal approach while practicing at the Johns Hopkins Hospital.
Full Text Available Objective: The safety of endoscopic skull base surgery can be enhanced by accurate navigation in preoperative computed tomography (CT and magnetic resonance imaging (MRI. Here, we report our initial experience of real-time intraoperative CT-guided navigation surgery for pituitary tumors in childhood. Materials and Methods: We report the case of a 15-year-old girl with a huge growth hormone-secreting pituitary adenoma with supra- and perisellar extension. Furthermore, the skull base was infiltrated. In this case, we performed an endonasal transsphenoidal approach for debulking the adenoma and for chiasma decompression. We used an MRI neuronavigation (Medtronic Stealth Air System which was registered via intraoperative CT scan (Siemens CT Somatom. Preexisting MRI studies (navigation protocol were fused with the intraoperative CT scans to enable three-dimensional navigation based on MR and CT imaging data. Intraoperatively, we did a further CT scan for resection control. Results: The intraoperative accuracy of the neuronavigation was excellent. There was an adjustment of <1 mm. The navigation was very helpful for orientation on the destroyed skull base in the sphenoid sinus. After opening the sellar region and tumor debulking, we did a CT scan for resection control because the extent of resection was not credible evaluable in this huge infiltrating adenoma. Thereby, we were able to demonstrate a sufficient decompression of the chiasma and complete resection of the medial part of the adenoma in the intraoperative CT images. Conclusions: The use of intraoperative CT/MRI-guided neuronavigation for transsphenoidal surgery is a time-effective, safe, and technically beneficial technique for special cases.
Boggs, L.; Liu, N.; Riousset, J. A.; Shi, F.; Rassoul, H.
Here we present observational and modeling results that provide insight into thunderstorm charge structures that produce gigantic jet discharges. The observational results include data from four different thunderstorms producing 9 negative gigantic jets from 2010 to 2014. We used radar, very high frequency (VHF) and low frequency (LF) lightning data to analyze the storm characteristics, charge structures, and lightning activity when the gigantic jets emerged from the parent thunderstorms. A detailed investigation of the evolution of one of the charge structures by analyzing the VHF data is also presented. The newly found charge structure obtained from the observations was analyzed with fractal modeling and compared with previous fractal modeling studies [Krehbiel et al., Nat. Geosci., 1, 233-237, 2008; Riousset et al., JGR, 115, A00E10, 2010] of gigantic jet discharges. Our work finds that for normal polarity thunderstorms, gigantic jet charge structures feature a narrow upper positive charge region over a wide middle negative charge region. There also likely exists a `ring' of negative screening charge located around the perimeter of the upper positive charge. This is different from previously thought charge structures of the storms producing gigantic jets, which had a very wide upper positive charge region over a wide middle negative charge region, with a very small negative screening layer covering the cloud top. The newly found charge structure results in leader discharge trees in the fractal simulations that closely match the parent flashes of gigantic jets inside and outside the thundercloud. The previously used charge structures, while vital to the understanding of gigantic jet initiation and the role of charge imbalances inside the cloud, do not produce leader discharge trees that agree with observed gigantic jet discharges.Finally, the newly discovered gigantic jet charge structures are formed near the end of a convective pulse [Meyer et al., JGR, 118
Finzi, Giovanna; Cerati, Michele; Marando, Alessandro; Zoia, Cesare; Ferreli, Fabio; Tomei, Giustino; Castelnuovo, Paolo; La Rosa, Stefano; Capella, Carlo
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.
Peppa, Melpomeni; Papaxoinis, Georgios; Xiros, Nikolaos; Raptis, Sotirios A; Economopoulos, Theofanis; Hadjidakis, Dimitrios
Pituitary metastasis occurs rarely in cancer patients and often remains undiagnosed. However, early detection and appropriate treatment can improve the patient's quality of life and possibly prolong survival. Herein, we describe the case of a 52-year-old woman with panhypopituitarism caused by metastases to the hypothalamus and pituitary from primary breast cancer. She had a 5-year history of breast cancer with metastases to the bones 1.5 years after initial diagnosis and mastectomy. She presented with severe headaches, generalized fatigue, dizziness, hypotension, difficulties with balance and coordination, polyuria, and polydipsia. Laboratory work-up revealed panhypopituitarism (central diabetes insipidus; hypothyroidism; and low prolactin, gonadotrophin, and adrenocorticotropic hormone levels), and magnetic resonance imaging confirmed the pituitary and hypothalamic involvement. She received hormone replacement therapy, radiation therapy of the sella turcica and suprasellar lesion, and chemotherapy, with significant improvement of her clinical status, but she died 15 months later.
L. M. Laskoski
Full Text Available ABSTRACTLaminitis in horses is often associated with endocrine disorders, especially the pituitary pars intermedia dysfunction (PPID in older animals. Morphologic exams of the laminar tissue of the hoof were performed in two horses with suspected PPID, with no clinical signs of laminitis. Changes compatible with laminitis of endocrine origin were observed, such as rounding of the nuclei of the basal cells, thinning and stretching of the secondary epidermal laminae and tissue proliferation. PPID horses with no clinical signs of laminitis may be affected by lesions of the laminar tissue of the hoof that compromise the integrity of the dermal-epidermal junction and may develop clinical symptoms of the disease. It has been suggested that the development stage of endocrine laminitis is longer, but further studies should be conducted to confirm it.
Nandini Chakrabarti; Chandan Chattopadhyay; Majhi Bhuban; Salil Kumar Pal
A congenital disorder with variable manifestations, including partial gigantism of the hands and feet with hypertrophy of soles, nevi, hemihypertrophy, gynecomastia, macrocephaly and other skull abnormalities, and abdominal lipomatosis. The cause is unknown, although a genetic origin, generally of autosomal-dominant transmission, has been conjectured. Symptoms can be treated, but there is no known cure. We present the case of a young male with grotesque overgrowth of the right lower limb, spl...
S Uma Maheswari; V Sampath; A Ramesh; K Manoharan
Macrodystrophia lipomatosa (MDL) is a rare congenital form of localized gigantism characterized by progressive overgrowth of all mesenchymal elements with a disproportionate increase in fibro adipose tissue. Here we report a case of 20 years old male who presented with history of painless gradual enlargement of entire left upper limb since childhood. Magnetic resonance imaging and histopathology confirmed the diagnosis of macrodystrophia lipomatosa. This condition has to be differentiated fro...
Ito, Jusuke; Tokiguchi, Susumu; Nakamori, Akitoshi; Watanabe, Akira; Yokoyama, Motoharu.
Fifty-one patients from a group of 344 patients undergoing the evaluation of intrasellar or parasellar tumors were diagnosed on CT as having an intrasellar pituitary adenoma. Axial transverse sections were performed at -10 0 to Reid's basal line, using 1.5-mm-thick slices and sagittal and coronal reformation. Of these 51 patients, 17 showed a shift of the pituitary stalk. The area where a tumor was thought to be located within the sella turcica on preoperative CT became defective on CT after transsphenoidal surgery in all cases. Histological verification was obtained in all cases. Also, the shift of the pituitary stalk was normalized or markedly improved after surgery in all cases. In functioning tumors, all cases except two showed an endocrinologically normal state or a marked improvement after transsphenoidal surgery. On the basis of the above-mentioned facts, it was concluded that the shift of the pituitary stalk in intrasellar pituitary adenomas indicated the evidence of a mass and its location in the sella turcica. However, a shift of the pituitary stalk was also observed under other conditions, such as empty sella and tuberculum sellae meningioma, and so it is not a pathognomonic finding in intrasellar pituitary adenomas. (author)
Capiel, Carlos A. h; Bouzas, Carlos A.; Mondino, Ana
We report a case of a 14 years old patient with growth arrest and laboratory evidence of hypothyroidism. MR revealed pituitary enlargement simulating macro adenoma. Thyroid replacement therapy resulted in regression of the pituitary size. Awareness of MR appearance of pituitary hyperplasia in children and juvenile patients with laboratory evidence of hypothyroidism might avoid misdiagnosis for pituitary tumor. (author)
Arroyo, Armando; Pernasetti, Flavia; Vasilyev, Vyacheslav V.; Amato, Paula; Yen, Samuel S. C.; Mellon, Pamela L.
Summary We report a 28-year-old-female who presented with primary amenorrhoea, absence of puberty, obesity and normal stature. The subject was clearly short as a child, with a height more than 2 SD below normal until the age of 15 years. The pubertal growth spurt failed to develop. She continued growing at a prepubertal rate until growth ceased at the age of 20 years, reaching her final adult height of 157 cm (SDS −0.86) without hormonal treatment. A combined pituitary hormone stimulation test of anterior pituitary function showed deficiencies of GH, LH and FSH, and low normal serum levels of TSH and PRL. Magnetic resonance imaging revealed a hypoplastic pituitary with markedly reduced pituitary height. In addition, a whole body dual energy X-ray absorptiometry scan showed high levels of body fat (54%). Combined pituitary hormone deficiencies with a hypoplastic pituitary suggested the diagnosis of a Prophet of Pit-1 (PROP1) gene mutation. Normal stature in this case, however, confounded this diagnosis. Sequencing of PROP1 revealed homozygosity for a single base-pair substitution (C to T), resulting in the replacement of an Arg by a Cys at codon 120 (R120C) in the third helix of the homeodomain of the Prop-1 protein. To our knowledge, this is the first report of a patient with a mutation in the PROP1 gene that attained normal height without hormonal treatment, indicating a new variability in the PROP1 phenotype, with important implications for the diagnosis of these patients. We suggest that this can be explained by (i) the presence of low levels of GH in the circulation during childhood and adolescence; (ii) the lack of circulating oestrogen delaying epiphyseal fusion, resulting in growth beyond the period of normal growth; and (iii) fusion of the epiphyseal plates, possibly as a result of circulating oestrogens originating from peripheral conversion of androgens by adipose tissue. PMID:12153609
Lymphocytic Hypophysitis: Differential Diagnosis and Effects of High-Dose Pulse Steroids, Followed by Azathioprine, on the Pituitary Mass and Endocrine Abnormalities — Report of a Case and Literature Review
Full Text Available We report on a man with a progressively increasing pituitary mass, as demonstrated by MRI. It produced neurological and ophthalmological symptoms, and, ultimately, hypopituitarism. MRI also showed enlargement of the pituitary stalk and a dural tail phenomenon. An increased titer of antipituitary antibodies (1:16 was detected in the serum. Pituitary biopsy showed autoimmune hypophysitis (AH. Neither methylprednisolone pulse therapy nor a subsequent treatment with azathioprine were successful in recovering pituitary function, or in inducing a significant reduction of the pituitary mass after an initial, transient clinical and neuroradiological improvement. Anterior pituitary function evaluation revealed persistent hypopituitarism. AH is a relatively rare condition, particularly in males, but it represents an emerging entity in the diagnostic management of pituitary masses. This case shows that response to appropriate therapy for hypophysitis may not be very favorable and confirms that diagnostic management of nonsecreting pituitary masses can be a challenge. Clinical, imaging, and laboratory findings are useful for suggesting the diagnosis, but pituitary biopsy may be necessary to confirm it.
Yamada, Shozo; Fukuhara, Noriaki; Horiguchi, Kentaro; Yamaguchi-Okada, Mitsuo; Nishioka, Hiroshi; Takeshita, Akira; Takeuchi, Yasuhiro; Ito, Junko; Inoshita, Naoko
The aim of this study was to analyze clinicopathological characteristics and treatment outcomes in a large single-center clinical series of cases of thyrotropin (TSH)-secreting pituitary adenomas. The authors retrospectively reviewed clinical, pathological, and treatment characteristics of 90 consecutive cases of TSH-secreting pituitary adenomas treated with transsphenoidal surgery between December 1991 and May 2013. The patient group included 47 females and 43 males (median age 42 years, range 11-74 years). Sixteen tumors (18%) were microadenomas and 74 (82%) were macroadenomas. Microadenomas were significantly more frequent in the more recent half of our case series (12 of 45 cases) (p = 0.0274). Cavernous sinus invasion was confirmed in 21 patients (23%). In 67 cases (74%), the tumors were firm elastic or hard in consistency. Acromegaly and hyperprolactinemia were observed, respectively, in 14 (16%) and 11 (12%) of the 90 cases. Euthyroidism was achieved in 40 (83%) of 48 patients and tumor shrinkage was found in 24 (55%) of 44 patients following preoperative somatostatin analog treatment. Conventional transsphenoidal surgery, extended transsphenoidal surgery, and a simultaneous combined supra- and infrasellar approach were performed in 85, 2, and 3 patients, respectively. Total removal with endocrinological remission was achieved in 76 (84%) of 90 patients, including all 16 (100%) patients with microadenomas, 60 (81%) of the 74 with macroadenomas, and 8 (38%) of the 21 with cavernous sinus invasion. None of these 76 patients experienced tumor recurrence during a median follow-up period of 2.8 years. Stratifying by Knosp grade, total removal with endocrinological remission was achieved in 34 of 36 patients with Knosp Grade 0 tumors, all 24 of those with Grade 1 tumors, 12 of the 14 with Grade 2 tumors, 6 of the 8 with Grade 3 tumors, and none of the 8 with Grade 4 tumors. Cavernous sinus invasion and tumor size were significant independent predictors of surgical
The anterior pituitary gland integrates the repertoire of hormonal signals controlling thyroid, adrenal, reproductive, and growth functions. The gland responds to complex central and peripheral signals by trophic hormone secretion and by undergoing reversible plastic changes in cell growth leading to hyperplasia, involution, or benign adenomas arising from functional pituitary cells. Discussed herein are the mechanisms underlying hereditary pituitary hypoplasia, reversible pituitary hyperplas...
Vinner, M.G.; Khachatryan, M.A.; Abelyan, A.M.
The paper is concerned with an analysis of the clinical X-ray picture in 100 patients with gigantic pulmonary abscesses (the diameter over 6 cm) and in 102 patients with retrostenotic abscesses in central lung cancer, gigantic peripheral cancer with disintegration, tuberculous infiltrate with dissociation, an echinococcal cyst with suppuration and rupture in the bronchus. The reliable clinical differential diagnostic symptoms were not revealed. The chief method of X-ray examination is tomography. In addition to examination of the gigantic focus of lesion in the lung, tomography of the major bronchi should be also performed. The difference between a gigantic pulmonary abscess and peripheral lung cancer is in the nature of the walls and contours; of particular importance is the symptom of nodularity and radiance of the outlines of the pathological shadow which is more distinctive in peripheral cancer. Correct diagnosis was established in 96.6% of the patients
Kim, Jung Hee; Lee, Jung Hyun; Lee, Ji Hyun; Hong, A Ram; Kim, Yoon Ji; Kim, Yong Hwy
The outcomes of recent endoscopic surgery of nonfunctioning pituitary adenomas (NFPAs) are controversial when compared with traditional microscopic surgery. We aimed to assess the outcomes of endoscopic transsphenoidal surgeries performed by 1 surgeon with 7 years of experience and elucidate the predictive factors for surgical outcomes for NFPAs. We included 331 patients (155 men and 176 women) with clinical NFPAs who underwent transsphenoidal surgery because of visual symptoms by a single surgeon in Seoul National University Hospital from March 2010 to May 2016. We assessed the tumor removal rate, hormonal outcomes, visual outcomes, and complications. The gross total resection rate of endoscopic transsphenoidal surgery for NFPAs by a single surgeon was 74.9%. Cavernous sinus invasion, a high Knosp grade, large tumor size, previous surgery, and lack of surgical experience in the neurosurgeon elevated the risk for residual tumors. Visual deficits were improved in 73.4% of the patients, which was associated with tumor size, preoperative visual impairment score, previous radiation, and surgical experience. Hormonal status was improved in 15.4% and aggravated in 32.9% after surgery. There were no predictors for hormonal recovery. Transient diabetes insipidus (DI) was the most common complication (9.1%), and among these patients, 3.0% had persistent DI. Endoscopic transsphenoidal surgery by a well-experienced surgeon was an effective and safe treatment for NFPAs, but the hormonal outcomes were not changed compared with previous reports of microscopic surgery. Large tumor size and cavernous sinus invasion were still the barriers for achieving total resection. Copyright © 2017 Elsevier Inc. All rights reserved.
Izumi, Yusuke; Masuda, Takeshi; Nabeshima, Shinji; Horimasu, Yasushi; Nakashima, Taku; Miyamoto, Shintaro; Iwamoto, Hiroshi; Fujitaka, Kazunori; Murakami, Yuji; Hamada, Hironobu; Nagata, Yasushi; Hattori, Noboru
Pituitary metastasis of lung cancer is rare; however, it often causes diabetes insipidus. Although the majority of such patients are treated with radiation therapy, it remains unclear whether diabetes insipidus can be controlled by radiation therapy. A 72-year-old man was admitted to our hospital for hemosputum, headache, and polyuria. A chest CT scan showed a 3.0 cm mass in the left upper lobe of his lung. Bronchofiberscopy results confirmed the pathological diagnosis of lung adenocarcinoma. Based on the findings from PET-CT, head MRI, and endocrine tests, the diagnosis of lung adenocarcinoma( cT1bN0M1b, stage IV)accompanied with central diabetes insipidus caused by pituitary metastasis was made. Oral administration of desmopressin reduced urine volumes; however, chemotherapy for achieving stable disease in the primary tumor was ineffective in controlling the symptoms of diabetes insipidus. Chemotherapy was discontinued after 4 months because of severe hematological toxicity. During 2 months after the cessation of chemotherapy, polyuria worsened and, therefore, radiation therapy for pituitary metastasis was started. Following the radiation therapy, an apparent reduction in urine volume was observed. Our experience of this case suggests that radiation therapy for pituitary metastasis should be considered at the time when diabetes insipidus becomes clinically overt.
Izumi, Yusuke; Masuda, Takeshi; Nabeshima, Shinji
Pituitary metastasis of lung cancer is rare; however, it often causes diabetes insipidus. Although the majority of such patients are treated with radiation therapy, it remains unclear whether diabetes insipidus can be controlled by radiation therapy. A 72-year-old man was admitted to our hospital for hemosputum, headache, and polyuria. A chest CT scan showed a 3.0 cm mass in the left upper lobe of his lung. Bronchofiberscopy results confirmed the pathological diagnosis of lung adenocarcinoma. Based on the findings from PET-CT, head MRI, and endocrine tests, the diagnosis of lung adenocarcinoma (cT1bN0M1b, stage four) accompanied with central diabetes insipidus caused by pituitary metastasis was made. Oral administration of desmopressin reduced urine volumes; however, chemotherapy for achieving stable disease in the primary tumor was ineffective in controlling the symptoms of diabetes insipidus. Chemotherapy was discontinued after 4 months because of severe hematological toxicity. During 2 months after the cessation of chemotherapy, polyuria worsened and, therefore, radiation therapy for pituitary metastasis was started. Following the radiation therapy, an apparent reduction in urine volume was observed. Our experience of this case suggests that radiation therapy for pituitary metastasis should be considered at the time when diabetes insipidus becomes clinically overt. (author)
Tokunaga, Hitoshi; Hoshi, Seiichiro; Sunada, Souichi; Sunami, Kenro [Kawatetsu Chiba Hospital (Japan); Saeki, Naokatsu; Yamaura, Akira
We retrospectively reviewed MRI findings of 17 patients with 3 histologically proven cystic pituitary tumors. They consisted of 10 cystic pituitary adenomas, 4 craniopharyngiomas and 3 Rathke`s cleft cysts. We analyzed the following MRI parameters such as cyst wall appearance, enhancement pattern of cyst wall, location of residual pituitary gland and location of tumor. They were clinically significant parameters for histological differentiation. Even though combinations of such MRI parameters helped for more accurate preoperative diagnosis, the differentiation between craniopharyngioma and Rathke`s cleft cyst was difficult in some cases. (author)
Tokunaga, Hitoshi; Hoshi, Seiichiro; Sunada, Souichi; Sunami, Kenro; Saeki, Naokatsu; Yamaura, Akira
We retrospectively reviewed MRI findings of 17 patients with 3 histologically proven cystic pituitary tumors. They consisted of 10 cystic pituitary adenomas, 4 craniopharyngiomas and 3 Rathke's cleft cysts. We analyzed the following MRI parameters such as cyst wall appearance, enhancement pattern of cyst wall, location of residual pituitary gland and location of tumor. They were clinically significant parameters for histological differentiation. Even though combinations of such MRI parameters helped for more accurate preoperative diagnosis, the differentiation between craniopharyngioma and Rathke's cleft cyst was difficult in some cases. (author)
Full Text Available Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs, is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI, only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.
Song, Ji-Yeon; Mun, Sue-Jean; Sung, Soon-Ki; Hwang, Jae-Yeon; Baik, Seung-Kug; Kim, Jee Yeon; Cheon, Chong-Kun; Kim, Su-Young; Kim, Yoo-Mi
Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs), is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI), only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.
Full Text Available Pituitary apoplexy is a rare complication of pituitary tumors. We report a case of a 41-year-old female with acromegaly due to a pituitary macroadenoma, who developed pituitary apoplexy after a thyrotropin-releasing hormone (TRH 200 mg intravenous injection stimulation test. Neither emergency computed tomography (CT scans nor magnetic resonance imaging (MRI, performed 6 hours and 12 hours, respectively, after the active episode, disclosed the evidence of acute hemorrhage or infarction. Two days later, the pituitary mass, removed by transsphenoidal approach, showed ischemic necrosis and acute hemorrhage. The TRH test is generally safe for evaluating pituitary function, but pituitary apoplexy may occur after the procedure. CT and MRI may miss the diagnosis of pituitary apoplexy, especially if performed immediately after the acute episode.
Eficácia terapêutica do interferon alfa em criança com hemangioma gigante craniofacial: relato de caso Therapeutical effectiveness of interferon alpha in a child with craniofacial giant hemangioma: case report
Nilson Lopes da Fonseca Junior
Full Text Available O hemangioma é o tumor benigno mais freqüente da infância. O hemangioma capilar geralmente apresenta-se como uma mancha ou tumoração violácea bem delimitada. O diagnóstico destas lesões é clínico. O hemangioma gigante é rara e extensa variação do hemangioma capilar, que geralmente ocorre em recém-nascidos e lactentes. Várias são as modalidades terapêuticas, como a injeção intralesional de corticóide, laserterapia, injeção intralesional de soluções esclerosantes, corticoterapia sistêmica, cirurgia, radioterapia e embolização. Novas modalidades terapêuticas têm sido desenvolvidas, com o objetivo de se obter melhores resultados e possibilitar o tratamento de lesões de difícil acesso cirúrgico e refratárias às modalidades terapêuticas utilizadas rotineiramente. Os melhores resultados tem sido obtidos com o interferon alfa. Este é um caso de uma paciente com três meses de idade, que apresentava desde o nascimento, tumoração arroxeada e amolecida em pálpebra superior do olho direito, lesões cutâneas planas e arroxeadas em região temporal e parietal direita. Realizada tomografia computadorizada de crânio evidenciando processo expansivo orbitário vascularizado com extensão para fossa média, seio cavernoso e fossa posterior. O tratamento inicial foi a corticoterapia oral durante quarenta dias, com redução progressiva por quatro semanas. Com o quadro praticamente inalterado, foi iniciado o tratamento com interferon alfa, na dose de 3.000.000 U/m², subcutâneo, três vezes por semana. Após 9 meses de tratamento, observa-se apenas uma pequena lesão orbitária residual. Neste caso, o interferon alfa apresentou-se como boa opção no tratamento do hemangioma gigante craniofacial.Hemangiomas are the most commom benign tumors of infancy. Capillary hemangioma generally is presented as a spot or well-defined purple lesion. The diagnosis of these tumors is based on physical examination. Giant hemangioma is a rare
Watt, Andrew J; Chung, Kevin C
Localized gigantism poses a challenging surgical dilemma, and it may be treated with amputation. This case report documents the application of a reconstructive approach to a severe case of pedal macrodystrophia lipomatosa in a 1-year-old girl. A series of 3 surgeries were designed to reduce the length, width, height, and overall bulk of the congenitally enlarged foot. The 3 procedures debulked the foot for normal ambulation and same-size shoe wear for both feet. The resulting functional and aesthetic improvements achieved through reconstructive treatment provided a desirable alternative to amputation.
Cattaneo, F A; Fahie-Wilson, M N
The authors report the case of a 37-year-old woman who presented with amenorrhea and an increased level of serum prolactin. Magnetic resonance images of the pituitary revealed a lesion with characteristics consistent with those of a microadenoma. Transsphenoidal exploration was performed, but a prolactinoma was not found. After endocrinological review, the patient's hyperprolactinemia was found to be caused by the presence of macroprolactin and her amenorrhea was due to intense exercise and low body weight. Macroprolactin is an isoform of prolactin that is variably reactive in assays for prolactin, but displays minimum bioactivity in vivo. Patients with macroprolactin are mostly asymptomatic. This phenomenon may cause elevated prolactin values, which the authors view as apparent hyperprolactinemia. The presence of macroprolactin is an underrecognized problem, occurring in as many as 15 to 20% of patients with elevated prolactin values and often leading to unnecessary, expensive diagnostic procedures and inappropriate treatment. The presence of macroprolactin should always be suspected when the patient's clinical history or clinical or radiological data are incompatible with the prolactin value. Physicians dealing with diagnosis and treatment of hyperprolactinemia (general practitioners, gynecologists, neurosurgeons, endocrinologists, and biochemists) should be aware of the potentially misleading nature of macroprolactin.
Ozawa, Y; Kameya, T; Kasuga, A; Naritaka, H; Kanda, N; Maruyama, H; Saruta, T
A 38-yr-old female with a TSH- and GH-secreting pituitary adenoma is described, who had both overt symptoms, hyperthyroidism and acromegaly. Her serum TSH was not suppressed despite high concentrations of free T3 and free T4, and her alpha-subunit/TSH molar ratio was high. Her serum GH was consistently high, and was not suppressed by an oral glucose tolerance test. Preoperative testing revealed that, although the TSH response was impaired, TSH, alpha-subunit and GH were increased by TRH injection, and that these hormones were reduced by bromocriptine or somatostatin analog. Although she did not have hyperprolactinemia, the in vitro culture and immunohistochemical studies revealed that the adenoma cells produced and released PRL, in addition to TSH, alpha-subunit and GH. Immunohistochemical studies showed the presence of GH in the cytoplasm of many adenoma cells. TSH beta-positive adenoma cells were less frequently seen than GH-positive adenoma cells. No cells showed the coexistence of GH and TSH beta, and a few cells were positive for PRL. By electron microscopy, the adenoma was found to be composed of a single cell type resembling thyrotrophs, and did not have any characteristics of somatotrophs. This case was considered to be of interest, because the adenoma was ultrastructurally monomorphous, but immunohistochemically polymorphous.
Mammis, Antonios; Eloy, Jean Anderson; Liu, James K
Giants have been a subject of fascination throughout history. Whereas descriptions of giants have existed in the lay literature for millennia, the first attempt at a medical description was published by Johannes Wier in 1567. However, it was Pierre Marie, in 1886, who established the term "acromegaly" for the first time and established a distinct clinical diagnosis with clear clinical descriptions in 2 patients with the characteristic presentation. Multiple autopsy findings revealed a consistent correlation between acromegaly and pituitary enlargement. In 1909, Harvey Cushing postulated a “hormone of growth" as the underlying pathophysiological trigger involved in pituitary hypersecretion in patients with acromegaly. This theory was supported by his observations of clinical remission in patients with acromegaly in whom he had performed hypophysectomy. In this paper, the authors present some of the early accounts of acromegaly and gigantism, and describe its historical evolution as a medical and surgical entity.
Ada Arleny Pérez Mayo
Full Text Available Se presenta el caso de un paciente de 87 años, intervenido por primera vez en el Hospital Nacional “San Pedro Necta” del Departamento de Huehuetenango en Guatemala, por presentar hidrocele gigante de más de veinte años de evolución. El examen físico mostró tumoración translúcida dolorosa en la bolsa escrotal izquierda, con el posible diagnóstico de hidrocele tabicado o no comunicante. El preoperatorio consistió en la canalización de vena periférica, administración de profilaxis antibiótica con ceftriaxona y rasurado de la piel. Durante el acto operatorio se constató hidrocele izquierdo, con acumulación de líquido acuoso de la túnica vaginal que rodeaba el testículo, cuantificable alrededor de los 500 ml. La técnica quirúrgica consistió en la eversión o plicatura de la vaginal. No se presentaron entidades asociadas, como quiste del cordón, hernia inguinal o fimosis. No existieron complicaciones. Se reportó una estadía hospitalaria de cuatro días y el paciente evolucionó favorablemente. No ocurrió recidiva durante el año de seguimiento
Holck, S; Wewer, U M; Albrechtsen, R
Light bodies are large cytoplasmic granules originally described in the gonadotrophic cells of the rat pituitary gland. In order to determine whether similar bodies occur in the human anterior pituitary gland, 89 pituitary adenomas and periadenomatous tissue from 20 cases were examined...... cells in periadenomatous tissue from 20 cases. These results show that some human pituitary adenomas may contain light bodies identical to those seen in gonadotrophs of rat pituitary....... by transmission electron microscopy. Double membrane bound bodies with filamentous internal structure identical to rodent light bodies were identified in 10 hormone-producing adenomas: 5 PRL, 1 PRL-GH, 2 GH, and 2 ACTH-producing tumours. No light bodies were found in the remaining 79 tumours nor in the pituitary...
Full Text Available Objective To explore the value of pituitary MRI in diagnosis of etiology and prognosis in children with short stature. Methods The MRI data of 130 children with short stature admitted from Jan. to Dec. 2012 were retrospectively analyzed. Of the 130 children, 79 were males and 51 were females, aged 3 to 18 years with mean of 9.8 years. Results Of the 130 children, 82 cases (63.1% were shown to have normal pituitary morphology and signal manifestation, and in 48 cases (36.9% pituitary morphology and signal manifestation were abnormal, and among them pituitary dysplasia was found in 30 cases, deficiency of bright signals in posterior pituitary lobe was found in 4 cases, in whom pituitary stalk deficiency was found in 2 cases. Pituitary microadenoma was found in 3 cases, and pituitary cystic lesions were found in 6 cases. Suprasellar cistern hernia was found in 4 cases, and empty sella was found in one case. The height of pituitary glands was 3.00-7.00mm in children with normal pituitary morphology and signal manifestation. Conclusion MRI pituitary examination can clearly display the anatomy and the signal of the pituitary gland, therefore MR imaging is of important value in the diagnosis of the etiology diagnosis, treatment, and prognosis of children with short stature. It should be the preferred examination. DOI: 10.11855/j.issn.0577-7402.2013.11.008
Aljabban, Lama; Kassab, Lina; Bakoura, Nour Alhuda; Alsalka, Mohammad Fayez; Maksoud, Ismaeil
Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome is a rare pediatric disorder with a variable sequence of clinical presentations, undefined etiology, and high risk of mortality. Our patient presented an unusual course of the disease accompanied by a homogenous mild enlargement of her pituitary gland with an intact pituitary-endocrine axis which, to the best of our knowledge, represents a new finding in rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome. We present a documented case of a 4 years and 8-month-old Syrian Arabic girl with a distinctive course of signs and symptoms of rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome accompanied by mature ganglioneuroma in her chest, a homogenous mild enlargement of her pituitary gland, generalized cortical brain atrophy, and seizures. Three months after her first marked symptoms were noted she had a sudden progression of severe respiratory distress that ended with her death. The findings of this case could increase our understanding of the pathogenetic mechanisms of rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation, and place more emphases on pediatricians to consider rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome whenever early rapid onset of obesity, associated with any malfunction, is observed in children. This knowledge could be lifesaving for children with rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation syndrome.
Lazaro, C.M.; Philippine General Hospital, Manila; Guo, W.Y.; Sami, M.; Hindmarsch, T.; Ericson, K.; Hulting, A.L.; Wersaell, J.
In a group of 69 patients with pituitary tumours, 12 were found to have evidence of intratumoral haemorrhage on MRI, characterized by high signal intensity on short TR/TE sequences. This was verified in all but 1 patient. The majority of the bleedings occurred in macroadenomas. Five (42%) were prolactinomas and 4 (33%) were non-functioning adenomas. There were 2 GH- and 1 ACTH-secreting tumours. All 5 patients with prolactinomas were on bromocriptine medication. Two of the patients had a clinical picture of pituitary apoplexy. The haemorrhage was not large enough to prompt surgery in any of the patients. However, surgical verification of the diagnosis was obtained in 5 cases, while 6 patients were examined with follow-up MRI. (orig.)
Castro, Douglas Guedes de; Salvajoli, Joao Victor; Canteras, Miguel Montes; Cecilio, Soraya A. Jorge
Pituitary adenomas represent nearly 15% of all intracranial tumors. Multimodal treatment includes microsurgery, medical management and radiotherapy. Microsurgery is the primary recommendation for nonfunctioning and most of functioning adenomas, except for prolactinomas that are usually managed with dopamine agonist drugs. However, about 30% of patients require additional treatment after microsurgery for recurrent or residual tumors. In these cases, fractionated radiation therapy has been the traditional treatment. More recently, radiosurgery has been established as a treatment option. Radiosurgery allows the delivery of prescribed dose with high precision strictly to the target and spares the surrounding tissues. Therefore, the risks of hypopituitarism, visual damage and vasculopathy are significantly lower. Furthermore, the latency of the radiation response after radiosurgery is substantially shorter than that of fractionated radiotherapy. The goal of this review is to define the efficacy, safety and role of radiosurgery for treatment of pituitary adenomas and to present the preliminary results of our institution. (author)
Full Text Available A congenital disorder with variable manifestations, including partial gigantism of the hands and feet with hypertrophy of soles, nevi, hemihypertrophy, gynecomastia, macrocephaly and other skull abnormalities, and abdominal lipomatosis. The cause is unknown, although a genetic origin, generally of autosomal-dominant transmission, has been conjectured. Symptoms can be treated, but there is no known cure. We present the case of a young male with grotesque overgrowth of the right lower limb, splenomegaly and multiple nevi. Angiography revealed venous malformation within the limb. The findings are in conformity to the criteria for the Proteus syndrome.
Chakrabarti, Nandini; Chattopadhyay, Chandan; Bhuban, Majhi; Pal, Salil Kumar
A congenital disorder with variable manifestations, including partial gigantism of the hands and feet with hypertrophy of soles, nevi, hemihypertrophy, gynecomastia, macrocephaly and other skull abnormalities, and abdominal lipomatosis. The cause is unknown, although a genetic origin, generally of autosomal-dominant transmission, has been conjectured. Symptoms can be treated, but there is no known cure. We present the case of a young male with grotesque overgrowth of the right lower limb, splenomegaly and multiple nevi. Angiography revealed venous malformation within the limb. The findings are in conformity to the criteria for the Proteus syndrome.
Fahimeh Soheilipour; Hossein Ghalaenov; Seyyed-Hossein Samedanifard; Fatemeh Jesmi
The importance of assessing precocious puberty, especially in boys, is not only due to the great complications it has for the affected patients, but also to the fatal underlying diseases. Therefore, children with central precocious puberty should first undergo neuroimaging. In this case study, we present a 9.5-year-old boy who was referred to Rasoul-e-Akram Medical Center with increased intracranial pressure, nausea/vomiting, and severe headache having begun three months earlier. ...
Cabral, Luciano Gusmao; Monteiro, Soraya Silveira; Rossi, Luiz Antonio; Araujo, Marcelo Antonio Nobrega; Iagarachi, Celso Kendy; Oliveira, Luiz Antonio Nunes de [Hospital do Servidor Publico Estadual de Sao Paulo, SP (Brazil). Serv. de Radiologia
The authors present a case of giant aneurysm of internal carotid artery with extensive bone erosion, which led to a fistula to the paranasal sinuses. The clinical aspects, radiological findings and differential diagnosis of giant intracranial aneurysms are discussed. (author) 9 refs., 5 figs.
Jing-min CHENG; Jian-wen GU; Yong-qin KUANG; Wei-qi HE; Xue-min XING; Hai-dong HUANG; Yuan MA; Xun XIA; Tao YANG; Xiu-zhong ZHANG; Lin CHENG; Fan-jun ZENG
Objective To explore the operative method and therapeutic efficacy of surgical resection of large invasive pituitary adenomas with individualized approach under neuronavigator guidance.Methods Seventeen patients(10 males and 7 females,aged from 22 to 78 years with a mean of 39.2±9.2 years) suffering from large invasive pituitary adenoma of higher than Hardy IV grade hospitalized from 2004 to 2009 were involved in the present study.All procedures were performed with the assistance of neuronavi...
George Jyothis T
Full Text Available Abstract Introduction Hyperthyroidism is a well established cause of atrial fibrillation (AF. Thyroid Stimulating Hormone-secreting pituitary tumours are rare causes of pituitary hyperthyroidism. Whilst pituitary causes of hyperthyroidism are much less common than primary thyroid pathology, establishing a clear aetiology is critical in minimising complications and providing appropriate treatment. Measuring Thyroid Stimulating Hormone (TSH alone to screen for hyperthyroidism may be insufficient to appropriately evaluate the thyroid status in such cases. Case presentation A 63-year-old Caucasian man, previously fit and well, presented with a five-day history of shortness of breath associated with wheeze and dry cough. He denied symptoms of hyperthyroidism and his family, social and past history were unremarkable. Initial investigation was in keeping with a diagnosis of atrial fibrillation (AF with fast ventricular response leading to cardiac decompensation. TSH 6.2 (Normal Range = 0.40 – 4.00 mU/L, Free T3 of 12.5 (4.00 – 6.8 pmol/L and Free T4 51(10–30 pmol/L. Heterophilic antibodies were ruled out. Testosterone was elevated at 43.10 (Normal range: 10.00 – 31.00 nmol/L with an elevated FSH, 18.1 (1.0–7.0 U/L and elevated LH, 12.4 (1.0–8.0 U/L. Growth Hormone, IGF-1 and prolactin were normal. MRI showed a 2.4 cm pituitary macroadenoma. Visual field tests showed a right inferotemporal defect. While awaiting neurosurgical removal of the tumour, the patient was commenced on antithyroid medication (carbimazole and maintained on this until successful trans-sphenoidal excision of the macroadenoma had been performed. AF persisted post-operatively, but was electrically cardioverted subsequently and he remains in sinus rhythm at twelve months follow-up off all treatment. Conclusion This case reiterates the need to evaluate thyroid function in all patients presenting with atrial fibrillation. TSH-secreting pituitary adenomas must be considered
Faucz, Fabio R; Horvath, Anelia D; Azevedo, Monalisa F; Levy, Isaac; Bak, Beata; Wang, Ying; Xekouki, Paraskevi; Szarek, Eva; Gourgari, Evgenia; Manning, Allison D; de Alexandre, Rodrigo Bertollo; Saloustros, Emmanouil; Trivellin, Giampaolo; Lodish, Maya; Hofman, Paul; Anderson, Yvonne C; Holdaway, Ian; Oldfield, Edward; Chittiboina, Prashant; Nesterova, Maria; Biermasz, Nienke R; Wit, Jan M; Bernard, Daniel J; Stratakis, Constantine A
IGSF1 is a membrane glycoprotein highly expressed in the anterior pituitary. Pathogenic mutations in the IGSF1 gene (on Xq26.2) are associated with X-linked central hypothyroidism and testicular enlargement in males. In this study, we tested the hypothesis that IGSF1 is involved in the development of pituitary tumors, especially those that produce growth hormone (GH). IGSF1 was sequenced in 21 patients with gigantism or acromegaly and 92 healthy individuals. Expression studies with a candidate pathogenic IGSF1 variant were carried out in transfected cells and immunohistochemistry for IGSF1 was performed in the sections of GH-producing adenomas, familial somatomammotroph hyperplasia, and in normal pituitary. We identified the sequence variant p.N604T, which in silico analysis suggested could affect IGSF1 function, in two male patients and one female with somatomammotroph hyperplasia from the same family. Of 60 female controls, two carried the same variant and seven were heterozygous for other variants. Immunohistochemistry showed increased IGSF1 staining in the GH-producing tumor from the patient with the IGSF1 p.N604T variant compared with a GH-producing adenoma from a patient negative for any IGSF1 variants and with normal control pituitary tissue. The IGSF1 gene appears polymorphic in the general population. A potentially pathogenic variant identified in the germline of three patients with gigantism from the same family (segregating with the disease) was also detected in two healthy female controls. Variations in IGSF1 expression in pituitary tissue in patients with or without IGSF1 germline mutations point to the need for further studies of IGSF1 action in pituitary adenoma formation. © 2015 Society for Endocrinology.
Keil, Margaret F.; Stratakis, Constantine A.
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
Philip C Johnston
Full Text Available Tumours metastasizing to the pituitary gland are uncommon. Symptomatic patients with pituitary metastases can present with diabetes insipidus, headache, visual field defects and/or anterior pituitary hormonal dysfunction. Treatment options for pituitary metastases include, surgical resection, cranial or parasellar irradiation and/or chemotherapy, and hormonal replacement if indicated. The overall prognosis of pituitary metastases is poor. We present a case of hypopituitarism as the presenting feature of bronchogenic carcinoma with metastases to the pituitary gland.
Domingue, Marie-Eve; Marbaix, Etienne; Do Rego, Jean-Luc; Col, Vincent; Raftopoulos, Christian; Duprez, Thierry; Vaudry, Hubert; Maiter, Dominique
Pituitary gangliocytomas are uncommon neuronal tumours that may present with endocrine disorders, the most frequent being acromegaly caused by growth hormone hypersecretion. Cushing's syndrome is very rarely seen with gangliocytomas. We report the unique case of a 62 year-old woman whose clinical picture and endocrine testing clearly demonstrated adrenocorticotropin (ACTH)-dependent Cushing's syndrome. Pituitary magnetic resonance imaging showed a 12-mm homogeneous, infra- and retrosellar mass first diagnosed as pituitary macroadenoma. Transsphenoidal surgery was performed and allowed complete resection of the tumour with sparing of normal anterior pituitary. Very low postoperative serum cortisol and ACTH levels were observed in the early postoperative period and the patient is still in remission 18 months after surgery, thus demonstrating that the resected lesion was entirely responsible for the clinical picture. Histological and immunocytochemical analyses demonstrated a benign tumour composed of mature neuronal cells suggestive of a gangliocytoma, expressing both ACTH and corticotropin-releasing hormone (CRH). The tumour was surrounded by a rim of pituitary tissue containing ACTH-producing endocrine cells. Careful analysis of the resected lesion did not reveal any pituitary microadenoma. We search literature for similar cases and retraced only nine cases of gangliocytomas associated with Cushing's syndrome. In most of them, the tumour was combined with either pituitary corticotroph adenoma or hyperplasia. Our case represents a unique case of an infrasellar pituitary gangliocytoma which was able to cause Cushing's syndrome by both direct ACTH production and CRH-induced stimulation of neighbour normal corticotroph cells.
Jialal, I; Nathoo, B C; Joubert, S; Asmal, A C; Pillay, N L
Over a 5-year period 14 patients with acromegaly and gigantism were seen at the endocrine clinic of King Edward VIII Hospital: 9 were Blacks and 5 Indians; 8 of the patients were women. The mean age of the patients was 46 years. Surprisingly, only 2 patients complained of acral overgrowth. Symptomatology was varied and not characteristic of the condition. On examination all patients had unequivocal signs of soft-tissue and bony overgrowth, 64% had visual abnormalities and 50% hypertension. Radiologically, 88% showed an enlarged pituitary fossa. On biochemical investigation, the fasting levels of growth hormone (GH) were increased in 12 patients and during oral glucose tolerance tests, the GH levels in these 12 patients were not suppressed. One patient in whom the fasting GH level was not increased had progressed to the stage of panhypopituitarism, in the remaining patient challenge with thyrotrophin-releasing hormone (TRH) led to increased GH levels and L-dopa challenge resulted in a paradoxical decrease in GH levels. Seven patients with increased GH levels who were challenged with L-dopa showed the typical decrease in GH levels found in this condition; in 5 of these patients, challenged with TRH, GH levels increased. The findings emphasize that despite the ease of clinical diagnosis, appropriate biochemical investigations are necessary to confirm the exact status of the disease, which is rare in the population studied.
Full Text Available The importance of assessing precocious puberty, especially in boys, is not only due to the great complications it has for the affected patients, but also to the fatal underlying diseases. Therefore, children with central precocious puberty should first undergo neuroimaging. In this case study, we present a 9.5-year-old boy who was referred to Rasoul-e-Akram Medical Center with increased intracranial pressure, nausea/vomiting, and severe headache having begun three months earlier. The development of secondary sexual changes had started two years earlier, and had been neglected. His testes, penis, and pubic hair were at the fourth Tanner stage. He had elevated luteinizing and follicle stimulating hormones. Microscopic evaluation confirmed low-grade pilocytic astrocytoma WHO grade 1. Emergency brain surgery was conducted in which the brain was decompressed, and chemotherapy was started postoperatively. Two years after the surgery, he remains under chemotherapy, with obvious sexual maturation and a height of 154 cm. Training families and medical staff efficiently can help prevent the late diagnosis and treatment of precocious puberty and, as a result, help patients in their social life.
Chentli, Farida; Bey, Abderrahim; Belhimer, Faiza; Azzoug, Said
Pituitary gigantism is a very rare condition; the occurrence of pituitary apoplexy in children younger than 10 years old is even rarer. The aim of our study is to report this exceptional association. A boy aged 9 years and 6 months was hospitalized for the first time in November 2011 for symptoms suggesting pituitary apoplexy. The onset of his disease was difficult to determine as his health record has been poorly maintained. On October 10, 2011, he presented to an emergency department with a sudden drop of visual acuity with diplopia and retro-orbital headaches. An ophthalmological exam found very low visual acuity (1/20) with papillary edema. An MRI of the patient's brain revealed a hemorrhagic pituitary process reaching the chiasma, which was compressed, especially on the right side. Thereafter, the patient's vision improved spontaneously. Clinical examination was normal except for gigantism (+5 SD compared to the target stature). Hormonal assessment argued for mixed secretion [growth hormone (GH) = 39 ng/mL, n ≤ 5, prolactin ( PRL) = 470 ng/mL, n gigantism: phenomenon quite exceptional and worth to be reported.
Full Text Available The pituitary gland’s role as a functional matrix for sella turcica has not been suggested in orthodontic literature. This paper is an attempt to correlate the role of pituitary gland in the development of sella turcica. A case report of dwarfism associated with hypopituitarism is presented to highlight the above hypothesis.
Salvatori, R.; Radian, S.; Diekmann, Y.; Iacovazzo, D.; David, A.; Grabovska, P.; Grassi, G.; Bussell, A-M; Stals, K.; Weber, A.; Quinton, R.; Crowne, E.; Corazzini, V.; Metherell, L. A.; Kearney, T.
OBJECTIVE: Mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are associated with pituitary adenoma, acromegaly and gigantism. Identical alleles in unrelated pedigrees could be inherited from a common ancestor or result from recurrent mutation events. DESIGN & METHODS: Observational, inferential and experimental study, including: AIP mutation testing; reconstruction of 14 AIP-region (8.3 Mbp) haplotypes; coalescent-based approximate Bayesian estimation of the time to mo...
Salvatori, Roberto; Radian, Serban; Diekmann, Yoan; Iacovazzo, Donato; David, Alessia; Gabrovska, Plamena; Grassi, Giorgia; Bussell, Anna-Marie; Stals, Karen; Weber, Astrid; Quinton, Richard; Crowne, Elizabeth C; Corazzini, Valentina; Metherell, Lou; Kearney, Tara
Objective Mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are associated with pituitary adenoma, acromegaly and gigantism. Identical alleles in unrelated pedigrees could be inherited from a common ancestor or result from recurrent mutation events. Design and methods Observational, inferential and experimental study, including: AIP mutation testing; reconstruction of 14 AIP-region (8.3?Mbp) haplotypes; coalescent-based approximate Bayesian estimation of the time to mo...
Xu, Chao; Zhang, Xinxian; Dong, Lina; Zhu, Bin; Xin, Tao
We verified the advantages of using magnetic resonance imaging (MRI) for improving the diagnostic quality of growth hormone deficiency (GHD) in children with short stature caused by pituitary lesions. Clinical data obtained from 577 GHD patients with short stature caused by pituitary lesions were retrospectively analyzed. There were 354 cases (61.3%) with anterior pituitary dysplasia; 45 cases (7.8%) of pituitary stalk interruption syndrome (PSIS); 15 cases (2.6%) of pituitary hyperplasia due...
Eun Kyoung Lee
Full Text Available A 53-year-old woman presented with complaints of blurred vision in the left eye. She had been treated for recurrent non-functioning pituitary adenoma (NFPA. A vitreous biopsy followed by histopathologic examination showed the presence of Candida albicans. Meanwhile, Cushing׳s disease was diagnosed and gamma knife surgery was performed. Vitrectomy and antifungal treatment improved ocular infection and inflammation. Herein, we describe the first case of C. albicans endophthalmitis in a patient with NFPA evolving into Cushing׳s disease.
Jesser, J.; Schlamp, K.; Bendszus, M. [Radiologische Klinik, Universitaetsklinikum Heidelberg, Abteilung fuer Neuroradiologie, Heidelberg (Germany)
This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15 % of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65 % of pituitary gland adenomas secrete hormones whereby approximately 50 % secrete prolactin, 10 % secrete growth hormone (somatotropin) and 6 % secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10 % of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland. (orig.) [German] Diese Arbeit ist eine Uebersicht ueber die haeufigsten Hypophysentumoren und deren Differenzialdiagnosen mit Augenmerk auf die
Lecoq, Anne-Lise; Zizzari, Philippe; Hage, Mirella; Decourtye, Lyvianne; Adam, Clovis; Viengchareun, Say; Veldhuis, Johannes D; Geoffroy, Valérie; Lombès, Marc; Tolle, Virginie; Guillou, Anne; Karhu, Auli; Kappeler, Laurent; Chanson, Philippe; Kamenický, Peter
Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene predispose humans to pituitary adenomas, particularly of the somatotroph lineage. Mice with global heterozygous inactivation of Aip (Aip(+/-)) also develop pituitary adenomas but differ from AIP-mutated patients by the high penetrance of pituitary disease. The endocrine phenotype of these mice is unknown. The aim of this study was to determine the endocrine phenotype of Aip(+/-) mice by assessing the somatic growth, ultradian pattern of GH secretion and IGF1 concentrations of longitudinally followed male mice at 3 and 12 months of age. As the early stages of pituitary tumorigenesis are controversial, we also studied the pituitary histology and somatotroph cell proliferation in these mice. Aip(+/-) mice did not develop gigantism but exhibited a leaner phenotype than wild-type mice. Analysis of GH pulsatility by deconvolution in 12-month-old Aip(+/-) mice showed a mild increase in total GH secretion, a conserved GH pulsatility pattern, but a normal IGF1 concentration. No pituitary adenomas were detected up to 12 months of age. An increased ex vivo response to GHRH of pituitary explants from 3-month-old Aip(+/-) mice, together with areas of enlarged acini identified on reticulin staining in the pituitary of some Aip(+/-) mice, was suggestive of somatotroph hyperplasia. Global heterozygous Aip deficiency in mice is accompanied by subtle increase in GH secretion, which does not result in gigantism. The absence of pituitary adenomas in 12-month-old Aip(+/-) mice in our experimental conditions demonstrates the important phenotypic variability of this congenic mouse model. © 2016 Society for Endocrinology.
Miki, Yukio; Nishizawa, Sadahiko; Kuroda, Yasumasa; Keyaki, Atsushi; Nabeshima, Sachio; Kawamura, Junichiro; Matsuo, Michimasa
The authors performed serial dynamic MR imaging in patients with 10 normal pituitary and 21 pituitary adenoma utilizing spin-echo sequence with a very short repetition time (SE 100/15) every minute immediately after a bolus injection of Gd-DTPA. Usual T 1 -weighted images (SE 600/15) were also obtained before and after the dynamic study. Pituitary adenomas included 10 adenomas confirmed by surgery, 4 adenomas confirmed by biologic data, and 7 postoperative adenomas. Out of 10 patients who underwent surgery after dynamic MRI, 9 patients underwent postoperative dynamic MRI. In normal patients, the pituitary gland was markedly enhanced on the early-phase images of the dynamic study, followed by gradual decrease of intensity throughout the dynamic study. In cases of microadenomas, the contrast between the normal pituitary gland and adenoma is better than that on the usual T 1 -weighted images by marked enhancement of the normal pituitary gland. Dynamic images clearly showed the residual normal pituitary glands in all cases of macroadenoma larger than 15 mm in diameter, whereas usual contrast-enhanced images showed the normal pituitary gland only in one case. In all patients who underwent both preoperative and postoperative dynamic MRI, postoperative dynamic MRI showed the normal pituitary glands which are markedly enhanced on the early-phase images in the sites which correspond to the preoperative dynamic study. The normal residual anterior gland was also visualized in four out of 7 patients who received only postoperative dynamic MRI. Dynamic MRI is a strong diagnostic modality for visualizing microadenoma and for visualizing the normal pituitary gland in cases of preoperative and postoperative macroadenoma. (author)
Herczeg, Gábor; Gonda, Abigél; Merilä, Juha
The relaxation of predation and interspecific competition are hypothesized to allow evolution toward "optimal" body size in island environments, resulting in the gigantism of small organisms. We tested this hypothesis by studying a small teleost (nine-spined stickleback, Pungitius pungitius) from four marine and five lake (diverse fish community) and nine pond (impoverished fish community) populations. In line with theory, pond fish tended to be larger than their marine or lake conspecifics, sometimes reaching giant sizes. In two geographically independent cases when predatory fish had been introduced into ponds, fish were smaller than those in nearby ponds lacking predators. Pond fish were also smaller when found in sympatry with three-spined stickleback (Gasterosteus aculeatus) than those in ponds lacking competitors. Size-at-age analyses demonstrated that larger size in ponds was achieved by both increased growth rates and extended longevity of pond fish. Results from a common garden experiment indicate that the growth differences had a genetic basis: pond fish developed two to three times higher body mass than marine fish during 36 weeks of growth under similar conditions. Hence, reduced risk of predation and interspecific competition appear to be chief forces driving insular body size evolution toward gigantism.
Two cases of metastasis in the pituitary stalk are presented. In both cases, presenting with diabetes insipidus, the demonstration of a small (under 1 cm) metastasis in the pituitary stalk was possible using a current generation scanner. Patients with known primary tumors presenting with a diabetes insipidus should be examined by CT before therapy with antidiuretic hormone is initiated. In cases of visible metastasis, a specific therapy together with the hormone-substitution should be discussed.
Naves, Luciana A.; Daly, Adrian Francis; Dias, Luiz Augusto; Yuan, Bo; Zakir, Juliano Coelho Oliveira; Barra, Gustavo Barcellos; Palmeira, Leonor; Villa, Chiara; Trivellin, Giampaolo; Junior, Armindo Jreige; Neto, Florencio Figueiredo Cavalcante; Liu, Pengfei; Pellegata, Natalia S.; Stratakis, Constantine A.; Lupski, James R.
X-linked acro-gigantism (X-LAG) syndrome is a newly described disease caused by microduplications on chromosome Xq26.3 leading to copy number gain of GPR101. We describe the clinical progress of a sporadic male X-LAG syndrome patient with an Xq26.3 microduplication, highlighting the aggressive natural history of pituitary tumor growth in the absence of treatment. The patient first presented elsewhere aged 5 years 8 months with a history of excessive growth for >2 years. His height was 163 cm,...
Full Text Available Ji-wen Wang,1,2 Ying Li,3 Zhi-gang Mao,1,2 Bin Hu,1,2 Xiao-bing Jiang,1,2 Bing-bing Song,4 Xin Wang,4 Yong-hong Zhu,4 Hai-jun Wang1,21Department of Neurosurgery and Pituitary Tumor Center, The First Affiliated Hospital, Sun Yat-sen University, 2Key Laboratory of Pituitary Adenoma in Guangdong Province, 3State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, 4Department of Histology and Embryology, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, People's Republic of ChinaAbstract: Excessive growth hormone (GH is usually secreted by GH-secreting pituitary adenomas and causes gigantism in juveniles or acromegaly in adults. The clinical complications involving cardiovascular, respiratory, and metabolic systems lead to elevated morbidity in acromegaly. Control of serum GH and insulin-like growth factor (IGF 1 hypersecretion by surgery or pharmacotherapy can decrease morbidity. Current pharmacotherapy includes somatostatin analogs (SAs and GH receptor antagonist; the former consists of lanreotide Autogel (ATG and octreotide long-acting release (LAR, and the latter refers to pegvisomant. As primary medical therapy, lanreotide ATG and octreotide LAR can be supplied in a long-lasting formulation to achieve biochemical control of GH and IGF-1 by subcutaneous injection every 4–6 weeks. Lanreotide ATG and octreotide LAR provide an effective medical treatment, whether as a primary or secondary therapy, for the treatment of GH-secreting pituitary adenoma; however, to maximize benefits with the least cost, several points should be emphasized before the application of SAs. A comprehensive assessment, especially of the observation of clinical predictors and preselection of SA treatment, should be completed in advance. A treatment process lasting at least 3 months should be implemented to achieve a long-term stable blood concentration. More satisfactory surgical outcomes for noninvasive macroadenomas treated
... hormones. They can press on or damage the pituitary gland and prevent it from secreting adequate levels of hormones. National Institute of Neurological Disorders and Stroke. (2010). NINDS pituitary tumors information page . ...
Appel, W; Scharphuis, T; Distelmaier, P
This is a report on a rare case of a recurring abscess in the pituitary gland. Diagnosis was extremely difficult to establish preoperatively. This is demonstrated via various radiological methods. Anamnesis and clinical disease pattern are important assisting factors in diagnosis.
Rehfeld, J F; Lindholm, J; Andersen, B N
We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained......'s disease and 7 acromegaly with adenomas containing ACTH. The cholecystokinin peptides from the tumors were smaller and less sulfated than cholecystokinin from normal pituitary glands. We conclude that ACTH-producing pituitary cells may also produce an altered form of cholecystokinin....
Ronald M Bonett
Full Text Available The Amphiumidae contains three species of elongate, permanently aquatic salamanders with four diminutive limbs that append one, two, or three toes. Two of the species, Amphiuma means and A. tridactylum, are among the largest salamanders in the world, reaching lengths of more than one meter, whereas the third species (A. pholeter, extinct amphiumids, and closely related salamander families are relatively small. Amphiuma means and A. tridactylum are widespread species and live in a wide range of lowland aquatic habitats on the Coastal Plain of the southeastern United States, whereas A. pholeter is restricted to very specialized organic muck habitats and is syntopic with A. means. Here we present analyses of sequences of mitochondrial and nuclear loci from across the distribution of the three taxa to assess lineage diversity, relationships, and relative timing of divergence in amphiumid salamanders. In addition we analyze the evolution of gigantism in the clade. Our analyses indicate three lineages that have diverged since the late Miocene, that correspond to the three currently recognized species, but the two gigantic species are not each other's closest relatives. Given that the most closely related salamander families and fossil amphiumids from the Upper Cretaceous and Paleocene are relatively small, our results suggest at least two extreme changes in body size within the Amphuimidae. Gigantic body size either evolved once as the ancestral condition of modern amphiumas, with a subsequent strong size reduction in A. pholeter, or gigantism independently evolved twice in the modern species, A. means and A. tridactylum. These patterns are concordant with differences in habitat breadth and range size among lineages, and have implications for reproductive isolation and diversification of amphiumid salamanders.
Full Text Available The basal cell carcinoma is the most common skin cancer but the giant vegetating basal cell carcinoma reaches less than 0.5 % of all basal cell carcinoma types. The Giant BCC, defined as a lesion with more than 5 cm at its largest diameter, is a rare form of BCC and commonly occurs on the trunk. This patient, male, 42 years old presents a Giant Basal Cell Carcinoma which reaches 180 cm2 on the right shoulder and was negligent in looking for treatment. Surgical treatment was performed and no signs of dissemination or local recurrence have been detected after follow up of five years.O carcinoma basocelular é o tipo mais comum de câncer de pele, mas o carcinoma basocelular gigante vegetante não atinge 0,5% de todos os tipos de carcinomas basocelulares. O Carcinoma Basocelular Gigante, definido como lesão maior que 5 cm no maior diâmetro, é uma forma rara de carcinoma basocelular e comumente ocorre no tronco. Este paciente apresenta um Carcinoma Basocelular Gigante com 180cm² no ombro direito e foi negligente em procurar tratamento. Foi realizado tratamento cirúrgico e nenhum sinal de disseminação ou recorrência local foi detectada após 5 anos.
Full Text Available Spindle cell oncocytoma is an infrequent benign non-endocrine sellar neoplasm. Due to its similar morphology to pituitary adenomas, consideration of this differential diagnosis would conduce to a more careful surgical approach in order to avoid intraoperative bleeding and aiming to a complete resection, on which depends long-term outcomes. We present the case of a 60-year-old male who complained about visual abnormalities, with computerized visual field confirmation. On biochemistry, a panhypopituitarism was detected. The brain magnetic resonance images showed a sellar mass. A non-functioning pituitary macroadenoma was presumptively diagnosed and due to the visual impairment, surgical transesphenoidal treatment was indicated. The histological diagnosis was spindle cell oncocytoma. Five months after surgery, the control image demonstrated a lesion that was considered as remnant tumor, hence radiosurgery was performed. During the follow-up, the tumor reduced its size and four years after initial treatment, the sellar resonance imaging showed disappearance of the residual tumor. Communication of new cases of this rare entity will enlarge the existing evidence and will help to determinate the most effective treatment and prognosis.
Radian, Serban; Diekmann, Yoan; Gabrovska, Plamena; Holland, Brendan; Bradley, Lisa; Wallace, Helen; Stals, Karen; Bussell, Anna‐Marie; McGurren, Karen; Cuesta, Martin; Ryan, Anthony W.; Herincs, Maria; Hernández‐Ramírez, Laura C.; Holland, Aidan; Samuels, Jade; Aflorei, Elena Daniela; Barry, Sayka; Dénes, Judit; Pernicova, Ida; Stiles, Craig E.; Trivellin, Giampaolo; McCloskey, Ronan; Ajzensztejn, Michal; Abid, Noina; Akker, Scott A.; Mercado, Moises; Cohen, Mark; Thakker, Rajesh V.; Baldeweg, Stephanie; Barkan, Ariel; Musat, Madalina; Levy, Miles; Orme, Stephen M.; Unterländer, Martina; Burger, Joachim; Kumar, Ajith V.; Ellard, Sian; McPartlin, Joseph; McManus, Ross; Linden, Gerard J.; Atkinson, Brew; Balding, David J.; Agha, Amar; Thompson, Chris J.; Hunter, Steven J.; Thomas, Mark G.; Morrison, Patrick J.
ABSTRACT The aryl hydrocarbon receptor interacting protein (AIP) founder mutation R304* (or p.R304*; NM_003977.3:c.910C>T, p.Arg304Ter) identified in Northern Ireland (NI) predisposes to acromegaly/gigantism; its population health impact remains unexplored. We measured R304* carrier frequency in 936 Mid Ulster, 1,000 Greater Belfast (both in NI) and 2,094 Republic of Ireland (ROI) volunteers and in 116 NI or ROI acromegaly/gigantism patients. Carrier frequencies were 0.0064 in Mid Ulster (95%CI = 0.0027–0.013; P = 0.0005 vs. ROI), 0.001 in Greater Belfast (0.00011–0.0047) and zero in ROI (0–0.0014). R304* prevalence was elevated in acromegaly/gigantism patients in NI (11/87, 12.6%, P gigantism cases. tMRCA is consistent with historical/folklore accounts of Irish giants. Forward simulations predict many undetected carriers; geographically targeted population screening improves asymptomatic carrier identification, complementing clinical testing of patients/relatives. We generated disease awareness locally, necessary for early diagnosis and improved outcomes of AIP‐related disease. PMID:27650164
Radian, Serban; Diekmann, Yoan; Gabrovska, Plamena; Holland, Brendan; Bradley, Lisa; Wallace, Helen; Stals, Karen; Bussell, Anna-Marie; McGurren, Karen; Cuesta, Martin; Ryan, Anthony W; Herincs, Maria; Hernández-Ramírez, Laura C; Holland, Aidan; Samuels, Jade; Aflorei, Elena Daniela; Barry, Sayka; Dénes, Judit; Pernicova, Ida; Stiles, Craig E; Trivellin, Giampaolo; McCloskey, Ronan; Ajzensztejn, Michal; Abid, Noina; Akker, Scott A; Mercado, Moises; Cohen, Mark; Thakker, Rajesh V; Baldeweg, Stephanie; Barkan, Ariel; Musat, Madalina; Levy, Miles; Orme, Stephen M; Unterländer, Martina; Burger, Joachim; Kumar, Ajith V; Ellard, Sian; McPartlin, Joseph; McManus, Ross; Linden, Gerard J; Atkinson, Brew; Balding, David J; Agha, Amar; Thompson, Chris J; Hunter, Steven J; Thomas, Mark G; Morrison, Patrick J; Korbonits, Márta
The aryl hydrocarbon receptor interacting protein (AIP) founder mutation R304 * (or p.R304 * ; NM_003977.3:c.910C>T, p.Arg304Ter) identified in Northern Ireland (NI) predisposes to acromegaly/gigantism; its population health impact remains unexplored. We measured R304 * carrier frequency in 936 Mid Ulster, 1,000 Greater Belfast (both in NI) and 2,094 Republic of Ireland (ROI) volunteers and in 116 NI or ROI acromegaly/gigantism patients. Carrier frequencies were 0.0064 in Mid Ulster (95%CI = 0.0027-0.013; P = 0.0005 vs. ROI), 0.001 in Greater Belfast (0.00011-0.0047) and zero in ROI (0-0.0014). R304 * prevalence was elevated in acromegaly/gigantism patients in NI (11/87, 12.6%, P acromegaly/gigantism cases. tMRCA is consistent with historical/folklore accounts of Irish giants. Forward simulations predict many undetected carriers; geographically targeted population screening improves asymptomatic carrier identification, complementing clinical testing of patients/relatives. We generated disease awareness locally, necessary for early diagnosis and improved outcomes of AIP-related disease. © 2016 The Authors. **Human Mutation published by Wiley Periodicals, Inc.
Niimi, Takayasu; Okamura, Masayoshi
The techniques and procedure for manufacturing gigantic ingots heavier than 200 t are described. Especially, practical results of an arc heating furnace which plays an important role in the procedure and segregation of gigantic ingots are discussed in detail. By appropriate operations of the arc heating furnance, hydrogen and phosphorus are kept unchanged, and oxygen and sulphur decrease to very low levels. Furthermore, the temperature can be accurately controlled. The application of multipour technique reduces segregation and its degree is dependent on kinds of steel. V-segregation and inverted V-segregation in steel deoxidized with carbon in vacuum seem to be very slight. (auth.)
Full Text Available Internal carotid artery (ICA pseudoaneurysm is a rare complication of endoscopic endonasal surgery occurring in 0.4–1.1% of cases. Pseudoaneurysms can subsequently result in other complications, such as subarachnoid hemorrhage, epistaxis, and caroticocavernous fistula with resultant death or permanent neurologic deficit. In this case, we illustrate endovascular treatment with a flow-diverting stent for an ICA pseudoaneurysm after endoscopic endonasal surgery for a pituitary adenoma in a 56-year-old male. Surgery was complicated by excessive intraoperative bleeding and emergent CT angiography confirmed an iatrogenic pseudoaneurysm on the anteromedial surface of the ICA. The pseudoaneurysm was treated endovascularly with flow-diverting stent implantation only. Follow-up CT angiography after three months demonstrated occlusion of the pseudoaneurysm.
Sicignano, Gianluisa; Losa, Marco; del Vecchio, Antonella; Cattaneo, Giovanni Mauro; Picozzi, Piero; Bolognesi, Angelo; Mortini, Pietro; Calandrino, Riccardo
Gamma Knife Surgery (GKS) can be an adjunctive option to surgery in the case of pituitary adenomas. The effect of dosimetric variables on the incidence of new anterior pituitary deficits after GKS requires better definition. This retrospective study considered 130 patients with a follow up after GKS >6 months. The diagnosis was nonfunctioning pituitary adenoma (NFPA) in 68 patients and secreting pituitary adenoma (SPA) in 62 patients. Median margin dose was 15/25 Gy for NFPA and SPA, respectively. The endocrinological median follow-up was 60 months. Hypopituitarism was defined as a new pituitary deficit in (at least) one of the three hormonal axes (hypogonadism, hypothyroidism and hypoadrenalism). The predictive value of clinical/dosimetric parameters was tested by univariate/multivariate analyses. Sixteen patients (12.3%) showed a new pituitary deficit in one or more axes. Multivariate analysis confirmed that the mean dose to the stalk/pituitary and the amount of healthy tissue within the high dose region were strong independent predictors of pituitary dysfunction; their best cut-off values were around 15.7 Gy, 7.3 Gy and 1.4 cm(3), respectively. Our data showed a dose-dependent incidence of new hormonal deficits after GKS for pituitary adenoma. During planning definition, the risk of hypopituitarism could be reduced using the outlined safe dose-volume values. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.
Sicignano, Gianluisa; Losa, Marco; Vecchio, Antonella del; Cattaneo, Giovanni Mauro; Picozzi, Piero; Bolognesi, Angelo; Mortini, Pietro; Calandrino, Riccardo
Background and purpose: Gamma Knife Surgery (GKS) can be an adjunctive option to surgery in the case of pituitary adenomas. The effect of dosimetric variables on the incidence of new anterior pituitary deficits after GKS requires better definition. Materials and methods: This retrospective study considered 130 patients with a follow up after GKS >6 months. The diagnosis was nonfunctioning pituitary adenoma (NFPA) in 68 patients and secreting pituitary adenoma (SPA) in 62 patients. Median margin dose was 15/25 Gy for NFPA and SPA, respectively. The endocrinological median follow-up was 60 months. Hypopituitarism was defined as a new pituitary deficit in (at least) one of the three hormonal axes (hypogonadism, hypothyroidism and hypoadrenalism). The predictive value of clinical/dosimetric parameters was tested by univariate/multivariate analyses. Results: Sixteen patients (12.3%) showed a new pituitary deficit in one or more axes. Multivariate analysis confirmed that the mean dose to the stalk/pituitary and the amount of healthy tissue within the high dose region were strong independent predictors of pituitary dysfunction; their best cut-off values were around 15.7 Gy, 7.3 Gy and 1.4 cm 3 , respectively. Conclusions: Our data showed a dose-dependent incidence of new hormonal deficits after GKS for pituitary adenoma. During planning definition, the risk of hypopituitarism could be reduced using the outlined safe dose–volume values.
Brandao, Rafael Augusto Castro Santiago; Dellaretti Filho, Marcos Antonio; Nunes, Tadeu Wilker; Totola, Paolo Victor Fernandes; Fonseca, Vinicius Silveira; Souza, Warley Cristiano de
Neurocysticercosis (NCC) is the most common parasitic infection of the nervous system. Considered a global epidemic, the parasite presents, mainly, as asymptomatic. Usually treatment is clinical, but in some selected cases surgical intervention is a good treatment option. In our case, keeping in view the magnitude of intracranial injury, surgery was the therapy chosen. A female patient, aged 54, Mulatto, native of Novo Cruzeiro (MG) and raised in Belo Horizonte. Presented with intense frontal headache, which progressed to right hemiparesis and seizure. Ten days after onset of symptoms, sought the service of emergency care. Tomography (CT) revealed a hypodense area in left frontoparietal region with characteristics of cystic lesion.
Xu, Chao; Zhang, Xinxian; Dong, Lina; Zhu, Bin; Xin, Tao
We verified the advantages of using magnetic resonance imaging (MRI) for improving the diagnostic quality of growth hormone deficiency (GHD) in children with short stature caused by pituitary lesions. Clinical data obtained from 577 GHD patients with short stature caused by pituitary lesions were retrospectively analyzed. There were 354 cases (61.3%) with anterior pituitary dysplasia; 45 cases (7.8%) of pituitary stalk interruption syndrome (PSIS); 15 cases (2.6%) of pituitary hyperplasia due to primary hypothyroidism; 38 cases (6.6%) of Rathke cleft cyst; 68 cases (11.8%) of empty sella syndrome; 16 cases (2.8%) of pituitary invasion from Langerhans cell histiocytosis; 2 cases (0.3%) of sellar regional arachnoid cyst and 39 cases (6.8%) of craniopharyngioma. MRI results showed that the height of anterior pituitary in patients was less than normal. Location, size and signals of posterior pituitary and pituitary stalk were normal in anterior pituitary dysplasia. In all cases pituitary hyperplasia was caused by hypothyroidism. MRI results showed that anterior pituitary was enlarged, and we detected upward apophysis and obvious homogeneous enhancement. There were no pituitary stalk interruption and abnormal signal. We also observed that after hormone replacement therapy the size of pituitary gland was reduced. Anterior pituitary atrophy was observed in Rathke cleft cyst, empty sella syndrome, sellar regional arachnoid cyst and craniopharyngioma. The microstructure of hypophysis and sellar region was studied with MRI. We detected pituitary lesions, and the characteristics of various pituitary diseases of GHD in children with short stature. It was concluded that in children with GHD caused by pituitary lesions, MRI was an excellent method for early diagnosis. This method offers clinical practicability and we believe it can be used for differential diagnosis and to monitor the therapeutic effects.
Caporalini, Chiara; Buccoliero, Anna Maria; Pansini, Luigi; Moscardi, Selene; Novelli, Luca; Baroni, Gianna; Bordi, Lorenzo; Ammannati, Franco; Taddei, Gian Luigi
Pituitary adenomas are benign tumors representing approximately 15-20% of intracranial neoplasms. There have been few reports of metaplastic osseous transformation and about 60 cases of neuronal metaplasia in pituitary adenoma but adipose metaplasia has not been previously described in the English literature. Here we report a case of pituitary adenoma with metaplastic adipose tissue in a 58-year-old male patient. Histologically this case fulfilled the criteria of a non-functioning pituitary adenoma, and moreover a central area of adipose tissue, made by mature adipocytes, and many tumor cells, containing fat droplet were evident. Lipomatous transformation of tumor cells in the CNS has been previously observed but, to the best of our knowledge, our case is the first pituitary adenoma with such change. The histogenesis of the adipose element in pituitary adenoma is not well understood, and could be a result of a metaplastic change or divergent differentiation from a common progenitor cell. © 2017 Japanese Society of Neuropathology.
Tanabe, Sumiyoshi; Uede, Teiji; Daibo, Masahiko; Niwa, Jun; Hashi, Kazuo
The advantage of high resolution CT in the diagnosis of pituitary microadenomas has been established, but the diagnosis becomes more difficult when the pituitary microadenoma is less than 5 mm in diameter. We have studied the usefulness of dynamic CT scans particularly for diagnosis of small microadenomas. The dynamic CT scans were performed for 61 normal pituitary glands and 68 pituitary adenomas (36 microadenomas, 32 macroadenomas) with a GECT/T 9800 scanner. Coronal sections of 1.5 mm thickness were taken at the plane just in front of the pituitary stalk of the pituitary gland. Following a bolus intra-venous injection of 40 - 60 ml of contrast media using an automatic injector, ten consecutive CT scans of 2 seconds scan time were obtained, beginning 2 seconds from the start of intravenous injection. The first seven scans were taken with an interval of 2.3 seconds, and the last three scans with an interval of 10 seconds. Then, time-density curves were obtained at the ROI which were set on the anterior pituitary gland, the vascular bed of the pituitary gland, the pituitary stalk and the area of the pituitary adenoma respectively. In a normal pituitary gland, the density increases gradually and makes an S shaped curve, then attains the maximum density value (92.3 CT number) approximately 60 seconds after the administration of contrast media. The pituitary vascular bed is located in midline on the upper surface of the pituitary gland, and shows a symmetrical square, triangular or rhomboid shape. In case of pituitary adenoma, the time-density curve makes a plateau curve and attains the maximum density value (60.1 CT number) approximately 60 seconds after the administration of contrast media. In microadenoma, the pituitary vascular bed is either deformed or compressed, showing an asymmetrical shape. (author)
Full Text Available The interaction between fungal pathogens with the host frequently results in morphological changes, such as hyphae formation. The encapsulated pathogenic fungus Cryptococcus neoformans is not considered a dimorphic fungus, and is predominantly found in host tissues as round yeast cells. However, there is a specific morphological change associated with cryptococcal infection that involves an increase in capsule volume. We now report another morphological change whereby gigantic cells are formed in tissue. The paper reports the phenotypic characterization of giant cells isolated from infected mice and the cellular changes associated with giant cell formation. C. neoformans infection in mice resulted in the appearance of giant cells with cell bodies up to 30 microm in diameter and capsules resistant to stripping with gamma-radiation and organic solvents. The proportion of giant cells ranged from 10 to 80% of the total lung fungal burden, depending on infection time, individual mice, and correlated with the type of immune response. When placed on agar, giant cells budded to produce small daughter cells that traversed the capsule of the mother cell at the speed of 20-50 m/h. Giant cells with dimensions that approximated those in vivo were observed in vitro after prolonged culture in minimal media, and were the oldest in the culture, suggesting that giant cell formation is an aging-dependent phenomenon. Giant cells recovered from mice displayed polyploidy, suggesting a mechanism by which gigantism results from cell cycle progression without cell fission. Giant cell formation was dependent on cAMP, but not on Ras1. Real-time imaging showed that giant cells were engaged, but not engulfed by phagocytic cells. We describe a remarkable new strategy for C. neoformans to evade the immune response by enlarging cell size, and suggest that gigantism results from replication without fission, a phenomenon that may also occur with other fungal pathogens.
Zaragoza, Oscar; García-Rodas, Rocío; Nosanchuk, Joshua D; Cuenca-Estrella, Manuel; Rodríguez-Tudela, Juan Luis; Casadevall, Arturo
The interaction between fungal pathogens with the host frequently results in morphological changes, such as hyphae formation. The encapsulated pathogenic fungus Cryptococcus neoformans is not considered a dimorphic fungus, and is predominantly found in host tissues as round yeast cells. However, there is a specific morphological change associated with cryptococcal infection that involves an increase in capsule volume. We now report another morphological change whereby gigantic cells are formed in tissue. The paper reports the phenotypic characterization of giant cells isolated from infected mice and the cellular changes associated with giant cell formation. C. neoformans infection in mice resulted in the appearance of giant cells with cell bodies up to 30 microm in diameter and capsules resistant to stripping with gamma-radiation and organic solvents. The proportion of giant cells ranged from 10 to 80% of the total lung fungal burden, depending on infection time, individual mice, and correlated with the type of immune response. When placed on agar, giant cells budded to produce small daughter cells that traversed the capsule of the mother cell at the speed of 20-50 m/h. Giant cells with dimensions that approximated those in vivo were observed in vitro after prolonged culture in minimal media, and were the oldest in the culture, suggesting that giant cell formation is an aging-dependent phenomenon. Giant cells recovered from mice displayed polyploidy, suggesting a mechanism by which gigantism results from cell cycle progression without cell fission. Giant cell formation was dependent on cAMP, but not on Ras1. Real-time imaging showed that giant cells were engaged, but not engulfed by phagocytic cells. We describe a remarkable new strategy for C. neoformans to evade the immune response by enlarging cell size, and suggest that gigantism results from replication without fission, a phenomenon that may also occur with other fungal pathogens.
Ikeda, H.; Okudaira, Y.
We have encountered a case of a pituitary mass which emerged and enlarged during pregnancy in a 29-year-old woman. On CT scanning the mass disappeared over the course of four months postpartum and was followed by pituitary hypofunction. The hypofunction was restricted to ACTH, GH, and PRL. The visual field defects, bitemporal hemianopsia, disappeared with disappearance of the pituitary mass on CT scanning, indicating that such deficits during this period were reversible. Based on the clinical course and laboratory data, this case is thought to have been lymphocytic adenohypophysitis. It was concluded that among pituitary tumors developing during pregnancy there are some such cases which do not require surgical therapy.
Ye Zhiqiu; Guo Qinglu; Feng Changzheng; Wei Beiyang; Liu Yongxi; Zhang Yan
Objective: To study the shape, size and signal intensity of pituitary gland in girls aged 3-10 year old with central precocious puberty. Methods: MRI data of pituitary glands in 40 girls aged 3-10 years old with central precocious puberty were selected. The shape, height and the appearances of pituitary glands were measured and observed on sagittal T 1 WI. Results: Quantitative data about size, shape and single intensity changes of pituitary glands in central precocious puberty were obtained in two groups, including girls aged from 3-5 and 5-10. The convex pituitary gland were 85.0% in former group. The height of pituitary gland were 6.1±0.2mm (former group) and 6.4± 0.4mm (latter one) respectively. The width of pituitary stalk was 1.93±0.50mm. The posterior pituitary gland demonstrated high signal intensity in all cases. Conclusion: Obvious changes of the size and shape of pituitary glands were found in central precocious puberty of girls aged from 3-10. The pituitary glands manifested physiologic hypertrophy with more convex in central precocious puberty girls than in normal ones. The changes on MRI could reflect the function of hypothalamus-pituitary-gonadal axis. It is of important value and significance in the diagnosis of central precocious puberty. (authors)
Full Text Available Se define onda T negativa gigante o inversión masiva de la onda T a la aparición de ondas T negativas con una amplitud = 1 mV en por lo menos dos derivaciones contiguas del ECG. Se presenta el caso de una paciente de 66 años con antecedentes de dislipidemia tipo IV e hipertensión arterial leve, a la que en buen estado de salud y totalmente asintomática en un examen de rutina se le detecta en el ECG de superficie un hemibloqueo anterior y ondas T negativas gigantes en las derivaciones I, II, aVL, aVF y de V1 a V6. La paciente fue internada en la UCI, donde se descartó que fueran de origen coronario. Durante el seguimiento, las ondas T se tornaron menos negativas hasta que al cabo de un tiempo (45 días aproximadamente se normalizaron. A pesar de los estudios cardiológicos y no cardiológicos realizados, no se pudo establecer su origen.REV ARGENT CARDIOL 2009;77:131-134.
Winters, Stephen J; Vitaz, Todd; Nowacki, Michael R; Craddock, Durrett C; Silverman, Craig
A 60-year-old man with Addison's disease, primary hypothyroidism and type 2 diabetes mellitus who was treated with stable doses of hydrocortisone and fludrocortisone developed increasing skin pigmentation and a bitemporal hemianopia. The plasma ACTH level was 14,464 pg/mL, and an invasive pituitary macroadenoma with suprasellar extension was found on magnetic resonance imaging leading to transnasal-transsphenoidal adenomectomy. The tumor demonstrated features of an eosinophilic adenoma and stained uniformly for ACTH. Residual tumor was treated with stereotactic radiotherapy. This case and the 13 cases published previously indicate that primary adrenal failure may predispose to corticotroph hyperplasia, and in some patients to the development of an invasive corticotroph adenoma. The ACTH level should be measured, and a pituitary magnetic resonance imaging is indicated when skin pigmentation increases in a patient with primary adrenal failure who is receiving customary treatment with glucocorticoids and mineralocorticoids.
Larranaga, J.; Fandino, J.; Gomez-Bueno, J.; Botana, C.; Rodriguez, D.; Gonzalez-Carrero, J.
Sphenoidal aspergillosis is an unusual cause of sella turcica enlargement. Pituitary abscess secondary to Aspergillus had been reported. In the present case, a woman with sphenoid sinus aspergillosis mimiced a pituitary tumor. This patient survived her infection with intact pituitary function following a transsphenoidal approach. No postoperative amphotericine-B and 5-fluorocytosine were necessary. CT scan revealed a mass occupying the sphenoid sinus extending to the sella turcica. Factors that should alert the clinican to the presence of a sphenoidal and pituitary abscess in a patient with sella turcica enlargement are prior episodes of sinusitis, meningitis and immunosuppression and, as in the present case, hyperglycemia. (orig.).
Wang, Ji-wen; Li, Ying; Mao, Zhi-gang; Hu, Bin; Jiang, Xiao-bing; Song, Bing-bing; Wang, Xin; Zhu, Yong-hong; Wang, Hai-jun
Excessive growth hormone (GH) is usually secreted by GH-secreting pituitary adenomas and causes gigantism in juveniles or acromegaly in adults. The clinical complications involving cardiovascular, respiratory, and metabolic systems lead to elevated morbidity in acromegaly. Control of serum GH and insulin-like growth factor (IGF) 1 hypersecretion by surgery or pharmacotherapy can decrease morbidity. Current pharmacotherapy includes somatostatin analogs (SAs) and GH receptor antagonist; the former consists of lanreotide Autogel (ATG) and octreotide long-acting release (LAR), and the latter refers to pegvisomant. As primary medical therapy, lanreotide ATG and octreotide LAR can be supplied in a long-lasting formulation to achieve biochemical control of GH and IGF-1 by subcutaneous injection every 4–6 weeks. Lanreotide ATG and octreotide LAR provide an effective medical treatment, whether as a primary or secondary therapy, for the treatment of GH-secreting pituitary adenoma; however, to maximize benefits with the least cost, several points should be emphasized before the application of SAs. A comprehensive assessment, especially of the observation of clinical predictors and preselection of SA treatment, should be completed in advance. A treatment process lasting at least 3 months should be implemented to achieve a long-term stable blood concentration. More satisfactory surgical outcomes for noninvasive macroadenomas treated with presurgical SA may be achieved, although controversy of such adjuvant therapy exists. Combination of SA and pegvisomant or cabergoline shows advantages in some specific cases. Thus, an individual treatment program should be established for each patient under a full evaluation of the risks and benefits. PMID:24421637
Pfäffle, R; Klammt, J
The somatotropic axis is the central postnatal regulator of longitudinal growth. One of its major components--growth hormone--is produced by the anterior lobe of the pituitary, which also expresses and secretes five additional hormones (prolactin, thyroid stimulating hormone, follicle stimulating hormone, luteinizing hormone, adrenocorticotropic hormone). Proper development of the pituitary assures the regulation of critical processes such as metabolic control, puberty and reproduction, stress response and lactation. Ontogeny of the adenohypophysis is orchestrated by inputs from neighbouring tissues, cellular signalling molecules and transcription factors. Perturbation of expression or function of these factors has been implicated in the aetiology of combined pituitary hormone deficiency (CPHD). Mutations within the genes encoding for the transcription factors LHX3, LHX4, PROP1, and POU1F1 (PIT1) that act at different stages of pituitary development result in unique patterns of hormonal deficiencies reflecting their differential expression during organogenesis. In the case of LHX3 and LHX4 the phenotype may include extra-pituitary manifestations due to the function of these genes/proteins outside the pituitary gland. The remarkable variability in the clinical presentation of affected patients indicates the influence of the genetic background, environmental factors and possibly stochastic events. However, in the majority of CPHD cases the aetiology of this heterogeneous disease remains unexplained, which further suggests the involvement of additional genes. Identification of these factors might also help to close the gaps in our understanding of pituitary development, maintenance and function. Copyright © 2010 Elsevier Ltd. All rights reserved.
Sakoda, K.; Mukada, K.; Yonezawa, M.; Matsumura, S.; Yoshimoto, H.; Mori, S.; Uozumi, T.
CT scan is an extremely useful, almost harmless means of diagnosing pituitary adenomas. Growth hormone (GH)-secreting adenomas tend to have higher absorption coefficent in plain CT than the nonfunctioning and prolactin (PRL)-secreting adenomas. The absorption coefficent on contrast-enhanced CT does not identify the specific type of adenoma. Ring-like enhancement was observed in five nonfunctioning and four PRL-secreting adenomas with suprasellar extension, while cystic components were observed in four nonfunctioning and four PRL-secreting adenomas. In three of ten cases of PRL-secreting microadenomas, the site corresponding to the adenoma was not enhanced, whereas the normal pituitary was. A correlation exists between the size of PRL-secreting adenoma and the serum PRL level, but not between the size of GH-secreting adenomas and the serum GH level. (orig.)
Luigi A. Lanterna
Full Text Available Occlusion of the intracranial internal carotid artery (ICA by a pituitary adenoma with resulting cerebral ischemia is a very rare but devastating occurrence. The authors present a case in which a condition of symptomatic ICA occlusion due to a giant pituitary adenoma was successfully treated using a preliminary extraintracranial bypass as a “bridge” to the tumor removal. A 52-year-old patient presented with a minor stroke followed by pressure-dependent transient ischemic attacks consistent with a condition of hypoperfusion. MR imaging and a digital subtraction angiography revealed a pituitary adenoma occluding the ICA on the right side. He underwent a superficial temporal artery to middle cerebral artery (STA-MCA bypass with the aim of revascularizing the ischemic hemisphere and reducing the risk of perioperative stroke or stroke evolution. The patient was subsequently operated on to remove the adenoma through a transsphenoidal approach. The postoperative course was uneventful and the patient has suffered no further ischemic events. When there are no emergency indications to decompress the optical pathways but the patient is at risk of impending stroke because of ICA occlusion, a two-step strategy consisting of a bypass and subsequent removal of the pituitary adenoma may be a valuable option.
Sakurai, Takashi; Sakamoto, Tatsuo; Sekino, Hiroaki; Inada, Yoichi; Ishikawa, Toru; Sato, Mitsuya
We evaluated the magnetic resonance imaging (MRI) of seventeen patients with pituitary adenomas (10 cases of prolactin secreting ; 2, growth hormone secreting and 5, non-functioning adenomas) and three patients with pituitary enlargement due to hypothyroidism. The volume of the functional adenomas or the enlarged pituitary glands correlated by MRI correlated with the level of serum pituitary hormone. We evaluated the points of differences on the T 1 weighted images of MRI between microadenomas and pituitary enlargements. The microadenomas appeared as the enlarged pituitary gland with isointensity area, but following administration of Gd-DTPA, the adenomas were recognized as a low intensity area (mass) with in an enhanced high intensity area of the normal gland with a shift of the pituitary stalk. On the other hand, the pituitary enlargements appeared as a large iso intensity area on T 1 weighted images, and were homogeneously enhanced as a high intensity area after Gd-DTPA administration, but without a shift of the pituitary stalk. Signal intensity ratios (SIR) and contrast enhancement ratios (CER) of all cases with pituitary adenomas were calculated using MRI. Differences in SIR and CER could not be demonstrated among the hormone-secreting adenomas. Thus, it is difficult to differentiate the various types of adenomas by using the differences in SIR or CER, since there are many, interfering factors, including hemorrhage, cyst, and necrosis. Postoperative permanent diabetes insipidus (DI) appeared in the cases that had no visualization of posterior high signal intensity area and a tumoral mass effect on the hypothalamus on preoperative MRI. The reason for postoperative permanent DI is thought to be the result of a disturbance of blood circulation in the pituitary gland due to a mass effect and surgical burden to the proximal pituitary stalk or the hypothalamus. (author)
Nakagawa, Yoshinobu; Fukami, Tsuneharu; Matsumoto, Keizo.
It seemed to be important to recognize the CT findings of normal pituitary gland and the stalk for the acurate morphological diagnosis of pituitary microadenoma. In a consecutive series of normal 103 cases, the CT scans obtained by high resolution CT (CE-CT, Metrizamide CT) were analized and compared with 6 cases of microadenoma. The pituitary stalk demonstrated by the reconstructed coronal CT was examined and the inclination of the stalk was measured. The mean value of the inclination of pituitary stalk was 1.4 +- 1.7 0 in normal group and 9.3 +- 2.4 0 in microadenoma group. The form of the pituitary gland demonstrated by a reconstructed mid-saggital CT were classified into the following 3 types. Type I : The gland filling the whole pituitary fossa. Type II : The gland filled with small CSF space localized in the upper-anterior part in the pituitary fossa. Type III : The enlarged CSF space of more than half of the depth of pituitary fossa and the gland localized in the retro-lower part. As for the shape of pituitary gland, type I was revealed in 26 cases (7 cases in male and 19 cases in female), Type II was revealed in 31 cases (12 cases in male and 19 cases in female), Type III was revealed in 46 cases (25 cases in male and 21 cases in female). Type I was shown in female, especially in 10 years old young female. In 19 cases of 30 years to 40 years female, Type II was shown in 9 cases. In 44 male cases, Type I and Type II were shown in all ages. In the aged, Type III was shown in more than the other types. On the other hand, Type I was noted in 5 out of 6 cases of microadenoma group. (author)
Zierhut, Dietmar; Flentje, Michael; Adolph, Juergen; Erdmann, Johannes; Raue, Friedhelm; Wannenmacher, Michael
Purpose: To evaluate therapeutic outcome and side effects of radiotherapy in pituitary adenomas as sole or combined treatment. Methods and Materials: Retrospective analysis of 138 patients (74 male, 64 female) irradiated for pituitary adenoma from 1972 to 1991 was performed. Mean age was 49.7 years (15-80 years). Regular follow-up (in the mean 6.53 ± 3.99 years) included radiodiagnostical [computed tomography (CT), magnetic resonance imaging (MRI), x-ray], endocrinological, and ophthalmological examinations. Seventy patients suffered from nonfunctional pituitary adenoma, 50 patients suffered from growth-hormone producing adenomas, 11 had prolactinomas, and 7 patients had adrenocorticotropic hormone (ACTH) producing pituitary adenomas. In 99 patients surgery was followed by radiotherapy in case of suspected remaining tumor (invasive growth of the adenoma, assessment of the surgeon, pathologic CT after surgery, persisting hormonal overproduction). Twenty-three patients were treated for recurrence of disease after surgery and 16 patients received radiation as primary treatment. Total doses from 40-60 Gy (mean: 45.5 Gy) were given with single doses of 2 Gy 4 to five times a week. Results: Tumor control was achieved in 131 patients (94.9%). In seven patients, recurrence of disease was diagnosed in the mean 2.9 years (9-98 months) after radiotherapy and salvaged by surgery. A statistically significant dose-response relationship was found in favor of doses ≥ 45 Gy. Ninety percent of the patients with hormonally active pituitary adenomas had a benefit from radiotherapy in means of complete termination (38%) or at least reduction (52%) of hormonal overproduction. Partial or complete hypopituitarism after radiotherapy developed, depending on hormonal axis, in 12 (prolactin) to 27% (follicle-stimulating hormone FSH) of patients who had not already had hypopituitarism prior to radiation. Two out of 138 patients suffered reduction of visual acuity, which was, in part
Papakonstantinou, O.; Bakantaki, A.; Papadaki, E.; Gourtsoyiannis, N.; Bitsori, M.; Mamoulakis, D.
Magnetic resonance imaging of pituitary hyperplasia has been rarely described in children with primary hypothyroidism. We report a case of pituitary hyperplasia in a child presented with significant growth arrest and laboratory evidence of hypothyroidism. Magnetic resonance imaging revealed symmetrical pituitary enlargement simulating macroadenoma. After thyroid hormone replacement therapy, the child's height increased and pituitary enlargement regressed to normal. Awareness of MRI appearance of pituitary hyperplasia in children with laboratory evidence of hypothyroidism might avoid misdiagnosis for pituitary tumor, which may also manifest as growth disorder, obviating unnecessary surgery. (orig.)
Cressler, Clayton E; Nelson, William A; Day, Troy; McCauley, Edward
Parasites often induce life-history changes in their hosts. In many cases, these infection-induced life-history changes are driven by changes in the pattern of energy allocation and utilization within the host. Because these processes will affect both host and parasite fitness, it can be challenging to determine who benefits from them. Determining the causes and consequences of infection-induced life-history changes requires the ability to experimentally manipulate life history and a framework for connecting life history to host and parasite fitness. Here, we combine a novel starvation manipulation with energy budget models to provide new insights into castration and gigantism in the Daphnia magna-Pasteuria ramosa host-parasite system. Our results show that starvation primarily affects investment in reproduction, and increasing starvation stress reduces gigantism and parasite fitness without affecting castration. These results are consistent with an energetic structure where the parasite uses growth energy as a resource. This finding gives us new understanding of the role of castration and gigantism in this system, and how life-history variation will affect infection outcome and epidemiological dynamics. The approach of combining targeted life-history manipulations with energy budget models can be adapted to understand life-history changes in other disease systems.
Cressler, Clayton E.; Nelson, William A.; Day, Troy; McCauley, Edward
Parasites often induce life-history changes in their hosts. In many cases, these infection-induced life-history changes are driven by changes in the pattern of energy allocation and utilization within the host. Because these processes will affect both host and parasite fitness, it can be challenging to determine who benefits from them. Determining the causes and consequences of infection-induced life-history changes requires the ability to experimentally manipulate life history and a framework for connecting life history to host and parasite fitness. Here, we combine a novel starvation manipulation with energy budget models to provide new insights into castration and gigantism in the Daphnia magna–Pasteuria ramosa host–parasite system. Our results show that starvation primarily affects investment in reproduction, and increasing starvation stress reduces gigantism and parasite fitness without affecting castration. These results are consistent with an energetic structure where the parasite uses growth energy as a resource. This finding gives us new understanding of the role of castration and gigantism in this system, and how life-history variation will affect infection outcome and epidemiological dynamics. The approach of combining targeted life-history manipulations with energy budget models can be adapted to understand life-history changes in other disease systems. PMID:25143034
Runemark, Anna; Sagonas, Kostas; Svensson, Erik I
Although rapid evolution of body size on islands has long been known, the ecological mechanisms behind this island phenomenon remain poorly understood. Diet is an important selective pressure for morphological divergence. Here we investigate if selection for novel diets has contributed to the multiple independent cases of island gigantism in the Skyros wall lizard (Podarcis gaigeae) and if diet, predation, or both factors best explain island gigantism. We combined data on body size, shape, bite force, and realized and available diets to address this. Several lines of evidence suggest that diet has contributed to the island gigantism. The larger islet lizards have relatively wider heads and higher bite performance in relation to mainland lizards than would be expected from size differences alone. The proportions of consumed and available hard prey are higher on islets than mainland localities, and lizard body size is significantly correlated with the proportion of hard prey. Furthermore, the main axis of divergence in head shape is significantly correlated with dietary divergence. Finally, a model with only diet and one including diet and predation regime explain body size divergence equally well. Our results suggest that diet is an important ecological factor behind insular body size divergence, but could be consistent with an additional role for predation.
Humberto G Ferrón
Full Text Available Otodontids include some of the largest macropredatory sharks that ever lived, the most extreme case being Otodus (Megaselachus megalodon. The reasons underlying their gigantism, distribution patterns and extinction have been classically linked with climatic factors and the evolution, radiation and migrations of cetaceans during the Paleogene. However, most of these previous proposals are based on the idea of otodontids as ectothermic sharks regardless of the ecological, energetic and body size constraints that this implies. Interestingly, a few recent studies have suggested the possible existence of endothermy in these sharks thus opening the door to a series of new interpretations. Accordingly, this work proposes that regional endothermy was present in otodontids and some closely related taxa (cretoxyrhinids, playing an important role in the evolution of gigantism and in allowing an active mode of live. The existence of regional endothermy in these groups is supported here by three different approaches including isotopic-based approximations, swimming speed inferences and the application of a novel methodology for assessing energetic budget and cost of swimming in extinct taxa. In addition, this finding has wider implications. It calls into question some previous paleotemperature estimates based partially on these taxa, suggests that the existing hypothesis about the evolution of regional endothermy in fishes requires modification, and provides key evidence for understanding the evolution of gigantism in active macropredators.
Ferrón, Humberto G
Otodontids include some of the largest macropredatory sharks that ever lived, the most extreme case being Otodus (Megaselachus) megalodon. The reasons underlying their gigantism, distribution patterns and extinction have been classically linked with climatic factors and the evolution, radiation and migrations of cetaceans during the Paleogene. However, most of these previous proposals are based on the idea of otodontids as ectothermic sharks regardless of the ecological, energetic and body size constraints that this implies. Interestingly, a few recent studies have suggested the possible existence of endothermy in these sharks thus opening the door to a series of new interpretations. Accordingly, this work proposes that regional endothermy was present in otodontids and some closely related taxa (cretoxyrhinids), playing an important role in the evolution of gigantism and in allowing an active mode of live. The existence of regional endothermy in these groups is supported here by three different approaches including isotopic-based approximations, swimming speed inferences and the application of a novel methodology for assessing energetic budget and cost of swimming in extinct taxa. In addition, this finding has wider implications. It calls into question some previous paleotemperature estimates based partially on these taxa, suggests that the existing hypothesis about the evolution of regional endothermy in fishes requires modification, and provides key evidence for understanding the evolution of gigantism in active macropredators.
A 23-Year-Old Female with a Mixed Germ Cell Tumor of the Pituitary Infundibulum: The Challenge of Differentiating Neoplasm from Lymphocytic Infundibuloneurohypophysitis—A Case Report and Literature Review
Sann Yu Mon
Full Text Available The pathologic spectrum of diseases that infiltrate the pituitary infundibulum includes a broad variety of clinical entities. There are significant differences in the prevalence of these etiologies depending on the age of presentation. Lymphocytic infundibuloneurohypophysitis (LINH predominates over other causes of infundibular disease in adults over age 21. Differentiating LINH from other causes of infundibular disease can be difficult because the various etiologies often have similar clinical presentations and radiologic imaging characteristics. We report the first case in an adult of a mixed germ cell tumor comprised of germinoma and embryonal cell carcinoma infiltrating the pituitary infundibulum. In our case, a 23-year-old female was initially misdiagnosed as having LINH. She presented with panhypopituitarism and diabetes insipidus, which is the most common initial presentation in both entities. The two diagnoses are difficult to distinguish based on MRI imaging, CSF findings, and histopathological examination. Our case demonstrates the need for close follow-up of patients with isolated lesions of the pituitary infundibulum and reinforces the need for biopsy of an infundibular lesion when progression of disease is demonstrated. In our case, biopsy with comprehensive immunohistochemical staining was the sole means of making a definitive diagnosis.
The efficacy and role of gamma knife (GK) in the treatment of various pituitary adenomas are described on author's experience and discussed with literature. GK subjects are 328 patients (M 126/F 202, av. age of 47.8 y) in author's hospital, and satisfactory follow-up (32-44 mo) for evaluation has been possible in 253 cases, who had tumors non-functional (129 cases), producing ACTH (23), HGH (70) and PRL (31). Stereotactic GK radiosurgery is done with navigation by Gamma Plan based on enhanced MRI images at various doses, and evaluation in the follow-up period is performed by hormonal levels and MRI which give efficacy of complete response (CR), partial response (PR), MR and standard deviation (SD)/ progressive disease (PD) on the tumor size. The overall tumor control rate is found to be 95-100%. Effectiveness (CR and PR) is found as high as 77.4% in PRL-producing tumor (marginal dose 14-32 Gy), 65% in non-functioning (15-25 Gy), 61% in ACTH (19-30 Gy) and 60% in GH (19-31 Gy), of which tendency is similar to that in literature. Even in ACTH-producing tumor, low ACTH and cortisol levels persisted with tendency of improved obese and hypertensive symptoms. GK radiosurgery has limitations in the tissue size and distance between the tumor and optic nerve/chiasm, but for the enough small tumor, it gives satisfactorily long term efficacy. (R.T.)
Full Text Available Pituitary abscess is a rare intracranial infection that can be life-threatening if not appropriately diagnosed and treated upon presentation. The most common presenting symptoms include headache, anterior pituitary hypofunction, and visual field disturbances. Brain imaging with either computed tomography or magnetic resonance imaging usually reveals intra- or suprasellar lesion(s. Diagnosis is typically confirmed intra- or postoperatively when pathological analysis is done. Clinicians should immediately start empiric antibiotics and request a neurosurgical consult when pituitary abscess is suspected. Escherichia coli (E. coli causing intracranial infections are not well understood and are uncommon in adults. We present an interesting case of an immunocompetent male with a history of hypogonadism presenting with worsening headache and acute right eye vision loss. He was found to have a polymicrobial pituitary abscess predominantly involving E. coli in addition to Actinomyces odontolyticus and Prevotella melaninogenica in the setting of an apoplectic pituitary prolactinoma. The definitive etiology of this infection was not determined but an odontogenic process was suspected. A chronic third molar eruption and impaction in close proximity to the pituitary gland likely led to contiguous spread of opportunistic oral microorganisms allowing for a polymicrobial pituitary abscess formation.
Full Text Available Introduction: Pituitary hypophysitis (PH is characterized by pituitary infiltration of lymphocytes, macrophages, and plasma cells that could lead to loss of pituitary function. Hypophysitis may be autoimmune or secondary to systemic diseases or infections. Based on the histopathological findings PH is classified into lymphocytic, granulomatous, xanthomatous, mixed forms (lymphogranulomatous, xanthogranulomatous, necrotizing and Immunoglobulin- G4 (IgG4 plasmacytic types. Objective: To report a case of lymphocytic hypophysitis (LH. Case Report: A 15-year-old girl presented with history of headache, amenorrhea, and history of polyuria for past 4 months. Initial evaluation had suppressed follicular stimulating hormone (<0.01 mIU/ml, high prolactin levels (110.85 ng/ml and diabetes insipidus (DI. Magnetic resonance imaging of sella was suggestive of pituitary macroadenoma with partial compression over optic chiasma. Patient underwent surgical decompression. Yellowish firm tissue was evacuated and xanthochromic fluid was aspirated. Histopathology was suggestive of LH. She resumed her cycles postoperatively after 4 months, prolactin levels normalized, however, she continues to have DI and is on desmopressin spray. This case has been presented here for its rare presentation in an adolescent girl because it is mostly seen in young females and postpartum period and its unique presentation as an expanding pituitary mass with optic chiasma compression. Conclusion: Definitive diagnosis of LH is based on histopathological evaluation. Therapeutic approach should be based on the grade of suspicion and clinical manifestations of LH.
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Jang, Seok Jin; Kim, Eui Jong; Choi, Woo Seok; Park, Bong Jin
Xanthomatous hypophysitis is a rare inflammatory disorder of the pituitary gland of unknown prevalence that causes pituitary dysfunction and a mass-like lesion. The authors report a case of 40-year-old man with a visual disturbance and a confirmed diagnosis of xanthomatous hypophysitis.
Sheehan, Nora K; Rylander, Helena; Christensen, Neil; Nafe, Laura A
An 8-year-old spayed female border collie dog was diagnosed with an invasive pituitary macrotumor. Five months after radiation therapy, the patient developed paraparesis and lumbosacral pain. Necropsy revealed a pituitary carcinoma with cauda equina drop metastasis. In cases of pituitary masses, meningeal dissemination should be considered if neurologic status declines.
Kashimada, Akio; Machida, Kikuo; Honda, Norinari; Mamiya, Toshio; Takahashi, Taku; Kamano, Tsuyoshi; Muramatsu, Masayuki; Inoue, Yusuke
Pituitary MR imaging was performed in 32 patients with clinically diagnosed pituitary dwarfism and 12 normal controls. The patients were divided into two groups according to the severity of pituitary dwarfism based on endocrinological data. The two patients with severe dwarfism showed transection of the pituitary stalk, ectopic posterior lobe and atrophy of the anterior lobe on MR imaging, while the 27 patients with mild dwarfism showed no abnormal MR findings of the pituitary gland. The former group corresponds to typical pituitary dwarfism and the latter to partial GH deficiency, which was recently proposed as another type of pituitary dwarfism. In conclusion, pituitary MR imaging may differentiate partial GH deficiency from typical (stalk-transected) pituitary dwarfism. (author)
Kashimada, Akio; Machida, Kikuo; Honda, Norinari; Mamiya, Toshio; Takahashi, Taku; Kamano, Tsuyoshi; Muramatsu, Masayuki; Inoue, Yusuke (Saitama Medical School, Kawagoe (Japan). Medical Center)
Pituitary MR imaging was performed in 32 patients with clinically diagnosed pituitary dwarfism and 12 normal controls. The patients were divided into two groups according to the severity of pituitary dwarfism based on endocrinological data. The two patients with severe dwarfism showed transection of the pituitary stalk, ectopic posterior lobe and atrophy of the anterior lobe on MR imaging, while the 27 patients with mild dwarfism showed no abnormal MR findings of the pituitary gland. The former group corresponds to typical pituitary dwarfism and the latter to partial GH deficiency, which was recently proposed as another type of pituitary dwarfism. In conclusion, pituitary MR imaging may differentiate partial GH deficiency from typical (stalk-transected) pituitary dwarfism. (author).
Payne, Jonathan L; Groves, John R; Jost, Adam B; Nguyen, Thienan; Moffitt, Sarah E; Hill, Tessa M; Skotheim, Jan M
Atmospheric hyperoxia, with pO(2) in excess of 30%, has long been hypothesized to account for late Paleozoic (360-250 million years ago) gigantism in numerous higher taxa. However, this hypothesis has not been evaluated statistically because comprehensive size data have not been compiled previously at sufficient temporal resolution to permit quantitative analysis. In this study, we test the hyperoxia-gigantism hypothesis by examining the fossil record of fusulinoidean foraminifers, a dramatic example of protistan gigantism with some individuals exceeding 10 cm in length and exceeding their relatives by six orders of magnitude in biovolume. We assembled and examined comprehensive regional and global, species-level datasets containing 270 and 1823 species, respectively. A statistical model of size evolution forced by atmospheric pO(2) is conclusively favored over alternative models based on random walks or a constant tendency toward size increase. Moreover, the ratios of volume to surface area in the largest fusulinoideans are consistent in magnitude and trend with a mathematical model based on oxygen transport limitation. We further validate the hyperoxia-gigantism model through an examination of modern foraminiferal species living along a measured gradient in oxygen concentration. These findings provide the first quantitative confirmation of a direct connection between Paleozoic gigantism and atmospheric hyperoxia. © 2012 The Author(s). Evolution© 2012 The Society for the Study of Evolution.
de Lara, Danielle; Filho, Leo F. S. Ditzel; Prevedello, Daniel M.; Otto, Bradley A.; Carrau, Ricardo L.
Background: Surgical treatment of pituitary pathologies has evolved along the years, adding safety and decreasing morbidity related to the procedure. Advances in the field of radiology, coupled with stereotactic technology and computer modeling, have culminated in the contemporary and widespread use of image guidance systems, as we know them today. Image guidance navigation has become a frequently used technology that provides continuous three-dimensional information for the accurate performance of neurosurgical procedures. We present a discussion about the application of image guidance in pituitary surgeries. Methods: Major indications for image guidance neuronavigation application in pituitary surgery are presented and demonstrated with illustrative cases. Limitations of this technology are also presented. Results: Patients presenting a history of previous transsphenoidal surgeries, anatomical variances of the sphenoid sinus, tumors with a close relation to the internal carotid arteries, and extrasellar tumors are the most important indications for image guidance in pituitary surgeries. The high cost of the equipment, increased time of surgery due to setup time, and registration and the need of specific training for the operating room personnel could be pointed as limitations of this technology. Conclusion: Intraoperative image guidance systems provide real-time images, increasing surgical accuracy and enabling safe, minimally invasive interventions. However, the use of intraoperative navigation is not a replacement for surgical experience and a systematic knowledge of regional anatomy. It must be recognized as a tool by which the neurosurgeon can reduce the risk associated with surgical approach and treatment of pituitary pathologies. PMID:22826819
Arao, Tadashi; Okada, Yosuke; Uemura, Fumi; Nishizawa, Shigeru; Tanaka, Yoshiya
A 54-year-old woman was admitted to our hospital for detailed examination of acromegaly because she noticed bilateral hand and finger swelling at the age of 43 and plantar thickening, facial changes and unclear articulation at the age of 49. She had prominent brow ridges, mandibular protrusion, and enlargement of the hands, feet, nasal wings, lips and tongue. Her growth hormone (GH) level was 39.8 ng/ml, insulin-like growth factor-1 (IGF-1) level was 717 ng/ml, GH level was not suppressed (22.9 ng/ml) during a 75-g oral glucose tolerance test (OGTT). Radiography showed cauliflower-like enlargement of the distal phalanx of the fingers, thickening/enlargement of the plantar soft tissues, and increased antero-posterior diameter of the sella turcica. Magnetic resonance imaging showed a mass (21×17 mm) growing towards the right suprasellar region and invading the cavernous sinus. She was diagnosed with acromegaly based on the characteristic physical findings, GH excess, high IGF-1, lack of GH suppression during the 75-g OGTT, and the presence of a pituitary tumor. She was started on octreotide long acting release (Oct-LAR) 20 mg/4w for tumor shrinkage. After three doses, her GH and IGF-1 levels decreased to 2.19 ng/ml (1.69 during the 75-g OGTT) and 205 ng/ml, respectively, meeting cure criteria for acromegaly. In this case, a decrease in GH and IGF-1 levels, tumor shrinkage, and resolution of cavernous sinus invasion allowed the patient to undergo surgery with curative intent (the first-line treatment for acromegaly) without postoperative complications. Thus, preoperative Oct-LAR administration has the potential to improve treatment outcomes of acromegaly.
Marro, B.; Zouaoui, A.; Sahel, M.; Crozat, N.; Gerber, S.; Sourour, N.; Sag, K.; Marsault, C.
Adenomas causing acromegaly represent at least a quarter of pituitary adenomas. We studied 12 patients presenting with active acromegaly due to a pituitary adenoma with a 1.5 T superconductive MRI unit. All had T1-weighted sagittal and coronal sections before and after Gd-DTPA; six had coronal T2-weighted images. Surgical correlation was obtained in seven patients. Histologically, there were eight growth hormone (GH)-secreting and three mixed [GH and prolactin (PRL) secreting[ adenomas, and one secreting GH, PRL and follicle-stimulating hormone. Macroadenomas (10) were more frequent than microadenomas (2). No correlation was found between serum GH and tumour size. There were nine adenomas in the lateral part of the pituitary gland; seven showed lateral or infrasellar invasion. Homogeneous, isointense signal on T1-and T2-weighted images was observed in six cases. Heterogeneous adenomas had cystic or necrotic components. (orig.). With 5 figs., 3 tabs
Marro, B. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Zouaoui, A. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sahel, M. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Crozat, N. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Gerber, S. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sourour, N. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Sag, K. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France); Marsault, C. [Service de Neuroradiologie, Batiment Babinsky, Hopital Salpetriere, 47, Bd de l`Hopital, F-75013 Paris (France)
Adenomas causing acromegaly represent at least a quarter of pituitary adenomas. We studied 12 patients presenting with active acromegaly due to a pituitary adenoma with a 1.5 T superconductive MRI unit. All had T1-weighted sagittal and coronal sections before and after Gd-DTPA; six had coronal T2-weighted images. Surgical correlation was obtained in seven patients. Histologically, there were eight growth hormone (GH)-secreting and three mixed [GH and prolactin (PRL) secreting] adenomas, and one secreting GH, PRL and follicle-stimulating hormone. Macroadenomas (10) were more frequent than microadenomas (2). No correlation was found between serum GH and tumour size. There were nine adenomas in the lateral part of the pituitary gland; seven showed lateral or infrasellar invasion. Homogeneous, isointense signal on T1- and T2-weighted images was observed in six cases. Heterogeneous adenomas had cystic or necrotic components. (orig.). With 5 figs., 3 tabs.
Taoka, Toshiaki; Iwasaki, Satoru; Okamoto, Shingo; Sakamoto, Masahiko; Nakagawa, Hiroyuki; Otake, Shoichiro; Fujioka, Masayuki; Hirohashi, Shinji; Kichikawa, Kimihiko
The purpose of this study was to evaluate the relationship between pituitary stalk compression by the dorsum sellae and clinical or laboratory findings in short stature children. We retrospectively reviewed magnetic resonance images of the pituitary gland and pituitary stalk for 34 short stature children with growth hormone (GH) deficiency and 24 age-matched control cases. We evaluated the degree of pituitary stalk compression caused by the dorsum sellae. Body height, GH level, pituitary height and onset age of the short stature were statistically compared between cases of pituitary stalk compression with associated stalk deformity and cases without compression. Compression of the pituitary stalk with associated stalk deformity was seen in nine cases within the short stature group. There were no cases observed in the control group. There were no significant differences found for body height, GH level and pituitary height between the cases of pituitary stalk compression with associated stalk deformity and cases without compression. However, a significant difference was seen in the onset age between cases with and without stalk compression. Pituitary stalk compression with stalk deformity caused by the dorsum sellae was significantly correlated with late childhood onset of short stature.
Chen Shuang; Qian Ruiling; Tang Zhiwei; Liu Ke; Huang Yong; Li Xi
Objective: To investigate the MR features of pituitary abscess. Methods: The MR features of 14 eases of pituitary abscess proved by surgical pathology and clinical treatments were analyzed retrospectively. Results: Pre-contrast MR showed hypointense heterogeneous intensity on T 1 WI in 12 cases and iso-hyperintense on T 1 WI in 2 cases, hyperintense on T 2 WI in all cases. Post-gadolinium MR showed the ring-like enhancement around the uneven edge of abscess and the surrounding enhanced meninges connecting to the focus. The normal pituitary could not be identified in all 14 cases. The MR specific findings include the fluid-fluid level, nodule on the edge and the enhanced patchy shadow. Conclusions: The pituitary abscess has specific findings on MR examination, which can be used to combine with clinical symptoms to achieve the diagnosis before operation, so that the cases could be treated with antibiotic without operation. (authors)
Cho, Charles H; Barkhoudarian, Garni; Hsu, Liangge; Bi, Wenya Linda; Zamani, Amir A; Laws, Edward R
Identification of the normal pituitary gland is an important component of presurgical planning, defining many aspects of the surgical approach and facilitating normal gland preservation. Magnetic resonance imaging is a proven imaging modality for optimal soft-tissue contrast discrimination in the brain. This study is designed to validate the accuracy of localization of the normal pituitary gland with MRI in a cohort of surgical patients with pituitary mass lesions, and to evaluate for correlation between presurgical pituitary hormone values and pituitary gland characteristics on neuroimaging. Fifty-eight consecutive patients with pituitary mass lesions were included in the study. Anterior pituitary hormone levels were measured preoperatively in all patients. Video recordings from the endoscopic or microscopic surgical procedures were available for evaluation in 47 cases. Intraoperative identification of the normal gland was possible in 43 of 58 cases. Retrospective MR images were reviewed in a blinded fashion for the 43 cases, emphasizing the position of the normal gland and the extent of compression and displacement by the lesion. There was excellent agreement between imaging and surgery in 84% of the cases for normal gland localization, and in 70% for compression or noncompression of the normal gland. There was no consistent correlation between preoperative pituitary dysfunction and pituitary gland localization on imaging, gland identification during surgery, or pituitary gland compression. Magnetic resonance imaging proved to be accurate in identifying the normal gland in patients with pituitary mass lesions, and was useful for preoperative surgical planning.
Berker, Dilek; Isik, Serhat; Aydin, Yusuf; Tutuncu, Yasemin; Akdemir, Gokhan; Ozcan, Hatice Nursun; Guler, Serdar
Thyroid stimulating hormone (TSH) secreting pituitary adenomas are rare tumors manifested as hyperthyroidism with goiter in the presence of elevated TSH. We present a case with pituitary adenoma secreting both TSH and growth hormone (GH) with the prominent clinical findings of hyperthyroidism but without clinical findings of acromegaly. Pituitary magnetic resonance imaging revealed a macroadenoma. Transsphenoidal surgery was performed twice. The immunohistochemical staining showed that tumor cells were strongly reactive to GH and relatively mildly reactive to TSH. Control pituitary imaging revealed a residual macroadenoma, and long acting octreotide treatment was administered. After two years of the treatment, tumor size remained the same while thyroid function tests and insulin-like growth factor 1 (IGF-I) values returned to normal ranges. In conclusion, we always recommend hormonal examinations for all patients who have pituitary adenoma without signs and symptoms of acromegaly.
T.A. Gomes-Rodrigues; A. López-Ojeda; J. Muñoz i Vidal; M.T. Soler-Monsó; J.M. Serra-Payro; A. Guma-Martínez; F. Chavarría-Marín; M. Dewever
El hamartoma gigante de la mama es una entidad clínico-patológica inusual que tiende a confundirse con otros tumores. A pesar del avance en las técnicas diagnósticas, su diagnóstico clínico es difícil y normalmente solo se confirma cuando se analiza toda la pieza de resección. El diagnóstico diferencial debe incluir entidades como el fibroadenoma, lipoma, tumor phyllodes y diversos tipos de carcinomas. Presentamos 2 casos de hamartoma gigante de mama en mujeres de edad media, de los cuales un...
Skyllouriotis, M L; Marx, M; Bittner, R E; Skyllouriotis, P; Gross, M; Wimmer, M
We report a 20-year-old man with gigantism syndrome, hypertrophic cardiomyopathy, muscle weakness, exercise intolerance, and severe psychomotor retardation since childhood. Histochemical and biochemical analysis of skeletal muscle biopsy revealed myoadenylate deaminase deficiency; molecular genetic analysis confirmed the diagnosis of primary (inherited) myoadenylate deaminase deficiency. Plasma, urine, and muscle carnitine concentrations were reduced. L-Carnitine treatment led to gradual improvement in exercise tolerance and cognitive performance; plasma and tissue carnitine levels returned to normal, and echocardiographic evidence of left ventricular hypertrophy disappeared. The combination of inherited myoadenylate deaminase deficiency, gigantism syndrome and carnitine deficiency has not previously been described.
Salvatori, Roberto; Radian, Serban; Diekmann, Yoan; Iacovazzo, Donato; David, Alessia; Gabrovska, Plamena; Grassi, Giorgia; Bussell, Anna-Marie; Stals, Karen; Weber, Astrid; Quinton, Richard; Crowne, Elizabeth C; Corazzini, Valentina; Metherell, Lou; Kearney, Tara; Du Plessis, Daniel; Sinha, Ajay Kumar; Baborie, Atik; Lecoq, Anne-Lise; Chanson, Philippe; Ansorge, Olaf; Ellard, Sian; Trainer, Peter J; Balding, David; Thomas, Mark G; Korbonits, Márta
Mutations in the aryl hydrocarbon receptor-interacting protein ( AIP ) gene are associated with pituitary adenoma, acromegaly and gigantism. Identical alleles in unrelated pedigrees could be inherited from a common ancestor or result from recurrent mutation events. Observational, inferential and experimental study, including: AIP mutation testing; reconstruction of 14 AIP -region (8.3 Mbp) haplotypes; coalescent-based approximate Bayesian estimation of the time to most recent common ancestor (tMRCA) of the derived allele; forward population simulations to estimate current number of allele carriers; proposal of mutation mechanism; protein structure predictions; co-immunoprecipitation and cycloheximide chase experiments. Nine European-origin, unrelated c.805_825dup-positive pedigrees (four familial, five sporadic from the UK, USA and France) included 16 affected (nine gigantism/four acromegaly/two non-functioning pituitary adenoma patients and one prospectively diagnosed acromegaly patient) and nine unaffected carriers. All pedigrees shared a 2.79 Mbp haploblock around AIP with additional haploblocks privately shared between subsets of the pedigrees, indicating the existence of an evolutionarily recent common ancestor, the 'English founder', with an estimated median tMRCA of 47 generations (corresponding to 1175 years) with a confidence interval (9-113 generations, equivalent to 225-2825 years). The mutation occurred in a small tandem repeat region predisposed to slipped strand mispairing. The resulting seven amino-acid duplication disrupts interaction with HSP90 and leads to a marked reduction in protein stability. The c.805_825dup allele, originating from a common ancestor, associates with a severe clinical phenotype and a high frequency of gigantism. The mutation is likely to be the result of slipped strand mispairing and affects protein-protein interactions and AIP protein stability. © 2017 The authors.
Brunet-LLobet, Lluís; Miranda-Rius, Jaume; Lahor-Soler, Eduard; Mrina, Ombeni; Nadal, Alfons
Pyogenic granuloma is defined as a benign neoplasm of vascular phenotype. This case describes the clinical and histopathological features of a gigantic mucogingival pyogenic granuloma, in a 14-year-old healthy black boy. This exophytic gray-purple mass, related to a toothpick injury, had more than twelve-month evolution on the anterior mandible involving lingual area besides to the floor of the mouth pressing the right salivary duct. Conservative excision was performed, followed by uncomplicated healing with no recurrence in two years. The histopathological examination reported a pyogenic granuloma (lobular capillary haemangioma). The authors provide a discussion of the presurgical differential diagnosis of the lesion. This case report presents an extremely uncommon location of a gigantic pyogenic granuloma, involving mucogingival complex and affecting the salivary outflow. This clinical manuscript may shed light on the controversies about possible mechanisms inducing oral pyogenic granuloma.
Full Text Available Abstract Background Pituitary tumours may present with a variety of neurological and endocrinological signs and symptoms. It is very rare however for them to present with sudden onset painful diplopia. The current literature and possible mechanisms for this are discussed. Case presentation We describe a case of a pituitary mass which presented with sudden onset painful diplopia with an associated restricted pattern on Lees Chart testing. This led to an initial working diagnosis of orbital myositis. Conclusion Awareness of different modes of presentation of pituitary lesions is important so that appropriate imaging may be requested and delay in diagnosis prevented.
Huan ZHOU; Ya-ling NIE; Wei FAN; Cong-ying WANG; An-sheng LI; Hong WANG; Meng-meng WU
Objective To explore the value of pituitary MRI in diagnosis of etiology and prognosis in children with short stature. Methods The MRI data of 130 children with short stature admitted from Jan. to Dec. 2012 were retrospectively analyzed. Of the 130 children, 79 were males and 51 were females, aged 3 to 18 years with mean of 9.8 years. Results Of the 130 children, 82 cases (63.1%) were shown to have normal pituitary morphology and signal manifestation, and in 48 cases (36.9%) pituitary morphol...
Lin Guangwu; Zhang Tao; Yang Ning; Cai Feng; Shi Yifan; Deng Jieying; Zhang Luodong; Jiang Yayun
Objective: To study the changes of shape, size and signal intensity of normal pituitary glands in adolescents and to correlate the size and shape of normal pituitary glands with the age, height and weight in stage of puberty. Methods: One hundred and fifty-five cases of MRI data of pituitary glands in normal adolescents range from 6.0 year to 18.9 year were used. Using high-field 1.5T MR scanner, the appearances of pituitary glands in 152 normal adolescents were analyzed on T 1 WI in standard median sagittal and coronal plane. Results: Three groups quantitative data of size, shape and single intensity changes of normal pituitary glands were obtained, which were divided into 6- m =0.74, t=3.624, P=0.004; r f =0.94, t=9.562, P=0.000), however, it was not markedly correlated with the height and weight (P>0.05). Conclusion: Obvious changes of the size and shape of pituitary glands were found in health adolescents. The pituitary glands manifest physiologic hypertrophy with more convex of upper border when age increased in stage of puberty. The spherical appearance of the pituitary glands is a normal developmental feature and should not warrant clinical investigation for the presence of an underlying micro-adenoma in teenage females. (authors)
Luis V. Syro
Full Text Available We briefly review the characteristics of pituitary tumors associated with multiple endocrine neoplasia type 1. Multiple endocrine neoplasia type 1 is an autosomal-dominant disorder most commonly characterized by tumors of the pituitary, parathyroid, endocrine-gastrointestinal tract, and pancreas. A MEDLINE search for all available publications regarding multiple endocrine neoplasia type 1 and pituitary adenomas was undertaken. The prevalence of pituitary tumors in multiple endocrine neoplasia type 1 may vary from 10% to 60% depending on the studied series, and such tumors may occur as the first clinical manifestation of multiple endocrine neoplasia type 1 in 25% of sporadic and 10% of familial cases. Patients were younger and the time between initial and subsequent multiple endocrine neoplasia type 1 endocrine lesions was significantly longer when pituitary disease was the initial manifestation of multiple endocrine neoplasia type 1. Tumors were larger and more invasive and clinical manifestations related to the size of the pituitary adenoma were significantly more frequent in patients with multiple endocrine neoplasia type 1 than in subjects with non-multiple endocrine neoplasia type 1. Normalization of pituitary hypersecretion was much less frequent in patients with multiple endocrine neoplasia type 1 than in subjects with non-multiple endocrine neoplasia type 1. Pituitary tumors in patients with multiple endocrine neoplasia type 1 syndrome tend to be larger, invasive and more symptomatic, and they tend to occur in younger patients when they are the initial presentation of multiple endocrine neoplasia type 1.
Normal MRI appearances of the pituitary glands and their surrounding structures were evaluated in 332 patients without sellar and parasellar diseases. The height of the pituitary gland was maximum at 10-19 years of age reflecting hormonal activity. The width of the pituitary gland decreased, while that of the cavernous sinus increased with aging. This is probably due to atherosclerotic change of the internal carotid artery. Females younger than 30 years of age tended to show a convex upper surface of the pituitary gland and the displacement of the pituitary stalk was common after 50 years of age. Almost all of the anterior lobe of the pituitary gland showed isointensity relative to the pons or cerebral cortex and the majority (85.1%) of the posterior lobe showed hyperintensity. However, the anterior lobe in 2 newborns showed hyperintensity similar to the normal posterior lobe in adults. The posterior lobe was located off the midline in 19.1% of the subjects. One case of pars intermedia cyst was discovered among 14 subjects who were administered Gd-DTPA. The dural membrane between the pituitary gland and cavernous sinus was recognizable only in 8.6% on the right side and 7.5% on the left side. Primary empty sella was identified in 4.5%. Knowledge of the above normal ranges and variations of the pituitary gland and its surrounding structures is important in diagnosing sellar and parasellar lesions. (author) 52 refs
Mortini, Pietro; Barzaghi, Lina Raffaella; Albano, Luigi; Panni, Pietro; Losa, Marco
We report the efficacy and safety of transsphenoidal microsurgery in a large and homogeneous cohort of patients with pituitary adenomas (PAs) treated at a single Institute by a single neurosurgeon. A total of 2145 consecutive patients undergoing first surgery for a PA were included: 795 (37.1%) had a nonfunctioning pituitary adenoma (NFPA), 595 (27.7%) acromegaly, 496 (23.1%) Cushing's disease, 208 (9.7%) a PRL-secreting adenoma, and 51 patients (2.4%) a TSH-secreting adenoma. Remission was achieved when strict hormonal and radiological criteria were met. Early surgical remission was achieved in 66% of acromegalic patients, 79.6% of patients with Cushing's disease, 64.4% of prolactinomas, 74.5% of patients with a TSH-secreting adenoma, and 66.9% of NFPAs. The mean (±SE) follow-up was 60.1 ± 1.3 months. The recurrence-free survival at 10 years was 78.2% in acromegalic patients, 68.1% in prolactinomas, 74.3% in Cushing's disease, 70.3% in TSH-secreting adenomas, and 75.3% in NFPAs. Preoperative hypoadrenalism recovered in 35.3%, hypogonadism in 43.3% and hypothyroidism in 37.4% of patients with impaired function before surgery. The mortality rate was 0.2% and major morbidity 2.1%. New onset hypoadrenalism occurred after surgery in 2.5% of patients at risk, hypogonadism in 4.1%, and hypothyroidism in 1.8%. Permanent diabetes insipidus (DI) occurred in 0.9% of patients. In experienced hands, transsphenoidal microsurgery for PAs achieves remission in most patients with a low complication rate. Pituitary function is preserved in most cases and can recover in more than one-third of patients with preoperative hypopituitarism.
Cansu, Güven Barış; Taşkıran, Bengür; Trivellin, Giampaolo; Faucz, Fabio R; Stratakis, Constantine A
Familial isolated pituitary adenomas (FIPA) constitute 2-3% of pituitary tumours. AIP is the most commonly mutated gene in FIPA. We herein report a novel germline mutation of the AIP gene in a family with FIPA. We present two patients, a father and his 12-year-old daughter, diagnosed clinically and using laboratory measures with acromegaly-gigantism. Both underwent transsphenoidal hypophyseal surgery for macroadenomas. We initially detected a novel heterozygous germline AIP mutation, c.836G>A (p.W279*), in the father's DNA. We then found the same mutation in his affected daughter. Pituitary adenomas associated with AIP mutations mostly present as FIPA (68%) at an early age (78% occur at treatment success, and genetic counseling.
Thawani, Jayesh P; Bailey, Robert L; Burns, Carrie M; Lee, John Y K
Examining the pathologic progression of a pituitary adenoma from the point of a prepubescent child to an adult with gigantism affords us an opportunity to consider why patients may develop secretory or functioning tumors and raises questions about whether therapeutic interventions and surveillance strategies could be made to avoid irreversible phenotypic changes. A patient underwent a sublabial transsphenoidal resection for a clinically non-functioning macroadenoma in 1999. He underwent radiation treatment and was transiently given growth hormone (GH) supplementation as an adolescent. His growth rapidly traversed several percentiles and he was found to have elevated GH levels. The patient became symptomatic and was taken for a second neurosurgical procedure. Pathology and immunohistochemical staining demonstrated a significantly higher proportion of somatotroph cells and dense granularity; he was diagnosed with a functional somatotroph adenoma. While it is likely that the described observations reflect the manifestations of a functional somatotroph adenoma in development, it is possible that pubertal growth, GH supplementation, its removal, or radiation therapy contributed to the described endocrine and pathologic changes.
Gabriel Torrealba-Acosta, MD
Full Text Available Pituitary macroadenomas may present with a pituitary apoplexy, commonly with associated hemorrhage, but rarely large enough to show an intraventricular bleeding. This is a case report of a 66-year-old woman with a 3-month history of headache, hyporexia, asthenia and altered consciousness. First evaluations showed a large intraventricular hemorrhage on CT scan that was initially misdiagnosed for an aneurysmal rupture etiology but later confirmed to be a pituitary apoplexy from a large pituitary adenoma. Intraventricular hemorrhage due to a pituitary apoplexy could be mistaken for an aneurysmal origin thus warranting for a careful evaluation of the sellar region.
Clarissa Groberio Borba
Full Text Available Pituitary carcinomas are very rare tumors that in most cases produce prolactin and adrenocorticotropic hormone (ACTH. It is a challenge to diagnosis of a pituitary carcinoma before disclosed symptomatic metastasis. We report the case of a female patient with Cushing’s disease who underwent three transsphenoidal surgeries, with pathological findings of common ACTH pituitary adenoma including Ki-67 expression <3%. She achieved hypocortisolism after the 3rd surgery although ACTH levels remained slightly elevated. The patient returned some time later with fast worsening of hypercortisolism. Magnetic resonance imaging showed clivus invasion, which led to a fourth surgery and radiation. This time, immunohistochemistry revealed strong Ki-67 (10% to 15% and p53 expression. Liver and lumbar spine metastases were found on workup. The patient died after few months due to lung infection. Pituitary carcinomas are rare, and the transformation of an ACTH-secreting pituitary adenoma into a carcinoma is exceptional. The difficulty of defining markers for the diagnosis of carcinoma, before metastasis diagnosis, in order to change the management of the disease, is a challenge.
M.V. Guillermo Pérez Jimeno
ResumenLos pasados días 2 y 3 de noviembre se llevó a cabo el 1er. Taller internacional sobre oso hormiguero gigante (Myrmecophaga tridactyla), en instalaciones del Zoológico de Florencio Varela. Participaron activamente representantes de Brasil, Colombia, Holanda, Alemania y Argentina.
Reddy, B S; Sheriff, M O; Garg, B R; Ratnakar, C
An unusual association of localized gigantism with hypertrophy of the long bones and soft tissues in the left lower limb in an 18-year-old male with tuberous sclerosis (TS) is reported. The significance of this association is discussed from the point of view of its common neural crest origin during embryogenesis.
Singh, Rajesh; Maurya, Ajeet; Chanrion, Olivier; Neubert, Torsten; Cummer, Steven; Mlynarczyk, Janusz; Bór, József; Siingh, Devendraa; Cohen, Morris; Kumar, Sushil
Gigantic Jets are electric discharges from thunderstorm cloud tops to the bottom of the ionosphere at ~80 km altitude. After their first discovery in 2001, relatively few observations have been reported. Most of these are from satellites at large distances and a few tens from the ground at higher spatial resolution. Here we report the first Gigantic Jets observed in India from two thunderstorm systems that developed over the land surface from monsoon activity, each storm producing two Gigantic Jets. The jets were recorded by a video camera system at standard video rate (20 ms exposure) at a few hundred km distance. ELF measurements suggest that the jets are of the usual negative polarity and that they develop in less than 40 ms, which is faster than most jets reported in the past. The jets originate from the leading edge of a slowly drifting convective cloud complex close to the highest regions of the clouds and carry ~25 Coulomb of charge to the ionosphere. One jet has a markedly horizontal displacement that we suggest is caused by a combination of close-range cloud electric fields at inception, and longer-range cloud fields at larger distances during full development. The Gigantic Jets are amongst the few that have been observed over land.
J.P. Crestanello Nese
Full Text Available Desde su descripción original, las lesiones de células gigantes (LCG han sido entidades controvertidas, desde el punto de vista de su origen, de su comportamiento clínico, de sus características radiográficas e histológicas, así como de su tratamiento. Para su tratamiento se han considerado alternativas quirúrgicas y no quirúrgicas. En este trabajo, se presentan tres nuevos casos de LCG, en los cuales se realizó infiltración intralesional con corticoides como una maniobra previa y complementaria a la quirúrgica. Luego de la infiltración, se observó una disminución del tamaño de las lesiones y un cambio en sus características macroscópicas, se trato por enucleación un caso y por remodelación quirúrgica los dos restantes.Giant cell lesions (GCL have been controversial entities since its original description. Its origin, clinical behavior, radiographic and histological features and also its treatment are polemical. The therapeutic possibilities are surgical or non surgical. In this paper, the intralesional infiltration with steroids is presented like a previous and complementary therapy to surgery alone. Three new cases of GCL are presented. All of them were first treated with intralesional infiltration with steroids. After that,a partial remission and a change of the macroscopic characteristics were observed and one of the lesion was then enucleated while for the others two surgical remodelation were necessary to do.
Full Text Available Hyponatremia is commonly seen electrolyte disturbance clinically. It is potentially life-threatening and requires prompt diagnosis and treatment. Non-functioning pituitary macroadenoma generally presents with a headache, visual disturbances, and the diagnosis is often delayed because of nonspecific nature of symptoms. Hyponatremia being initial manifestation before other common symptoms of pituitary macroadenoma is rare. We present a case of 55-year-old male with recurrent episodes of hyponatremia who was found to non-functioning pituitary macroadenoma along with panhypopituitarism. He was managed with hormone replacements. Our case highlights the importance of correct diagnosis of hyponatremia, measurement of the thyroid, adrenal and pituitary function in cases of hyponatremia.
Malenković, Vesna; Gvozdenović, Ljiljana; Milaković, Branko; Sabljak, Vera; Ladjević, Nebojsa; Zivaljević, Vladan
This paper presents the most common disorders of pituitary function: acromegaly, hypopituitarism, diabetes insipidus and syndrome similar to diabetes insipidus, in terms of their importance in preoperative preparation of patients. Pituitary function manages almost the entire endocrine system using the negative feedback mechanism that is impaired by these diseases. The cause of acromegaly is a pituitary adenoma, which produces growth hormone in adults. Primary therapy of acromegaly is surgical, with or without associated radiotherapy. If a patient with acromegaly as comorbidity prepares for non-elective neurosurgical operation, then it requires consultation with brain surgeons for possible delays of that operation and primary surgical treatment of pituitary gland. If operative treatment of pituitary gland is carried out, the preoperative preparation (for other surgical interventions) should consider the need for perioperative glucocorticoid supplementation. Panhypopituitarism consequences are different in children and adults and the first step in diagnosis is to assess the function of target organs. Change of electrolytes and water occurs in the case of pituitary lesions in the form of central or nephrogenic diabetes insipidus as a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Preoperative preparation of patients with pituitary dysfunction should be multidisciplinary, whether it is a neurosurgical or some other surgical intervention. The aim is to evaluate the result of insufficient production of pituitary hormones (hypopituitarism), excessive production of adenohypophysis hormones (acromegaly, Cushing's disease and hyperprolactinemia) and the influence of pituitary tumours in surrounding structures (compression syndrome) and to determine the level of perioperative risk. Pharmacological suppressive therapy of the hyperfunctional pituitary disorders can have significant interactions with drugs used in the perioperative period.
Bladowska, Joanna; Sokolska, Violetta; Sozański, Tomasz; Bednarek-Tupikowska, Grażyna; Sąsiadek, Marek
Post-surgical evaluation of the pituitary gland in MRI is difficult because of a change of anatomical conditions. It depends also on numerous other factors, including: size and expansion of a tumour before surgery, type of surgical access, quality and volume of filling material used and time of its resorption.The aim of the study was to compare MR image of the pituitary gland after surgery with clinical findings and to establish a correlation between MRI presentation of spared pituitary and its hormonal function. 124 patients after resection of pituitary adenomas - 409 MRI results in total - were studied. With a 1.5-T unit, T1-weighted sagittal and coronal, enhanced and unenhanced images were obtained. The pituitary gland seemed to be normal in MRI in 11 patients, 8 of them had completely regular pituitary function but in 3 of them we noticed a partial hypopituitarism. In 99 patients only a part of the pituitary gland was recognised, 53 of them had hypopituitarism but 46 of them were endocrinologically healthy. 14 patients seemed to have no persistent pituitary gland in MRI, in comparison to hormonal studies: there was panhypopituitarism in 6 and hypopituitarism in 8 cases. MRI presentation of post - surgical pituitary gland doesn't necessarily correlate with its hormonal function - there was a significant statistical difference. Some patients with partial pituitary seems normal hormonal function. In some cases the pituitary seem normal in MRI but these patients have hormonal disorders and need substitution therapy.
Bladowska, J.; Sokolska, V.; Sasiadek, M.; Sozanski, T.; Bednarek-Tupikowska, G.
Background: Post-surgical evaluation of the pituitary gland in MRI is difficult because of a change of anatomical conditions. It depends also on numerous other factors, including: size and expansion of a tumour before surgery, type of surgical access, quality and volume of filling material used and time of its resorption.The aim of the study was to compare MR image of the pituitary gland after surgery with clinical findings and to establish a correlation between MRI presentation of spared pituitary and its hormonal function. Material/Methods: 124 patients after resection of pituitary adenomas - 409 MRI results in total - were studied. With a 1.5-T unit, T1-weighted sagittal and coronal, enhanced and unenhanced images were obtained. Results: The pituitary gland seemed to be normal in MRI in 11 patients, 8 of them had completely regular pituitary function but in 3 of them we noticed a partial hypopituitarism. In 99 patients only a part of the pituitary gland was recognised, 53 of them had hypopituitarism but 46 of them were endocrinologically healthy. 14 patients seemed to have no persistent pituitary gland in MRI, in comparison to hormonal studies: there was panhypopituitarism in 6 and hypopituitarism in 8 cases. Conclusions: MRI presentation of post - surgical pituitary gland doesn't necessarily correlate with its hormonal function - there was a significant statistical difference. Some patients with partial pituitary seems normal hormonal function. In some cases the pituitary seem normal in MRI but these patients have hormonal disorders and need substitution therapy. (authors)
Full Text Available Objectives: Pituitary abscess is a rare occurrence among pituitary conditions, but one which carries life-threatening potential. An immunocompromised status is a risk factor for the development of a pituitary abscess; however, literature describes only one case among HIV-infected patients. Methods and results: We present here a case of pituitary abscess in an HIV-1-positive patient, who demonstrated a shock status, disturbance of consciousness and generalized skin rash with laboratory findings of hypovolemia, acute inflammatory reaction and blood electrolyte abnormality. We first diagnosed the dermal manifestation as atypical generalized zoster, however, the other clinical findings could not be explained by VZV infection only. Combination with anamnesis, head magnetic resonance imaging scan and endocrine function test helped us to diagnose pituitary abscess. Although the etiology of the pituitary abscess could not be detected, the patient was successfully treated with antibiotics but followed by panhypopituitarism as sequela. Conclusion: A pituitary abscess should be considered in HIV-infected patients with endocrinological abnormalities, visual field defects, and central nervous system infection signs or symptoms, regardless of CD4 T-cell counts.
Full Text Available We describe a male patient who presented with sudden onset severe headache and right sided ptosis that was diagnosed to be secondary to pituitary apoplexy on the background of diabetes mellitus. This was complicated by left ventricular failure and acute coronary syndrome. The case highlights the importance of considering hypocortisolism/hypopituitarism as an important and rare precipitant of an acute coronary event as occurred in the case.
Olival Cirilo Lucena da Fonseca-Neto
Full Text Available INTRODUÇÃO: A doença cística das vias biliares é anomalia congênita que pode acometer as vias biliares intra e/ou extra-hepáticas. A extra-hepática inclui os cistos de colédoco e a intra-hepática é conhecida por Doença de Caroli. Os cistos de colédoco de tamanho gigante são muito raros. OBJETIVO: Relatar o caso de um cisto de colédoco de tamanho gigante em uma paciente feminina. RELATO DE CASO: Mulher de 19 anos foi admitida com história de icterícia e acolia fecal há sete dias. Referia dor epigástrica associada com ingestão de dieta rica em gordura. Nos antecedentes pessoais relatou dois episódios de icterícia, aos 8 e 14 anos, que progrediram espontaneamente. No exame físico apresentava icterícia (+3/+4 e uma massa palpável indolor em mesogástrio foram os únicos achados. A ultrassonografia demonstrou grande formação cística de paredes finas adjacente ao hepatocolédoco, pâncreas e rim direito que media 18,5 x 10,2 cm. A colangioressonância confirmou o grande cisto de colédoco e hepatojejunoanastomose em "Y" de Roux após excisão do cisto e colecistectomia foi realizada. A formação cística media 20 x 15,5 x 12,5 cm e com um volume médio de 1000 mL. A paciente encontra-se em acompanhamento ambulatorial sem alterações hepatobiliares após o sétimo mês da operação. CONCLUSÃO: O cisto de colédoco deve fazer parte do diagnóstico diferencial em pacientes adultos jovens com icterícia e massa palpável; no entanto, a diferenciação entre ele e neoplasia maligna deve ser pesquisada.BACKGROUND: Choledochal cyst represents a rare congenital anomaly, eventually associated with intra and extrahepatic biliary tract disorders. Extrahepatic diseases include choledochal cysts and congenital dilation of the lower intrahepatic bile duct is known as Caroli's disease. Giant choledochal cyst constitutes a very rare abnormality. AIM:To report a giant choledochal cyst in a female patient. CASE REPORT: A 19-year
Aneurisma gigante do segmento intracavernoso da carótida interna associado a doença renal policística autossômica dominante: relato de caso Giant aneurysm of the intracavernous internal carotid artery associated with autosomal dominant polycystic kidney disease: case report
Keven F. Ponte
Full Text Available Apresenta-se o caso de mulher de 60 anos com doença renal policística autossômica dominante (DRPAD que desenvolveu quadro de cefaléia e oftalmoplegia completa à direita. A TC levantou a hipótese de um aneurisma gigante do segmento intracavernoso da carótida interna direita, o que foi confirmado pela arteriografia. Realizou-se, então, tratamento endovascular por oclusão do vaso parental com molas destacáveis no segmento supraclinóideo. A paciente evoluiu com a interrupção da cefaléia e com redução parcial da ptose e da oftalmoplegia. Neste artigo, enfatiza-se a relação entre DRPAD e aneurismas intracranianos. Comenta-se a história natural dos aneurismas originados no segmento intracavernoso da artéria carótida interna e comparam-se as opções terapêuticas no manejo destas lesões.We report the case of a 60 years-old woman with autosomal dominant polycystic kidney disease (ADPKD that presented with headache and right complete ophthalmoplegia. The CT scan raised the possibility of a giant aneurysm of the right intracavernous internal carotid artery, confirmed by angiography. The patient underwent endovascular occlusion of parent vessel with detachable coils, then she presented interruption of headache and partial recovery of ptosis and ophthalmoplegia. We emphasize the relationship between ADPKD and intracranial aneurysms. We also discuss the natural history and compare the therapeutic options for the management of giant aneurysms of the cavernous portion of the carotid artery.
Kang, Byung Chul; Kim, Dong Ik; Chung, Tae Sup; Cho, Yong Kook; Lee, Eun Gig; Jung, Joon Keun
To demonstrate and evaluate the value of MRI findings of the TSH(Thyroid-Stimulating Hormone, TSH, Thyrotropin)-secreting pituitary adenoma. The authors reviewed retrospectively the MR images of 4 patients with TSH-secreting pituitary adenoma. Evaluation of the anatomical location, signal characteristics, enhancement patterns, size, shape and circunferential changes were made. No characteristic common MR findings in size, shape, signal intensity, and circumferential changes of TSH-secreting pituitary adenoma waere observed among 4 cases (size; 5 x 7 mm to 10 x 11 mm, shape; ovoid to round signal intensity; high in 1 case on T1 and T2WI, isosignal intensity in the other 3 cases, circumferential change; stalk deviation in 1 case, no stalk deviation in 3 cases). But, the tumors were centrally located at the anterior pituitary gland and showed relatively homogeneous signal intensity on MR images of all 4 patients. We conclude that centrally-located mass at the anterior pituitary gland with homogeneous signal intensity on MR image may be suggestive of the TSH-secreting pituitary adenoma, although the MR findings are not specific for the disease
Vela, A. C.; Oleaga, L.; Ibanez, A. M.; Campo, M.; Grande, D.
To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone deficiencies. In our series of patients, hypothalamic-pituitary dysgenesiss was the most frequent cause of adenohypophyseal deficiencies, and most cases of central diabetes insipidus were secondary to masses in the sellar and suprasellar region. In patients with thalassemia, T2-weighted MR images showed the amount of iron deposited in adenophypophysis. Gadolinium-enhanced studies were useful in the study of masses and when the presence of microadenoma was suspected. (Author) 26 refs
Full Text Available Sarcoidosis is a non-caseating, granulomatous inflammatory disorder that can affect the central nervous system (CNS, including the hypothalamic–pituitary region, although rarely. The clinical manifestations of hypothalamic–pituitary neurosarcoidosis are heterogeneous and require a prompt diagnosis to ensure the most appropriate treatment. We have reviewed the cases of neurosarcoidosis affecting the hypothalamic–pituitary axis published since 2002 and compared them with the cases reported in the literature up to 2002, which were previously meta-analysed by our research group. Since 2002, 64 cases were identified in the literature: 37 cases presented with diabetes insipidus, 36 were found to have secondary amenorrhoea, 30 with hypogonadotropic hypogonadism, 17 with hyperprolactinaemia, 15 with thyroid-stimulating hormone deficiency, and 8 cases of panhypopituitarism. Uncommon manifestations included hyperphagia, sudden death, and thermodysregulation. We confirm that neurosarcoidosis affecting the hypothalamic–pituitary axis is an uncommon manifestation of sarcoidosis. Neither changes in the clinical manifestations and diagnosis nor significantly novel management options have appeared in the last decade. While it is a rare disorder, the involvement of the CNS is an indication to treat sarcoidosis and as the symptoms of CNS involvement, including hypothalamic–pituitary alterations, may precede the diagnosis of sarcoidosis, it is important to include neurosarcoidosis in the differential diagnosis of hypothalamic–pituitary axis dysfunction in order to facilitate prompt and appropriate treatment.
Ogaki, Satoko; Suzuki, Masatsune; Shimano, Hitoshi; Toyoshima, Hideo; Sone, Hirohito; Okuda, Yukichi; Yamada, Nobuhiro
We report a case of cerebral hemorrhagic infarction after radiation for pituitary adenoma. A 55-year-old woman was hospitalized to check for aldosteronism, post-operative pituitary function, and recurrence of thyroid cancer. She had short-term memory disturbance beginning two months prior to admission. Brain MRI showed a T1 and T2 high intensity lesion of her left anterolateral thalamus. Brain MRA revealed a narrowing in her left middle cerebral artery. The abnormal brain lesion was diagnosed as cerebral hemorrhagic infarction. She had received radiation therapy for pituitary adenoma 20 years earlier. It was considered that her cerebral hemorrhagic infarction was caused by radiation therapy. (author)
Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden [Istanbul Faculty of Medicine, Department of Radiology, Istanbul (Turkey); Ozmen, Meral [Istanbul Faculty of Medicine, Department of Paediatric Neurology, Istanbul (Turkey)
Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)
Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden; Ozmen, Meral
Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)
Full Text Available Objective The purpose of this study was to describe the endoscopic combined “transseptal/transnasal” approach with a pedicled nasoseptal flap for pituitary adenoma and skull base reconstruction, especially with respect to cerebrospinal fluid (CSF fistula.Method Ninety-one consecutive patients with pituitary adenomas were retrospectively reviewed. All patients underwent the endoscopic combined “transseptal/transnasal” approach by the single team including the otorhinolaryngologists and neurosurgeons. Postoperative complications related to the flap were analyzed.Results Intra- and postoperative CSF fistulae were observed in 36 (40% and 4 (4.4% patients, respectively. Among the 4 patients, lumbar drainage and bed rest healed the CSF fistula in 3 patients and reoperation for revision was necessary in one patient. Other flap-related complications included nasal bleeding in 3 patients (3.3%.Conclusion The endoscopic combined “transseptal/transnasal” approach is most suitable for a two-surgeon technique and a pedicled nasoseptal flap is a reliable technique for preventing postoperative CSF fistula in pituitary surgery.
Abele, T.A., E-mail: firstname.lastname@example.org [University of Texas Southwestern Medical Center at Dallas, Dallas, TX (United States); Yetkin, Z.F.; Raisanen, J.M.; Mickey, B.E.; Mendelsohn, D.B. [University of Texas Southwestern Medical Center at Dallas, Dallas, TX (United States)
Although the large majority of sellar tumours are pituitary adenomas, several other pituitary and non-pituitary origin tumours arise in the sellar and parasellar regions. Given their location, non-adenomatous lesions frequently mimic pituitary macroadenomas and can pose a diagnostic challenge for the radiologist. Distinguishing rare sellar lesions from the common macroadenoma helps to direct the correct surgical approach and reduce the risk of incomplete resection and/or complications such as cerebrospinal fluid leak with the potential for meningitis. The purpose of this article is to review the imaging features of non-pituitary-origin sellar tumours, focusing on characteristics that may distinguish them from pituitary macroadenomas. Lesions include meningioma, metastatic disease, epidermoid cyst, germinoma, chondrosarcoma, giant cell tumour, and giant aneurysm.
Peng, Zesheng; Tian, Daofeng; Wang, Hongliu; Kong, Derek Kai; Zhang, Shenqi; Liu, Baohui; Deng, Gang; Xu, Zhou; Wu, Liquan; Ji, Baowei; Wang, Long; Cai, Qiang; Li, Mingchang; Wang, Junmin; Zhang, Aimin; Chen, Qianxue
Epistaxis due to ruptured internal carotid artery (ICA) aneurysm embedded within a pituitary adenoma (PA) has seldom been reported in the literature. Here we want to elaborate the incidence, mechanisms, clinical manifestations, and treatment strategy for this condition. The first survived case of a patient with epistaxis and pituitary apoplexy due to ruptured aneurysm embedded within PA was reported and the literature was reviewed. A 53-year-old male patient presented to our institution with sudden onset epistaxis and progressive vision loss. Neurological examination revealed bilateral ptosis and dilated unresponsive pupils. A CT scan showed a large mass in the pituitary fossa with bony erosion. MRI revealed a large pituitary tumor and abnormal signal intensity in the tumor. No aneurysm was noted during the pre-operative MR angiography. Abundant arterial bleeding suddenly occurred during urgent transsphenoidal surgery. Digital subtraction angiography confirmed the presence of a 14 mm unexpected saccular aneurysm of right ICA in the cavernous sinus with the dome protruding into the sella turcica. Balloon test occlusion of the right ICA was undertaken and permanent occlusion was performed. The patient recovered well and received bromocriptine and thyroid hormone replacement therapy during the follow-up period. At 14-month followup, the patient had no neurological deficits, no features of ischaemia relating to the right ICA therapeutic occlusion. Our case indicated that epistaxis and pituitary apoplexy could be due to the rupture of an ICA aneurysm embedded in a PA. Clinical suspicion should remain high when evaluating any case of epistaxis and pituitary apoplexy. Optimal treatment should take into consideration individual features of the tumor, aneurysm, and patient. Making the correct diagnosis as well as identifying an appropriate management strategy is critical in the care of such patients.
Williams, Fred; Hunter, Steven; Bradley, Lisa; Chahal, Harvinder S; Storr, Helen L; Akker, Scott A; Kumar, Ajith V; Orme, Stephen M; Evanson, Jane; Abid, Noina; Morrison, Patrick J; Korbonits, Márta; Atkinson, A Brew
Germline AIP mutations usually cause young-onset acromegaly with low penetrance in a subset of familial isolated pituitary adenoma families. We describe our experience with a large family with R304* AIP mutation and discuss some of the diagnostic dilemmas and management issues. The aim of the study was to identify and screen mutation carriers in the family. Forty-three family members participated in the study. The study was performed in university hospitals. We conducted genetic and endocrine screening of family members. We identified 18 carriers of the R304* mutation, three family members with an AIP-variant A299V, and two family members who harbored both changes. One of the two index cases presented with gigantism and pituitary apoplexy, the other presented with young-onset acromegaly, and both had surgery and radiotherapy. After genetic and clinical screening of the family, two R304* carriers were diagnosed with acromegaly. They underwent transsphenoidal surgery after a short period of somatostatin analog treatment. One of these two patients is in remission; the other achieved successful pregnancy despite suboptimal control of acromegaly. One of the A299V carrier family members was previously diagnosed with a microprolactinoma; we consider this case to be a phenocopy. Height of the unaffected R304* carrier family members is not different compared to noncarrier relatives. Families with AIP mutations present particular problems such as the occurrence of large invasive tumors, poor response to medical treatment, difficulties with fertility and management of pregnancy, and the finding of AIP sequence variants of unknown significance. Because disease mostly develops at a younger age and penetrance is low, the timing and duration of the follow-up of carriers without overt disease requires further study. The psychological and financial impact of prolonged clinical screening must be considered. Excellent relationships between the family, endocrinologists, and
Chan, V W; Tindal, S
The management of anaesthesia for transsphenoidal removal of a pituitary adenoma in a true pituitary giant with acromegaly is described. Problems which may be anticipated in such a patient and an approach to their management are discussed, with particular emphasis upon the need for thorough preoperative assessment of the upper airway and the provision of adequate pulmonary ventilation during anaesthesia.
Figueiredo, Eberval Gadelha; Welling, Leonardo Christiaan; de Faria, Jose Weber Vieira; Teixeira, Manoel Jacobsen
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
INTRODUCTION: Ectopic pituitary adenomas are extremely rare. We report a case of ectopic pituitary adenoma in the midline of the nasopharynx. This adenoma probably arose from the pharyngeal remnant of Rathke\\'s pouch. METHODS: We discuss a case of a lady who presented to our unit with 2 months history of dryness and sensation of lump in her throat and a long standing history of hypothyroidism. Examination of nasopharynx revealed a smooth and fluctuant midline mass. CT scan of nose and paranasal sinuses confirmed the midline mass with small defect communicating with the sphenoid sinus. An initial diagnosis of Thornwaldt\\'s cyst was made and she underwent upper aerodigestive tract endoscopy and marsupialization of the mass. Histopathological examination revealed ectopic pituitary adenoma. CONCLUSION: Ectopic pituitary adenoma is an important differential diagnosis for a midline nasopharyngeal mass. It is recommended that prior to surgical resection of midline nasopharyngeal mass biopsy is taken and MRI is performed.
Hayashi, Motohiro; Chernov, Mikhail F.; Taira, Takaomi; Ochiai, Taku; Nakaya, Kotaro; Tamura, Noriko; Goto, Shinichi; Yomo, Shoji; Kouyama, Nobuo; Katayama, Yoko; Kawakami, Yoriko; Izawa, Masahiro; Muragaki, Yoshihiro
Purpose: To evaluate outcomes after pituitary radiosurgery in patients with post-stroke thalamic pain syndrome. Methods and Materials: From 2002 to 2006, 24 patients with thalamic pain syndrome underwent pituitary radiosurgery at Tokyo Women's Medical University and were followed at least 12 months thereafter. The radiosurgical target was defined as the pituitary gland and its connection with the pituitary stalk. The maximum dose varied from 140 to 180 Gy. Mean follow-up after treatment was 35 months (range, 12-48 months). Results: Initial pain reduction, usually within 48 h after radiosurgery, was marked in 17 patients (71%). However, in the majority of cases the pain recurred within 6 months after treatment, and at the time of the last follow-up examination durable pain control was marked in only 5 patients (21%). Ten patients (42%) had treatment-associated side effects. Anterior pituitary abnormalities were marked in 8 cases and required hormonal replacement therapy in 3; transient diabetes insipidus was observed in 2 cases, transient hyponatremia in 1, and clinical deterioration due to increase of the numbness severity despite significant reduction of pain was seen once. Conclusions: Pituitary radiosurgery for thalamic pain results in a high rate of initial efficacy and is accompanied by acceptable morbidity. It can be used as a primary minimally invasive management option for patients with post-stroke thalamic pain resistant to medical therapy. However, in the majority of cases pain recurrence occurs within 1 year after treatment
Full Text Available Background: The use of endoscope for the management of pituitary adenoma is not new. The better magnification and illumination provided by the endoscope gives better outcome than microscopic pituitary surgery. Objective: To find out the benefits of endoscope in relation to microscopic surgery. Materials and Methods: We performed 45 cases of pituitary adenoma surgery by endoscopic endonasal approach from July 2008 to July 2010. Results: Forty five cases underwent endoscopic transsphenoidal approach. Gross total removal was done in 35 cases and subtotal removal was done in 10 cases. Residual tumours were seen in 10 cases (22% in postoperative follow-up MRI scan. Visual improvement was satisfactory, and hormonal improvement of functional adenoma was nice. Postoperative visual acuity and visual field were improved in 75% cases. There were 37% cases of temporary diabetes insipidus and about 4.5% cases of permanent diabetes insipidus. The average duration of follow-up was 20 months. One patient required reexploration to correct visual deterioration in the immediate postoperative period. There were 4.5% cases of CSF leak and 6.6% mortality. Mortality was due to electrolyte imbalance and improper management of infection and hydrocephalus. Conclusion: Endoscopic endonasal pituitary surgery now has become a gold standard surgery for most of the pituitary adenomas because of its better advantages in relation to microscopic surgery and less complications and less hospital stay.
Full Text Available Management of pituitary tumors is multidisciplinary, with medical therapy playing an increasingly important role. With the exception of prolactin-secreting tumors, surgery is still considered the first-line treatment for the majority of pituitary adenomas. However, medical/pharmacological therapy plays an important role in controlling hormone-producing pituitary adenomas, especially for patients with acromegaly and Cushing disease (CD. In the case of non-functioning pituitary adenomas (NFAs, pharmacological therapy plays a minor role, the main objective of which is to reduce tumor growth, but this role requires further studies. For pituitary carcinomas and atypical adenomas, medical therapy, including chemotherapy, acts as an adjuvant to surgery and radiation therapy, which is often required to control these aggressive tumors. In the last decade, knowledge about the pathophysiological mechanisms of various pituitary adenomas has increased, thus novel medical therapies that target specific pathways implicated in tumor synthesis and hormonal over secretion are now available. Advancement in patient selection and determination of prognostic factors has also helped to individualize therapy for patients with pituitary tumors. Improvements in biochemical and “tumor mass” disease control can positively affect patient quality of life, comorbidities and overall survival. In this review, the medical armamentarium for treating CD, acromegaly, prolactinomas, NFA, and carcinomas/aggressive atypical adenomas will be presented. Pharmacological therapies, including doses, mode of administration, efficacy, adverse effects, and use in special circumstances are provided. Medical therapies currently under clinical investigation are also briefly discussed.
Rosenstock, J.; Doyle, F.H.; Joplin, G.F.; Jung, R.T.; Mashiter, K.
A brief case history is presented of a patient, who, after yttrium-90 implantation, showed a complete clinical and hormonal remission of her acromegaly, maintaining normal pituitary function. The remarkable feature was the rapid disappearance of her attacks of somnolence within 96 hours of pituitary implantation, despite persistence of nocturnal snoring and well before any remodelling of soft tissues could have occurred. This response suggests that her daytime somnolence had a narcoleptic component. (author)
Rosenstock, J.; Doyle, F.H.; Joplin, G.F.; Jung, R.T.; Mashiter, K. (Hammersmith Hospital, London (UK). Postgraduate Medical School)
A brief case history is presented of a patient, who, after yttrium-90 implantation, showed a complete clinical and hormonal remission of her acromegaly, maintaining normal pituitary function. The remarkable feature was the rapid disappearance of her attacks of somnolence within 96 hours of pituitary implantation, despite persistence of nocturnal snoring and well before any remodelling of soft tissues could have occurred. This response suggests that her daytime somnolence had a narcoleptic component.
Full Text Available Michelin Tire Baby Syndrome is a rare congenital disorder and characterized clinically well defined multiple ring shaped skin creases. Our patient was born to onconsanguineous healthy parents as the third child of the family at 40 weeks of uneventful gestation with distinctive skin creases and gigantism. He was 4,950 g in weight (>90 percentile, 57.5 cm in length (>90 percentile, and had a head circumferences of 39.5 cm (>90 percentile at birth. The physical examination showed a rough face, brachicephaly and craniosynostosis. His vital and laboratory findings were within normal limits at birth. Cranial and renal ultrasonograms, Xray graphics and cytogenetic analyses were normal. Echocardiography revealed small patent ductus arteriosis and patent foramen ovale. In this report, we present a new case of Michelin Tire Baby Syndrome who is the first neonate associated with severe gigantism and craniosynostosis, in the literature. A review of the related literature has also been presented.
Ambrosetto, P.; Zucchini, S.; Cicognani, A.; Cacciari, E.
A girl with Diamond-Blackfan syndrome and hypopituitarism was suspected of having pituitary haemosiderosis because of the clinical picture and the long history of blood transfusions. On T1-weighted MR images the pituitary exhibited a markedly hypointense anterior lobe (mimicking the empty sella), suggesting iron deposition, while on T2W MRI the low signal of the pituitary was surrounded by the high signal of the CSF. MR may be considered the examination of choice for detecting iron overload in the pituitary. (orig.)
Pituitary tumors represent from 10% to 25% of all intracranial neoplasms. Pituitary tumors can be classified into three groups: benign adenoma, invasive adenoma, and carcinoma. Find evidence-based information on pituitary tumors treatment.
Yan, Qing; Zhang, Hua-qiu; Wang, He-ping; Guo, Dong-sheng; Lei, Ting; Li, Ling
To study the relationship between the clinical presentation, endocrinal findings and pathological types in patients with pituitary microadenomas, so as to improve the accuracy of clinical diagnosis and choose the best therapy strategy before the operation. From January 2007 to June 2009, the clinical data of 94 patients who were surgically removed pituitary microadenomas were obtained, including the clinical presentation, endocrinal findings and pathological diagnosis. The analysis was accomplished with Chi-square test. Hormonal symptoms were found in 86 patients (91.5%), it occurred more frequently in immunopositive patients (85/92, 92.4%) than in immunonegative patients (1/2, 50.0%) (P gigantism or acromegaly in growth hormone (GH) positive group. The coincidence of endocrinal findings and immunohistochemistry diagnosis was 69.0%; 87.7% patients had high level of blood PRL in PRL positive group and 21.9% patients had high level of blood GH in GH positive group. There is an obvious relationship between the clinical presentation, endocrinal findings and pathological diagnosis in patients with pituitary microadenomas, which may contribute to the clinical diagnosis and treatment of pituitary secreting microadenomas.
Yamanaka, Masami; Uozumi, Tohru; Sakoda, Katsuaki; Ohta, Masahiro; Kagawa, Yoshihiro; Kajima, Toshio.
Magnetic resonance imaging (MRI) is a suitable procedure for diagnosing such midline-positioned lesions as pituitary adenomas. To differentiate them from microadenomas fifty-seven cases (9 - 74 years old, 29 men and 28 women), including 50 patients without any sellar or parasellar diseases and seven normal volunteers, were studied in order to clarify the MR findings of the shape, height, and signal intensity of the normal pituitary gland, especially at the median sagittal section. The height of a normal pituitary gland varied from 2 to 9 mm (mean: 5.7 mm); the upper surface of the gland was convex in 19.3 %, flat in 49.1 %, and concave in 31.6 %. The mean height of the gland in women in their twenties was 7.5 mm, and the upper convex shape appeared exclusively in women of the second to fourth decades. Nine intrasellar pituitary adenomas (PRL-secreting: 4, GH-secreting: 4, ACTH-secreting: 1), all verified by surgery, were diagnosed using a resistive MR system. The heights of the gland in these cases were from 7 to 15 mm (mean: 11.3 mm); the upper surface was convex in 7 cases. A localized bulging of the upper surface of the gland and a localized depression of the sellar floor were depicted on the coronal and sagittal sections in most cases. Although the GH- and ACTH-secreting adenoma cases showed homogeneous intrasellar contents, in all the PRL-secreting adenoma cases a low-signal-intensity area was detected in the IR images. The mean T1 values of the intrasellar content of the normal volunteers, the PRL-, GH-, and ACTH-secreting adenoma cases, were 367, 416, 355, and 411 ms respectively. However, in the PRL-secreting adenoma cases, the mean T1 value of the areas showing a low signal intensity on IR images was 455 ms; this was a significant prolongation in comparison with that of a normal pituitary gland. (J.P.N.)
Wu, Ping; Ligatchev, Valeri; Yu, Zhi Gen; Zheng, Jianwei; Sullivan, Michael B.; Zeng, Yingzhi
We combine first-principles, statistical, and phenomenological methods to investigate the electronic and dielectric properties of NiO and clarify the nature of the gigantic dielectric response in codoped NiO. Unlike previous models which are dependent on grain-boundary effects, our model based on small polaron hopping in homogeneous material predicts the dielectric permittivity (104-5) for heavily Li- and MD -codoped NiO (MD=Ti,Al,Si) . Furthermore, we reproduce the experimental trends in dielectric properties as a function of the dopants nature and their concentrations, as well as the reported activation energies for the relaxation in Li- and Ti-codoped NiO (0.308 eV or 0.153 eV depending on the Fermi-level position). In this study, we demonstrate that small polaron hopping on dopant levels is the dominant mechanism for the gigantic dielectric response in these codoped NiO.
Bell, Phil R; Campione, Nicolás E; Persons, W Scott; Currie, Philip J; Larson, Peter L; Tanke, Darren H; Bakker, Robert T
Recent evidence for feathers in theropods has led to speculations that the largest tyrannosaurids, including Tyrannosaurus rex , were extensively feathered. We describe fossil integument from Tyrannosaurus and other tyrannosaurids ( Albertosaurus, Daspletosaurus, Gorgosaurus and Tarbosaurus ), confirming that these large-bodied forms possessed scaly, reptilian-like skin. Body size evolution in tyrannosauroids reveals two independent occurrences of gigantism; specifically, the large sizes in Yutyrannus and tyrannosaurids were independently derived. These new findings demonstrate that extensive feather coverings observed in some early tyrannosauroids were lost by the Albian, basal to Tyrannosauridae. This loss is unrelated to palaeoclimate but possibly tied to the evolution of gigantism, although other mechanisms exist. © 2017 The Author(s).
Sen, Debraj; Arora, Vijinder
Duplication of the pituitary gland (DPG) is a very rare developmental anomaly that is often associated with other anomalies – the DPG-plus syndrome and occurs due to splitting of the rostral notochord and prechordal plate during blastogenesis. DPG with the constellation of associated anomalies as in our patient has not been reported previously. This article illustrates the importance of imaging the brain in all patients with obvious midline facial anomalies and the complementary role of MRI and CT in such cases
Gondim, Jackson A; Schops, Michele; de Almeida, João Paulo C; de Albuquerque, Lucas Alverne F; Gomes, Erika; Ferraz, Tânia; Barroso, Francisca Andréa C
Pituitary tumors are challenging tumors in the sellar region. Surgical approaches to the pituitary have undergone numerous refinements over the last 100 years. The introduction of the endoscope have revolutionized pituitary surgery. The aim of this study is to report the results of a consecutive series of patients undergoing pituitary surgery using a pure endoscopic endonasal approach and to evaluate the efficacy and safety of this procedure. We reviewed the data of 228 consecutive patients who underwent endonasal transsphenoidal adenoma removal over an 10-year period. Pre- and post-operative hormonal status (at least 3 months after surgery) were analyzed and compared with clinical parameters presented by the patients. Tumor removal rate, endocrinological outcomes, and complications were retrospectively assessed in 228 patients with pituitary adenomas who underwent 251 procedures between December 1998 and December 2007. There were 93 nonfunctioning adenomas, 58 growth hormone-secreting, 41 prolactin-secreting, 28 adrenocorticotropin hormone secreting, 7 FSH-LH secreting and 1 thyroid-stimulating hormone-secreting adenomas. Gross total removal was achieved in 79.3% of the cases after a median follow-up of 61.5 months. The remission results for patients with nonfunctioning adenomas was 83% and for functioning adenomas were 76.3% (70.6% for GH hormone-secreting, 85.3% for prolactin hormone-secreting, 71.4% for ACTH hormone-secreting, 85.7% for FSH-LH hormone-secreting and 100% for TSH hormone-secreting), with no recurrence at the time of the last follow-up. Post-operative complications were present in 35 (13.9%) cases. The most frequent complications were temporary and permanent diabetes insipidus (six and two cases, respectively), syndrome of inappropriate antidiuretic hormone secretion (two cases) and CSF leaks (eight cases). There was no death related to the procedure in this series. The endoscopic endonasal approach for resection of pituitary adenomas, provides
Tsai, Cheng-Hsiu; Kohno, Naoki
Living baleen whales (Mysticeti) include the world's largest animals to have ever lived—blue whales ( Balaenoptera musculus) can reach more than 30 m. However, the gigantism in baleen whales remains little explored. Here, we compiled all published stem mysticetes from the Eocene and Oligocene and then mapped the estimated body size onto different phylogenies that suggest distinct evolutionary histories of baleen whales. By assembling all known stem baleen whales, we present three novel findings in early mysticete evolution. Results show that, regardless of different phylogenetic scenarios, large body size (more than 5-m long) evolved multiple times independently in their early evolutionary history. For example, the earliest known aetiocetid ( Fucaia buelli, 33-31 Ma) was small in size, about 2 m, and a later aetiocetid ( Morawanocetus-like animal, 26-23 Ma) can reach 8-m long—almost four times the size of Fucaia buelli—suggesting an independent gigantism in the aetiocetid lineage. In addition, our reconstruction of ancestral state demonstrates that the baleen whales originated from small body size (less than 5 m) rather than large body size as previously acknowledged. Moreover, reconstructing the evolution of body size in stem baleen whales suggests that the initial pulse of mysticete gigantism started at least back to the Paleogene and in turn should help to understand the origin, pattern, and process of the extreme gigantism in the crown baleen whales. This study illustrates that Cope's rule is insufficient to explain the evolution of body size in a group that comprises the largest animals in the history of life, although currently the lack of exact ancestor-descendant relationships remains to fully reveal the evolutionary history of body size.
Post-partum pituitary insufficiency and livedo reticularis presenting a diagnostic challenge in a resource limited setting in Tanzania: a case report, clinical discussion and brief review of existing literature
Sheriff Faheem G
Full Text Available Abstract Background Pituitary disorders following pregnancy are an important yet under reported clinical entity in the developing world. Conversely, post partum panhypopituitarism has a more devastating impact on women in such settings due to high fertility rates, poor obstetric care and scarcity of diagnostic and therapeutic resources available. Case presentation A 37 year old African female presented ten years post partum with features of multiple endocrine deficiencies including hypothyroidism, hypoadrenalism, lactation failure and secondary amenorrhea. In addition she had clinical features of an underlying autoimmune condition. These included a history of post-partum thyroiditis, alopecia areata, livedo reticularis and deranged coagulation indices. A remarkable clinical response followed appropriate hormone replacement therapy including steroids. This constellation has never been reported before; we therefore present an interesting clinical discussion including a brief review of existing literature. Conclusion Post partum pituitary insufficiency is an under-reported condition of immense clinical importance especially in the developing world. A high clinical index of suspicion is vital to ensure an early and correct diagnosis which will have a direct bearing on management and patient outcome.
Chatterjee, Anirban; Chatterjee, Shamita
Subcutaneous and deep fungal infections in the hand are rare among children. These are usually found in immunocompromised adults or in persons engaged in soil handling activities, due to direct exposure, especially in the tropics. Delay in diagnosis is usual because pyogenic and other granulomatous infections are considered first. The authors present the case of a healthy, immunocompetent 2½-year-old child who presented with progressive swelling of the right hand mimicking a localized gigantism of the entire hand. Multiple operative drainage procedures done previously had failed to resolve the condition. A biopsy established the presence of fungal hyphae, thus confirming the diagnosis of deep fungal infection of the hand and guided proper therapeutic intervention. A strong index of suspicion needs to be maintained in cases not responding to conventional antibacterial therapy, and both microbiologic and histopathologic samples need to be obtained to establish the diagnosis.
Camila Ferro Miele
Full Text Available OBJETIVO: Relatar um caso de teratoma cervical congênito, destacando a gravidade e as dificuldades terapêuticas associadas. DESCRIÇÃO DO CASO: Mãe de 30 anos, com gestação por fertilização assistida. Com 23 semanas, diagnosticada malformação cervical fetal à direita. Parto cesáreo por indicação fetal com 31 semanas. Recém-nascido masculino, peso ao nascer de 1800g, Apgar 4 e 9, com volumoso processo expansivo à direita, ocupando toda a região cervical, comprometendo a mandíbula e estendendo-se para o terço superior do tórax. Com 40 horas de vida, apresentou insuficiência cardíaca congestiva de alto débito por roubo de fluxo pelo tumor. A partir de 54 horas de vida, houve progressiva deterioração hemodinâmica e respiratória, com hipotensão, anúria e labilidade de oxigenação, refratárias às aminas vasoativas, reposição de volume e aumento do suporte ventilatório. Indicada abordagem cirúrgica para ressecção tumoral, todavia o paciente não apresentou estabilidade clínica que permitisse seu transporte ao centro cirúrgico e faleceu com 70 horas de vida. COMENTÁRIOS: O caso demonstra as dificuldades relacionadas à abordagem pós-natal dos teratomas cervicais volumosos. Apesar do diagnóstico pré-natal, o paciente evoluiu com obstrução de vias aéreas, complicada por um choque cardiogênico refratário, que culminou no óbito. A abordagem intraparto é fundamental nesses pacientes, consistindo em exérese tumoral, enquanto a manutenção da circulação materno-fetal permite a oxigenação fetal contínua. A evolução neonatal no caso descrito é condizente com a literatura que mostra prognóstico reservado quando não é realizada a abordagem cirúrgica intraparto.OBJECTIVE: To report a case of congenital cervical teratoma, highlighting the severity and the therapeutic difficulties associated. CASE DESCRIPTION: A 30-year old mother, with pregnancy by assisted fertilization. At 23 weeks, a cervical fetal
meningeal irritation is not considered a classic feature of pituitary apoplexy.2,3 The pathophysiology behind this symptom complex involves leakage of blood into the subarachnoid space, which, in conjunction with the necrotic tissue in the pituitary itself, induces a cytokine response, resulting in meningeal irritation and the.
Di Iorgi, Natascia; Morana, Giovanni; Gallizia, Anna Lisa; Maghnie, Mohamad
Magnetic resonance imaging (MRI) allows a detailed and precise anatomical study of the pituitary gland by differentiating between the anterior and posterior pituitary lobes. The identification of posterior pituitary hyperintensity, now considered a marker of neurohypophyseal functional integrity, has been the most striking advance for the diagnosis and understanding of anterior and posterior pituitary diseases. The advent of MRI has in fact led to a significant improvement in the understanding of the pathogenesis of disorders that affect the hypothalamo-pituitary area. Today, there is convincing evidence to support the hypothesis that marked MRI differences in pituitary morphology indicate a diverse range of disorders which affect the organogenesis and function of the anterior pituitary gland with different prognoses. Furthermore, the association of extrapituitary malformations accurately defined by MRI has supported a better definition of several conditions linked to pituitary hormone deficiencies and midline defects. MRI is a very informative procedure that should be used to support a diagnosis of hypopituitarism. It is useful in clinical management, because it helps endocrinologists determine which patients to target for further molecular studies and genetic counselling, which ones to screen for additional hormone deficits, and which ones may need growth hormone replacement into adult life. Copyright © 2012 S. Karger AG, Basel.
Nov 4, 2013 ... crinology with short stature, delayed bone age and biochemical features suggestive of hypo pituitarism. Magnetic resonance imaging of the brain demonstrated a flattened anterior pituitary gland within the sella, associated with absence of the infundibular stalk and an ectopic posterior pituitary gland (Fig.
Chaudhary, Vikas; Bano, Shahina
Accurate investigation of the hypothalamic-pituitary area is required in pediatric patients for diagnosis of endocrine-related disorders. These disorders include hypopituitarism, growth failure, diencephalic syndrome, delayed puberty, precocious puberty, diabetes insipidus, syndrome of inappropriate antidiuretic hormone (SIADH) secretion, and hyperpituitarism. Magnetic resonance imaging (MRI) is the modality of choice to visualize hypothalamic-pituitary axis and associated endocrinopathies. Neuroimaging can be normal or disclose abnormalities related to pituitary-hypothalamic axis like (i) congenital and developmental malformations; (ii) tumors; (iii) cystic lesions; and (iv) infectious and inflammatory conditions. Classical midline anomalies like septo-optic dysplasias or corpus callosum agenesis are commonly associated with pituitary endocrinopathies and also need careful evaluation. In this radiological review, we will discuss neuroendocrine disorders related to hypothalamic pituitary-axis. PMID:23087850
Yu, Huanxin; Liu, Gang
To evaluate the effectiveness of transsphenoidal endoscopic endonasal approach for the surgery of pituitary abscess. Eighteen pathologically diagnosed pituitary abscess were resected through transsphenoidal endoscopic endonasal approach at Tianjing Huanhu hospital between January 2000 and December 2011.Retrospective analysis was done upon clinical presentations and imaging features. There were 6 males and 12 females. The average age was 48.5 years old and the average disease course was 5.8 years. The typical clinical manifestations included headache (13 cases), pituitary dysfunction (10 cases), Diabetes Insipidus (4 cases) visual interference (8 cases) and fever (4 cases). All cases were resected by transsphenoidal endoscopic endonasal approach with general anesthesia. The postoperative symptoms and follow-up results were recorded. All patients were followed up from 6 months to 6 years. Postoperatively, headache was recovered in 13 cases, visual was improved in 6 cases, hypopituitarism was relieved in 8 cases and polyuria was disappeared in 3 cases. One case was recurrent and cured by transsphenoidal endoscopic endonasal approach. Transsphenoidal endoscopic endonasal approach for the surgery of pituitary abscess is effective.
Full Text Available More than 99% of patients with acromegaly harbor a growth hormone (GH secreting pituitary adenoma. As the time from onset of signs/symptoms to diagnosis of acromegaly is long (symptom onset to diagnosis is often 4-10 years, pituitary adenomas that cause GH excess are often large and are nearly always visible on conventional magnetic resonance imaging (MRI. However, in rare circumstances, acromegalic patients without an ectopic source will not have imaging evidence of a pituitary adenoma. Management of these patients poses special challenge, and once ectopic source of GH/growth-hormone-releasing hormone (GHRH is ruled out, an exploration of pituitary might be useful. We herein report a case of acromegaly with imaging evidence of sellar floor osteoma, but no pituitary adenoma, and negative work up for an ectopic source of GH/GHRH tumor, and on surgical exploration pituitary adenoma could be identified and removed and confirmed on histopathologic examination.
Full Text Available Sixty patients with non-functional pituitary adenomas were observed. Most patients had large-cell chromophobe pituitary adenomas (81.6%. Small-cell chromophobe adenomas occurred in 10 % cases. Only 1 patient (3.3 % had giant carcinoma with regrowth and metastasis into the brain. Markers of aggression of non-functional pituitary adenomas are the young age of a patient, expressed first symptoms of disease manifestation, large size of tumor, asymmetry and deformation of pituitary, invasion of tumor to the neighboring tissues/arteries/cavernous sinus, presence of small cell and dark-cell chromophobe adenoma, panhypopituitarism.
Uppal, Surabhi; Jee, Youn Hee; Lightbourne, Marissa; Han, Joan C; Stratakis, Constantine A
Tetrasomy X is a rare chromosomal aneuploidy seen in girls, associated with facial dysmorphism, premature ovarian insufficiency and intellectual disability. A Rathke's cleft cyst (RCC) is a remnant of Rathke's pouch which may cause multiple pituitary hormone deficiencies by exerting pressure on the pituitary gland in the sella. The patient was diagnosed with tetrasomy X by karyotyping during infancy. Brain MRI and multiple endocrine stimulation tests revealed RCC and combined pituitary hormone deficiency (growth hormone deficiency, secondary adrenal insufficiency and central hypothyroidism) likely due to RCC. We report the first case in the literature of a girl with 48, XXXX and combined pituitary hormone deficiency due to Rathke's cyst.
Full Text Available Objective. In pediatric central diabetes insipidus (CDI, etiology diagnosis and pituitary function monitoring are usually delayed. This study aimed to illustrate the importance of regular follow-up and pituitary function monitoring in pediatric CDI. Methods. The clinical, hormonal, and neuroradiological characteristics of children with CDI at diagnosis and during 1.5–2-year follow-up were collected and analyzed. Results. The study included 43 CDI patients. The mean interval between initial manifestation and diagnosis was 22.29 ± 3.67 months (range: 2–108 months. The most common complaint was polyuria/polydipsia. Causes included Langerhans cell histiocytosis, germinoma, and craniopharyngioma in 2, 5, and 4 patients; the remaining were idiopathic. No significant changes were found during the 1.5–2 years after CDI diagnosis. Twenty-three of the 43 cases (53.5% had ≥1 anterior pituitary hormone deficiency. Isolated growth hormone deficiency was the most frequent abnormality (37.5% and was not associated with pituitary stalk diameter. Multiple pituitary hormone deficiencies were found in 8 cases with pituitary stalk diameter > 4.5 mm. Conclusion. Diagnosis of CDI is usually delayed. CDI with a pituitary stalk diameter > 4.5 mm carries a higher risk of multiple pituitary hormone deficiencies. Long-term MRI and pituitary function follow-ups are necessary for children with idiopathic CDI.
Ayelén María Portelles Massó
Full Text Available El granuloma reparativo central de células gigantes es una lesión proliferativa no neoplásica de etiología desconocida. Se presenta un paciente masculino de 40 años de edad, portador de prótesis parcial superior. Fue remitido al Servicio de Cirugía Maxilofacial del Hospital "V. I. Lenin" por presentar aumento de volumen en reborde alveolar superior, de color rojo grisáceo y que provocaba expansión de corticales óseas. Una vez analizados los exámenes clínicos, radiográficos e histopatológicos se diagnosticó un granuloma reparativo central de células gigantes Se realizó exéresis quirúrgica de la lesión y extracción de dientes adyacentes con una evolución satisfactoria sin señales de recidivas luego de tres años del tratamiento. El granuloma reparativo central de células gigantes se presentó como respuesta a un trauma. La correcta interpretación de los datos clínicos, radiográficos e histopatológicos nos permitió llegar al correcto diagnóstico y plan de tratamiento.Giant-cell central reparative granuloma is non neoplastic proliferative lesion of unknown etiology. We report a 40 years old male patient who was admitted at the Maxillofacial Service of the "V. I. Lenin" Hospital. The patient had partial upper prosthesis and was complaining of red-grey volume increase lesion in upper alveolar ridge which led to the expansion of cortical bone. Having analyzed clinical, radiographic and histopathological findings the case was concluded as a giant-cell central reparative granuloma. Surgical exeresis and adjunct tooth extraction were done. After three years of treatment, satisfactory follow up without recurrence is reported.
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.
Tratamiento quirúrgico del granuloma central de células gigantes: estudio y seguimiento de 10 casos. Revisión de la literatura Surgical treatment of central giant-cell granuloma: Study and follow-up of 10 cases. Literature review
M. Fernández Ferro
Full Text Available Objetivos: El granuloma central de células gigantes (GCCG es una lesión benigna, no odontogénica, de origen incierto y con unas características epidemiológicas, clínicas y radiológicas bien conocidas. El objetivo de nuestro estudio es recoger, describir y evaluar nuestra experiencia en el tratamiento quirúrgico de este tipo de lesiones y su seguimiento, haciendo especial hincapié en los datos clínicos y radiológicos así como en las complicaciones y recidivas tras el mismo. Se realiza, además, una revisión de la literatura científica. Material y métodos: Se trata de un estudio retrospectivo de 10 casos intervenidos en nuestro centro entre los años 1998 y 2008. La edad media fue de 35 años. Se describen las características clínicas y radiológicas de cada caso, así como la mejor opción de tratamiento quirúrgico, evaluación del mismo, complicaciones y recidivas. Resultados: Se realiza tratamiento quirúrgico en los 10 casos, de los cuales 4 presentan características clínicas y radiológicas de agresividad. El tratamiento de elección es la escisión y curetaje hasta hueso sano en el 60%, y en el resto resección amplia con márgenes. El periodo de seguimiento medio es de 6,7 años, con una tasa de recidiva del 1%. Conclusión: En nuestra experiencia coincidimos con la mayoría de los autores al considerar el tratamiento quirúrgico de elección en el momento actual, señalando la importancia de realizar un adecuado diagnóstico y una clara diferenciación entre lesiones agresivas y no agresivas para determinar la radicalidad de la intervención.Objectives: Central giant-cell granuloma (CGCG is a benign, non-odontogenic lesion of uncertain origin and well-known epidemiologic, clinical and radiologic characteristics. The aim of this study was to compile, describe and evaluate the authors' experience with the surgical treatment of CGCG and its follow-up, with special emphasis on clinical and radiologic data, complications and
Splitt, M. E.; Lazarus, S. M.
The meteorological conditions of gigantic jet (GJ) producing thunderstorms tend to be connected to maritime tropical environments. In particular, they have an affinity toward tropical disturbances including those with moderate values of upper tropospheric environmental wind shear. Wind shear related effects (including turbulence) in association with deep convection in these environments have been proposed as mechanisms for the arrangement of GJ favorable charge structures. This study focuses on a climatological evaluation in an effort to assess whether the proposed ingredients are consistent with observed GJ event regions. The Climate System Forecast System - Version 2 (CFSR V2) is used here to test for the proposed GJ conditions.
Mahta, Ali; Buhl, Ralf; Huang, Hongguang; Jansen, Olav; Kesari, Santosh; Ulmer, Stephan
A few number of suprasellar gliomas have been reported thus far of which, some of them developed several years after radiation therapy for pituitary adenomas or craniopharyngiomas. Herein, we report a case of sellar glioblastoma with suprasellar extension with no prior radiation history who mimicked clinical and radiologic findings of a pituitary macroadenoma.
Autoimmune endocrine disorders are characterised by the development of autoantibodies to specific autoantigens in the target organs. Lymphocytic hypophysitis (LyH) is a disease characterised by inflammation of the pituitary gland, often resulting in hypopituitarism. The aetiology of LyH is considered to be autoimmune. However, only a few pituitary autoantigens have so far been identified. Reliable autoantibody markers are requested in the diagnostic procedure of LyH to avoid...
Matsusaki, Takashi; Morimatsu, Hiroshi; Matsumi, Junya; Matsuda, Hiroaki; Sato, Tetsufumi; Sato, Kenji; Mizobuchi, Satoshi; Yagi, Takahito; Morita, Kiyoshi
Pituitary apoplexy occurring after surgery is a rare but life-threatening acute clinical condition that follows extensive hemorrhagenous necrosis within a pituitary adenoma. Pituitary apoplexy has been reported to occur spontaneously in the majority of cases or in association with various inducing factors. Reported is a case of pituitary apoplexy complicated by diabetes insipidus following living donor liver transplantation (LDLT). To the best of our knowledge, this has not been previously reported. A 56-year-old woman with nonalcoholic steatohepatitis underwent LDLT from her daughter. The patient also required dopamine support and transfusions because of massive intraoperative bleeding. Postoperatively, her coagulopathy continued, and she underwent a second laparotomy because of unknown bleeding on postoperative day 7, when she needed transfusions and dopamine support to maintain her vital signs. She complained of severe headache, excessive thirst, frequent urination, and diplopia from postoperative day 10. She also had polyuria greater than 300 ml/h and was diagnosed with pituitary apoplexy precipitating diabetes insipidus on postoperative day 13. She was treated conservatively without surgery because of the hormonally inactive status and slight mass effect of her tumor. It is important for anesthesiologists and critical care personnel in LDLT settings to take into consideration this complication as a differential diagnosis.
Manuel César Fontes Maestri
Full Text Available El liposarcoma es, entre los sarcomas, el tumor maligno de los tejidos blandos más frecuente en el adulto. Se presenta un caso de liposarcoma situado en el mediastino, localización infrecuente, y que resultó ser un liposarcoma bien diferenciado. El paciente fue un hombre de 48 años de edad que ingresa en la Sala de Neumotisiología con disnea y una masa mediastínica situada hacia el hemitórax izquierdo. Se estudió con radiografía de tórax anteroposterior y lateral y, además, con tomografía axial computadorizada. Fue necesaria una toracotomía con urgencia relativa por la agudización del cuadro clínico mediastínico compresivo. La evolución fue buena durante la intervención quirúrgica y después de ella y actualmente ha concluido su tratamiento adyuvante (radioterapia y quimioterapia y se siente bienLiposarcoma is the most frequent malignant soft tissue tumor. This article presented a case of well-differentiated liposarcoma located in the mediastinum, which is a rare location. The patient was a 48 years-old man that was admitted to the pneumothysiology service because he was short of breath and had a mediastinal mass located near left hemithorax. He was studied using anteroposterior and lateral thoracic radiography in addition to computerized tomography. It was necessary to urgently perform thorachotomy due to his acute clinical picture with mediastinal compression. The patient evolved positively during surgery and afterwards; at present, he has finished his adjuvant treatment based on radiotherapy and chemotherapy and he feels good
Jul 28, 2016 ... Pituitary adenomas are almost always benign (>99.9%), arise from the anterior pituitary and ... Non-functioning pituitary macroadenomas (NFMA) are the most ... pituitary gland, most likely due to alterations in perfusion.
Full Text Available Duplication of the pituitary gland (DPG is a very rare developmental anomaly that is often associated with other anomalies - the DPG-plus syndrome and occurs due to splitting of the rostral notochord and prechordal plate during blastogenesis. DPG with the constellation of associated anomalies as in our patient has not been reported previously. This article illustrates the importance of imaging the brain in all patients with obvious midline facial anomalies and the complementary role of MRI and CT in such cases.
Koutourousiou, Maria; Gardner, Paul A; Fernandez-Miranda, Juan C; Paluzzi, Alessandro; Wang, Eric W; Snyderman, Carl H
Giant pituitary adenomas (> 4 cm in maximum diameter) represent a significant surgical challenge. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for these tumors. The authors present the results of EES for giant adenomas and analyze the advantages and limitations of this technique. The authors retrospectively reviewed the medical files and imaging studies of 54 patients with giant pituitary adenomas who underwent EES and studied the factors affecting surgical outcome. Preoperative visual impairment was present in 45 patients (83%) and partial or complete pituitary deficiency in 28 cases (52%), and 7 patients (13%) presented with apoplexy. Near-total resection (> 90%) was achieved in 36 patients (66.7%). Vision was improved or normalized in 36 cases (80%) and worsened in 2 cases due to apoplexy of residual tumor. Significant factors that limited the degree of resection were a multilobular configuration of the adenoma (p = 0.002) and extension to the middle fossa (p = 0.045). Cavernous sinus invasion, tumor size, and intraventricular or posterior fossa extension did not influence the surgical outcome. Complications included apoplexy of residual adenoma (3.7%), permanent diabetes insipidus (9.6%), new pituitary insufficiency (16.7%), and CSF leak (16.7%, which was reduced to 7.4% in recent years). Fourteen patients underwent radiation therapy after EES for residual mass or, in a later stage, for recurrence, and 10 with functional pituitary adenomas received medical treatment. During a mean follow-up of 37.9 months (range 1-114 months), 7 patients were reoperated on for tumor recurrence. Three patients were lost to follow-up. Endoscopic endonasal surgery provides effective initial management of giant pituitary adenomas with favorable results compared with traditional microscopic transsphenoidal and transcranial approaches.
Guo Lingchuan; Zheng Yushuang; Wang Shouli; Hui Guozhen; Li Xiangdong
Objective: To study the pathological morphologic characteristics of invasive pituitary tumor and the affect of vascularization to the tumor's invasion. Methods: One hundred and thirty cases of pituitary adenoma patients were divided into two groups, including invasive pituitary adenomas and non-invasive pituitary adenomas, and the clinical data of two groups were analysed and compared. Results : The difference was statistically significant between the invasive group and the non-invasive group in the incidence rate of pathological morphologic characteristics such as high nuclear cytoplasmic ratio, cell pleomorphism, nuclear atypia and nucleoli appearance (P<0.05); there were nuclear atypia and nucleolus margination in the invasive group through electron microscopy. And there was statistical significant difference in rate of MVD expression which was higher in the invasive group than that of noninvasive group (P<0.05). Conclusion: The pathological morphologic characteristics of pituitary tumor and the high expression of MVD are significantly reference valuable in tumor aggression diagnosis, which provides valuable indicators for early clinical diagnosis of tumor invasion. (authors)
Aryan, Saritha; Thakar, Sumit; Hegde, A S
Infarction in the artery of Percheron territory is a rare phenomenon in which occlusion of an unpaired perforating artery arising from the P1 segment on one side results in infarcts in the bilateral paramedian thalami with or without midbrain infarcts. We describe the case of a 40-year-old male who developed this complication following re-exploratory trans-sphenoidal surgery for a pituitary adenoma. In this first report of its kind in endoscopic pituitary surgery, the pathogenesis and clinico-radiological features of this rare vascular event are discussed.
Miller, Brandon A; Ioachimescu, Adriana G; Oyesiku, Nelson M
To analyze current indications for transsphenoidal pituitary surgery. The current literature regarding transsphenoidal surgery for all subtypes of pituitary adenomas and other sellar lesions was examined. Alternate approaches for pituitary surgery were also reviewed. Transsphenoidal surgery continues to be the mainstay of surgical treatment for pituitary tumors, and has good outcomes in experienced hands. Pre- and postoperative management of pituitary tumors remains an important part of the treatment of patients with pituitary tumors. Even as medical and surgical treatment for pituitary tumors evolves, transsphenoidal surgery remains a mainstay of treatment. Outcomes after transshenoidal surgery have improved over time. Neurosurgeons must be aware of the indications, risks and alternatives to transsphenoidal pituitary surgery. Copyright © 2014 Elsevier Inc. All rights reserved.
Gadelha, M R; Prezant, T R; Une, K N; Glick, R P; Moskal, S F; Vaisman, M; Melmed, S; Kineman, R D; Frohman, L A
Familial acromegaly/gigantism occurring in the absence of multiple endocrine neoplasia type I (MEN-1) or the Carney complex has been reported in 18 families since the biochemical diagnosis of GH excess became available, and the genetic defect is unknown. In the present study we examined 2 unrelated families with isolated acromegaly/gigantism. In family A, 3 of 4 siblings were affected, with ages at diagnosis of 19, 21, and 23 yr. In family B, 5 of 13 siblings exhibited the phenotype and were diagnosed at 13, 15, 17, 17, and 24 yr of age. All 8 affected patients had elevated basal GH levels associated with high insulin-like growth factor I levels and/or nonsuppressible serum GH levels during an oral glucose tolerance test. GHRH levels were normal in affected members of family A. An invasive macroadenoma was found in 6 subjects, and a microadenoma was found in 1 subject from family B. The sequence of the GHRH receptor complementary DNA in 1 tumor from family A was normal. There was no history of consanguinity in either family, and the past medical history and laboratory results excluded MEN-1 and the Carney complex in all affected and unaffected screened subjects. Five of 8 subjects have undergone pituitary surgery to date, and paraffin-embedded pituitary blocks were available for analysis. Loss of heterozygosity on chromosome 11q13 was studied by comparing microsatellite polymorphisms of leukocyte and tumor DNA using PYGM (centromeric) and D11S527 (telomeric), markers closely linked to the MEN-1 tumor suppressor gene. All tumors exhibited a loss of heterozygosity at both markers. Sequencing of the MEN-1 gene revealed no germline mutations in either family, nor was a somatic mutation found in tumor DNA from one subject in family A. The integrity of the MEN-1 gene in this subject was further supported by demonstration of the presence of MEN-1 messenger ribonucleic acid, as assessed by RT-PCR. These data indicate that loss of heterozygosity in these affected family
Cristian Ferrareze Nunes
Full Text Available ABSTRACT Objective To evaluate the intercarotid distance (ICD of patients with pituitary macroadenoma and compare to heatlhy controls. Method We retrospectively reviewed contrast-enhanced MRI images from twenty consecutive patients diagnosed with non-functioning pituitary macroadenoma, measured the ICD at two different levels (petrous segment – ICD1 and horizontal cavernous segment – ICD2 and compared to twenty paired controls. Results There was no statistically significant difference of the mean ICD1 between the groups and subgroups. For the ICD2 there was statistically significant difference between the case and controls. However, there was no significant difference between the patients with smaller adenomas and the controls. In contrast, the patients with giant adenomas showed statistically significantly higher ICD2 than the controls. Conclusion The ICD at the horizontal segment of the cavernous carotid tends to be wider in patients with giant pituitary adenomas than in healthy individuals or patients with smaller adenomas.
Chanson, P; Salenave, S
Pituitary tumors cause symptoms by secreting hormones (prolactin, PRL, responsible for amenorrhea-galactorrhea in women and decreased libido in men; growth hormone, GH, responsible for acromegaly; adrenocorticotropic hormone, ACTH, responsible for Cushing's syndrome; thyroid-stimulating hormone, TSH, responsible for hyperthyroidism), depressing the secretion of hormones (hypopituitarism), or by mass-related effects (headaches, visual field abnormalities...). All patients with pituitary tumors should be evaluated for gonadal, thyroid and adrenal function as well as PRL and GH secretion. Specific stimulation and suppression tests for pituitary hormones are performed in selected situations for detecting the type of hypersecretion or the response to treatment. Imaging procedures (mainly magnetic resonance imaging, MRI, nowadays) determine the presence, size and extent of the lesion. The classification of pituitary tumors is based on the staining properties of the cell cytoplasm viewed by light microscopy and immunocytochemistry revealing the secretory pattern of the adenoma. Treatment of pituitary adenomas consists of surgery (performed in more than 99% of cases via a transphenoidal route) and radiotherapy, generally fractionated or, in selected cases, using stereotactic techniques such as gamma-knife. The availability of medical treatment (dopamine, DA, agonists, somatostatin analogs, GH-receptor antagonists...) has profoundly modified the indications of radiotherapy, drugs being now generally used as a second-line treatment, after surgery (or even as first-line treatment). Based on the results of the different treatment modalities for each type of pituitary adenoma, recommendations will be proposed. They may be summarized as follows. For treatment of GH-secreting adenomas, trans-sphenoidal surgery is the first-line therapy except when the macroadenoma is giant or if surgery is contra-indicated; postoperative radiation therapy (fractionated, or by gamma-knife) is
Rabelink, N.M.; Lips, P.T.A.M.; Castelijns, J.
In this case report we describe a 38 year-old-female with galactorrhea several months after the birth of an anencephalic child. She had hyperpolactemia and imaging of the pituitary gland revealed a midline defect and a nasopharyngeal mass compatible with a meningo-(hypophyso-) encephalocele and
Pedro A.S. Rocha Filho
Full Text Available For twelve years, the subject of this report, a 38-year-old man, presented a clinical condition compatible with the SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome. He presented a stabbing and intense daily pain located in the left pre-auricular and temporal regions. Each of these intense pain attacks lasted around one minute and presented a frequency of two to eight times per day. The pain was associated with ipsilateral lacrimation, conjunctival injection and rhinorrhea. MRI revealed a pituitary tumor with little suprasellar extent. The subject’s serial assays of prolactin, GH, TSH and ACTH were within normal levels. Following transsphenoidal hypophysectomy, with complete removal of the tumor, the subject no more presented pain. The pathological diagnosis was non-secreting adenoma. Fourteen months after the surgery, he remains symptom-free.O paciente relatado neste artigo apresentou uma condição clínica compatível com síndrome SUNCT (cefaléia de curta duração, unilateral, neuralgiforme com hiperemia conjuntival e lacrimejamento. Ele referia dor diária, intensa, em facada, localizada na região pré-auricular e temporal esquerdas. Cada ataque de dor permanecia por cerca de um minuto, com freqüência de duas a oito vezes por dia. A dor se acompanhava de lacrimejamento ipsolateral, congestão conjuntival e rinorréia. A RM mostrou um tumor de hipófise com pouca extensão suprasselar. Dosagens de prolactina, GH, TSH e ACTH estavam em níveis normais. Foi então submetido a hipofisectomia transesfenoidal com remoção completa do tumor após o que a dor cessou completamente. O diagnóstico anátomo-patológico foi adenoma não secretor. Quatorze meses após a cirurgia, o paciente permanecia livre de dor.
Soni, Brijesh Kumar; Joish, Upendra Kumar; Sahni, Hirdesh; George, Raju A; Sivasankar, Rajeev; Aggarwal, Rohit
The growing belief that endocrine abnormalities may underlie many mental conditions has led to increased use of imaging and hormonal assays in patients attending to psychiatric OPDs. People who are in an acute phase of a psychiatric disorder show Hypothalamic Pituitary Adrenal (HPA) axis hyperactivity, but the precise underlying central mechanisms are unclear. To assess the pituitary gland volume variations in patients presenting with new onset acute psychiatric illness in comparison with age and gender matched controls by using MRI. The study included 50 patients, with symptoms of acute psychiatric illness presenting within one month of onset of illness and 50 age and gender matched healthy controls. Both patients and controls were made to undergo MRI of the Brain. A 0.9 mm slices of entire brain were obtained by 3 dimensional T1 weighted sequence. Pituitary gland was traced in all sagittal slices. Anterior pituitary and posterior pituitary bright spot were measured separately in each slice. Volume of the pituitary (in cubic centimetre- cm 3 ) was calculated by summing areas. Significance of variations in pituitary gland volumes was compared between the cases and controls using Analysis of Covariance (ANOVA). There were significantly larger pituitary gland volumes in the cases than the controls, irrespective of psychiatric diagnosis (ANOVA, f=15.56; p=0.0002). Pituitary volumes in cases were 15.36% (0.73 cm 3 ) higher than in controls. There is a strong likelihood of HPA axis overactivity during initial phase of all mental disorders along with increased pituitary gland volumes. Further studies including hormonal assays and correlation with imaging are likely to provide further insight into neuroanatomical and pathological basis of psychiatric disorders.
Lopes, M Beatriz S
The 4th edition of the World Health Organization (WHO) classification of endocrine tumors has been recently released. In this new edition, major changes are recommended in several areas of the classification of tumors of the anterior pituitary gland (adenophypophysis). The scope of the present manuscript is to summarize these recommended changes, emphasizing a few significant topics. These changes include the following: (1) a novel approach for classifying pituitary neuroendocrine tumors according to pituitary adenohypophyseal cell lineages; (2) changes to the histological grading of pituitary neuroendocrine tumors with the elimination of the term "atypical adenoma;" and (3) introduction of new entities like the pituitary blastoma and re-definition of old entities like the null-cell adenoma. This new classification is very practical and mostly based on immunohistochemistry for pituitary hormones, pituitary-specific transcription factors, and other immunohistochemical markers commonly used in pathology practice, not requiring routine ultrastructural analysis of the tumors. Evaluation of tumor proliferation potential, by mitotic count and Ki-67 labeling index, and tumor invasion is strongly recommended on individual case basis to identify clinically aggressive adenomas. In addition, the classification offers the treating clinical team information on tumor prognosis by identifying specific variants of adenomas associated with an elevated risk for recurrence. Changes in the classification of non-neuroendocrine tumors are also proposed, in particular those tumors arising in the posterior pituitary including pituicytoma, granular cell tumor of the posterior pituitary, and spindle cell oncocytoma. These changes endorse those previously published in the 2016 WHO classification of CNS tumors. Other tumors arising in the sellar region are also reviewed in detail including craniopharyngiomas, mesenchymal and stromal tumors, germ cell tumors, and hematopoietic tumors. It is
Veyssiere, Georges; Berger, Michel; Jean-Faucher, Christiane; Turckheim, Marc de; Jean, Claude
LH was measured by radioimmunology from the 18th day of gestation to birth in 318 male and female pituitary glands. LH was first detectable in the pituitary of 19 day old fetuses of both sexes. In both sexes pituitary LH levels increased from the 18th to the 31st day of gestation with a marked acceleration from the 24th day. LH pituitary levels were not sex-dependent [fr
Hypothalamic-pituitary vascularization in pituitary stalk transection syndrome: is the pituitary stalk really transected? The role of gadolinium-DTPA with spin-echo T1 imaging and turbo-FLASH technique
Genovese, E. [Dept. of Radiology, IRCCS Policlinico S. Matteo, Pavia (Italy); Maghnie, M. [Dept. of Pediatrics, Univ. of Pavia (Italy); Beluffi, G. [Dept. of Radiodiagnosis, Section of Pediatric Radiology, IRCCS Policlinico S. Matteo, Pavia (Italy); Villa, A. [Dept. of Radiology, IRCCS Policlinico S. Matteo, Pavia (Italy); Sammarchi, L. [Dept. of Radiology, IRCCS Policlinico S. Matteo, Pavia (Italy); Severi, F. [Dept. of Pediatrics, Univ. of Pavia (Italy); Campani, R. [Dept. of Radiology, IRCCS Policlinico S. Matteo, Pavia (Italy)
We examined 14 patients, aged 10-25 years, with idiopathic hypopituitarism. All presented an ectopic posterior pituitary at the median eminence with a hypoplastic anterior pituitary on magnetic resonance imaging (MRI). Eight patients had isolated growth hormone deficit (IGHD) and six had multiple hormone deficits (MPHD). Unenhanced MRI showed the pituitary stalk, which was extremely thin, in only three patients, while T1-weighted images obtained after intravenous injection of gadopentetate dimeglumine (Gd-DTPA) showed a thin pituitary stalk in seven patients (six with IGHD and one with MPHD), demonstrating a preserved vascular component of the stalk. MRI with Gd-DTPA was more sensitive than unenhanced MRI in detecting the pituitary stalk in patients with hypopituitarism with an ectopic posterior pituitary: the stalk was demonstrated in 50 % of the cases (seven patients), versus 21.4 % (three patients) by unenhanced MRI. The dynamic study of the hypothalamo-hypophyseal axis performed with turbo-FLASH sequences after bolus injection of Gd-DTPA showed the residual anterior pituitary to have arterial enhancement times, which suggests that an arterial system compensates for the absent or diminished blood supply from the portal system, independent of stalk detection. (orig.). With 5 figs., 1 tab.
Hypothalamic-pituitary vascularization in pituitary stalk transection syndrome: is the pituitary stalk really transected? The role of gadolinium-DTPA with spin-echo T1 imaging and turbo-FLASH technique
Genovese, E.; Maghnie, M.; Beluffi, G.; Villa, A.; Sammarchi, L.; Severi, F.; Campani, R.
We examined 14 patients, aged 10-25 years, with idiopathic hypopituitarism. All presented an ectopic posterior pituitary at the median eminence with a hypoplastic anterior pituitary on magnetic resonance imaging (MRI). Eight patients had isolated growth hormone deficit (IGHD) and six had multiple hormone deficits (MPHD). Unenhanced MRI showed the pituitary stalk, which was extremely thin, in only three patients, while T1-weighted images obtained after intravenous injection of gadopentetate dimeglumine (Gd-DTPA) showed a thin pituitary stalk in seven patients (six with IGHD and one with MPHD), demonstrating a preserved vascular component of the stalk. MRI with Gd-DTPA was more sensitive than unenhanced MRI in detecting the pituitary stalk in patients with hypopituitarism with an ectopic posterior pituitary: the stalk was demonstrated in 50 % of the cases (seven patients), versus 21.4 % (three patients) by unenhanced MRI. The dynamic study of the hypothalamo-hypophyseal axis performed with turbo-FLASH sequences after bolus injection of Gd-DTPA showed the residual anterior pituitary to have arterial enhancement times, which suggests that an arterial system compensates for the absent or diminished blood supply from the portal system, independent of stalk detection. (orig.). With 5 figs., 1 tab
The fourth edition of the World Health Organization classification of endocrine tumors (EN-WHO2017) was released in 2017. In this new edition, changes in the classification of non-neuroendocrine tumors are proposed particularly in tumors arising in the posterior pituitary. These tumors are a distinct group of low-grade neoplasms of the sellar region that express thyroid transcription factor-1, and include pituicytoma, granular cell tumor of the sellar region, spindle cell oncocytoma, and sellar ependymoma. This short review focuses on the classification of posterior pituitary tumors newly proposed in EN-WHO2017, and controversies in their pathological differential diagnosis are discussed based on recent cases.
Chitra Munusamy Rajendran
Full Text Available BACKGROUND Pituitary adenoma is a benign tumour that originates from the adenohypophyseal cells of the anterior lobe of pituitary gland. It accounts for 12% to 15% of all intracranial tumours. A spectrum of ocular manifestations are seen with these tumours ranging from the absence of any visual symptoms to severe visual field defects and loss of vision. The aim of the study is to study the various ocular features and its effect on vision, visual fields and ocular motility in cases of pituitary adenoma diagnosed on CT or MRI. MATERIALS AND METHODS This is a prospective study for a period of 1 year and 8 months conducted in Regional Institute of Ophthalmology, Madras Medical College. 25 patients aged between 25 to 65 years diagnosed as pituitary adenomas on radiological imaging who presented to squint clinic were evaluated after detailed history with visual acuity, pupillary reaction, colour vision, extraocular movements, slit lamp and fundus examination. Visual field examination was done with Octopus field analyser. RESULTS In our study, 25 patients of pituitary adenoma diagnosed on radio imaging were enrolled and evaluated. Most patients were above 50 years, 15 patients of the 25 were above 50 yrs. (60%. Females were predominantly affected (76%. Visual acquity of the patient was between 6/12-6/6 (62% on presentation. Headache was the commonest mode of presentation (80%. 76% showed field defects of which bitemporal hemianopia was the commonest in 52.9%. Pituitary macroadenoma 96% was the commonest type. Optic atrophy was seen only in 4 cases. Others had normal fundus. CONCLUSION Neuro-ophthalmic evaluation plays a major role not only in early detection, planning of treatment and further follow up, but also prevents visual loss if intervened early.
Ibis Beltrán Pérez
Full Text Available Se presenta un paciente masculino de 72 años de edad de la raza blanca, que es remitido al Servicio de Coloproctología por presentar desde hace más de un año, aumento de volumen alrededor del ano que le dificulta la defecación y sentarse. Se han realizado múltiples tratamientos tópicos con resultados no satisfactorios por el servicio de dermatología. Se le realiza estudio histopatológico de la lesión y se obtuvo un condiloma acuminado gigante (tumor de Buschke-Lowenstein. Se ha dado seguimiento sin presentarse hasta el momento recurrencia. El tumor de Buschke-Lowenstein es una entidad rara, considerada una lesión premaligna provocada por el papiloma virus humano. Hasta el momento no se ha podido encontrar el tratamiento ideal y continúa presentando una alta tasa de recurrencia.
Lazarus, S. M.; Splitt, M. E.
While not exclusively oceanic, gigantic jets (GJ) appear to have a preference for the tropical environment. In particular, a number of GJs have been observed in conjunction with tropical disturbances (i.e., weak tropical storms, depressions, and remnant lows). Given the remote aspect of TC convection and general lack of radar coverage, we explore this subset of events via analysis of their infrared and water vapor satellite presentations. The satellite perspective is relevant given that storm top mixing (dilution) of charge associated with storm-scale turbulence in this portion of the storm is thought to be connected to GJs. The thunderstorm overshoot, upper level divergence / outflow are examined in an effort to better understand the tropical paradigm. Specifically, an analysis of cloud top temperature, anvil expansion rates and asymmetries as well as placement of the GJ events with respect to the large (storm) scale circulation will be conducted.
Milikh, G M; Shneider, M N
Analysis of UV flashes observed by the UV detector on board the 'Tatiana' microsatellite suggests, based on their location, pulse width and energy of the source of the photons, that the flashes were generated by gigantic blue jets (GBJs). Presented in this paper is a numerical model of UV flashes due to a bunch of long streamers which form a leader, a prong such as that observed in a GBJ. Using a previously developed model of upward propagation of a long streamer in the exponential atmosphere the paper describes temporal evolution of the UV flux generated by a bunch of long streamers, in the given spectral range 300-400 nm used by the UV detector on board 'Tatiana'. The model is in agreement with the observations.
Okuyama, D.; Hatano, T.; Nakano, M.; Takeshita, S.; Ohsumi, H.; Tardif, S.; Shibuya, K.; Yumoto, H.; Koyama, T.; Ohashi, H.; Takata, M.; Kawasaki, M.; Tokura, Y.; Iwasa, Y.; Arima, T.
We examined the impact of electric field on crystal lattice of vanadium dioxide (VO 2 ) in a field-effect transistor geometry by in-situ synchrotron x-ray diffraction measurements. Whereas the c-axis lattice parameter of VO 2 decreases through the thermally induced insulator-to-metal phase transition, the gate-induced metallization was found to result in a significant increase of the c-axis length by almost 1% from that of the thermally stabilized insulating state. We also found that this gate-induced gigantic lattice deformation occurs even at the thermally stabilized metallic state, enabling dynamic control of c-axis lattice parameter by more than 1% at room temperature
Majos, C.; Coll, S.; Aguilera, C.; Pons, L.C. [Bellvitge Univ., Barcelona (Spain). Inst. de Diagnostice per la Imatge; Acebes, J.J. [Department of Neurosurgery, Ciutat Sanitaria i Universitaria de Bellvitge, L`Hospitalet de Llobregat, Barcelona (Spain)
We present five proven giant pituitary adenomas studied by CT and MRI, and review the clinical and imaging findings. Our aim was to examine the radiologic appearances and to search for criteria useful in distinguishing these tumors from other sellar and suprasellar tumours, mainly craniopharyngioma. The main differences from small adenomas were high prevalence of macrocysts, a more invasive behaviour and a clinical picture dominated by mass effect rather than endocrine disturbance. Factors supporting the diagnosis of pituitary adenoma in a giant intra- and suprasellar mass include: infrasellar extension, absence of calcification and presence of low-signal cysts on T1-weighted images. (orig.) (orig.) With 4 figs., 2 tabs., 9 refs.
Kanabrocki, E.L.; Greco, J.; Graham, L.A.; Kaplan, E.; Rubnitz, M.E.; Oester, Y.T.; Brar, S.; Gustafson, P.S.; Nelson, D.M.; Molinari, F.; Moore, C.E.; Kanabrocki, J.A.
Neutron activation analysis was used to determine several trace elements in human pituitaries from adult subjects aged 42 to 83 years who had died of ischemic heart disease, carcinoma and cirrhosis of the liver. None of these subjects suffered from mental disease. The concentrations of Fe, Zn, Rb, Se, Br, Cr, Hg, Cs and Co in dried tissue showed no correlation with the diseases, and the results may therefore be representative of the general population. These elements may play a very fundamental role in pituitary function. (U.K.)
Full Text Available Subarachnoid hemorrhage (SAH is the bleeding into the subarachnoid space containing cerebrospinal fluid. The most common cause of SAH is trauma. Rupture of aneurysms, vascular anomalies, tumor bleeds and hypertension are other important etiologies. SAH in the setting of pituitary tumor can result from various causes. It can be due to intrinsic tumor related pathology, injury to surrounding the vessel during the operative procedure or due to an associated aneurysm. We discuss the pathological mechanisms and review relevant literature related to this interesting phenomenon. Early and accurate diagnosis of the cause of the SAH in pituitary tumors is important, as this influences the management.
Fordyce, R Ewan; Marx, Felix G
Baleen whales (Mysticeti) are the largest animals on Earth, thanks to their ability to filter huge volumes of small prey from seawater. Mysticetes appeared during the Late Eocene, but evidence of their early evolution remains both sparse and controversial [1, 2], with several models competing to explain the origin of baleen-based bulk feeding [3-6]. Here, we describe a virtually complete skull of Llanocetus denticrenatus, the second-oldest (ca. 34 Ma) mysticete known. The new material represents the same individual as the type and only specimen, a fragmentary mandible. Phylogenetic analysis groups Llanocetus with the oldest mysticete, Mystacodon selenensis , into the basal family Llanocetidae. Llanocetus is gigantic (body length ∼8 m) compared to other early mysticetes [7-9]. The broad rostrum has sharp, widely spaced teeth with marked dental abrasion and attrition, suggesting biting and occlusal shearing. As in extant mysticetes, the palate bears many sulci, commonly interpreted as osteological correlates of baleen . Unexpectedly, these sulci converge on the upper alveoli, suggesting a peri-dental blood supply to well-developed gums, rather than to inter-alveolar racks of baleen. We interpret Llanocetus as a raptorial or suction feeder, revealing that whales evolved gigantism well before the emergence of filter feeding. Rather than driving the origin of mysticetes, baleen and filtering most likely only arose after an initial phase of suction-assisted raptorial feeding [2, 4, 5]. This scenario differs strikingly from that proposed for odontocetes, whose defining adaptation-echolocation-was present even in their earliest representatives . Copyright © 2018 Elsevier Ltd. All rights reserved.
In the ionosphere, more than 80 kilometers above Earth's surface, incoming radiation reacts with the thin air to produce highly charged ions, inducing an electric potential between the ionosphere and the surface. This charge difference is dissipated by a slow leak from the ionosphere during calm weather and reinvigorated by a charge built up near the surface during a thunderstorm. In 2001, however, researchers discovered gigantic jets (GJs), powerful lightning that arcs from tropospheric clouds up to the ionosphere, suggesting there may be an alternate path by which charge is redistributed. GJs are transient species, and little is known about how much charge they can carry, how they form, or how common they are. In a step toward answering these questions, Lu et al. report on two GJs that occurred near very high frequency (VHF) lightning detection systems, which track the development of lightning in three spatial dimensions, giving an indication of the generation mechanism. The researchers also measured the charge transfer in the two GJs through remote sensing of magnetic fields. They found that both jets originated from the development of otherwise normal intracloud lightning. The dissipation of the cloud's positively charged upper layer allowed the negative lightning channel to break through and travel up out of the top of the cloud to the ionosphere. The first jet, which occurred off the coast of Florida, leapt up to 80 kilometers, depositing 110 coulombs of negative charge in 370 milliseconds. The second jet, observed in Oklahoma, traveled up to 90 kilometers, raising only 10-20 coulombs in 300 milliseconds. Each new observation of gigantic jets such as these can provide valuable information toward understanding this novel atmospheric behavior. (Geophysical Research Letters, doi:10.1029/2011GL047662, 2011)
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
Feb 8, 2016 ... pituitary metastases can be characteristic and evocative but in no case pathognomic. The diabetes insipidus is the most common clinical manifestation of the disease [2, 3]. We report herein a case of an acute adrenal insufficiency revealing pituitary metastases of lung cancer. Patient and observation.
Thompson, Michael; Mathuram Thiyagarajan, Umasankar; Akoh, Jacob A
Prosthetic arteriovenous grafts (AVGs) are associated with greater morbidity than autogenous arteriovenous fistulas (AVFs), but their use is indicated when AVF formation is not possible. This report adds to the literature a case of lower limb gigantism, painful varicosities, and lymphedema following long-term use of AVG in the upper thigh. The patient's past medical history included renal transplantation on the same side well before the AVG was inserted and right leg deep vein thrombosis. Suspicion of AVG thrombosis was excluded by Doppler ultrasound, which demonstrated an access flow of 1700 mL/min. A computed tomography (CT) scan of the abdomen and pelvis did not identify the cause of her symptoms. Whereas functional incompetence of the iliac vein valve might be responsible for the varicosities, the extent of hypertrophy in this case raises the suspicion of lymphatic blockage possibly secondary to groin dissection undertaken at the time of graft insertion, in addition to the previous dissection at the time of transplantation. This case highlights the need for minimal groin dissection during AVG insertion, particularly in patients with a history of previous abdominopelvic surgery. © 2014 International Society for Hemodialysis.
Full Text Available FSH-secreting pituitary adenomas can affect sexual and reproductive function. In this article, we have reported the case of a 32-year-old male with secondary infertility. The patient had sexual and reproductive disturbances. The test results of the blood samples indicated obviously decreased testosterone (T and estradiol (E2 levels. Based on previous hormonal results, the patient received pituitary stimulation and human chorionic gonadotropin (hCG tests. Both follicle stimulating hormone (FSH and luteinizing hormone (LH showed low response during the pituitary stimulation test. The results of the hCG test indicated that T/E2 could recover to a normal level. In addition, this patient was diagnosed with pituitary macroadenoma, which was supported by the pituitary MRI. The man’s sexual and reproductive functions recovered following surgery. The pathological results confirmed that the tumor tissue was an FSH-secreting pituitary adenoma by immunohistochemical staining. The purpose of this report was to review the relative literature and discuss the influence of FSH-secreting pituitary adenomas on hormones through the hypothalamus-pituitary-testis axis.
Casar-Borota, Olivera; Botling, Johan; Granberg, Dan; Stigare, Jerker; Wikström, Johan; Boldt, Henning Bünsow; Kristensen, Bjarne Winther; Pontén, Fredrik; Trouillas, Jacqueline
Differential diagnosis based on morphology and immunohistochemistry between a clinically nonfunctioning pituitary neuroendocrine tumor (NET)/pituitary adenoma and a primary or secondary NET of nonpituitary origin in the sellar region may be difficult. Serotonin, a frequently expressed marker in the NETs, has not been systematically evaluated in pituitary NETs. Although mutations in ATRX or DAXX have been reported in a significant proportion of pancreatic NETs, the mutational status of ATRX and DAXX and their possible pathogenetic role in pituitary NETs are unknown. Facing a difficult diagnostic case of an invasive serotonin and adrenocorticotroph hormone immunoreactive NET in the sellar region, we explored the immunohistochemical expression of serotonin, ATRX, and DAXX in a large series of pituitary endocrine tumors of different types from 246 patients and in 2 corticotroph carcinomas. None of the pituitary tumors expressed serotonin, suggesting that serotonin immunoreactive sellar tumors represent primary or secondary NETs of nonpituitary origin. Normal expression of ATRX and DAXX in pituitary tumors suggests that ATRX and DAXX do not play a role in the pathogenesis of pituitary endocrine tumors that remain localized to the sellar and perisellar region. A lack of ATRX or DAXX in a sellar NET suggests a nonpituitary NET, probably of pancreatic origin. One of the 2 examined corticotroph carcinomas, however, demonstrated negative ATRX immunolabeling due to an ATRX gene mutation. Further studies on a larger cohort of pituitary carcinomas are needed to clarify whether ATRX mutations may contribute to the metastatic potential in a subset of pituitary NETs.
Pendleton, Courtney; Wand, Gary; Quinones-Hinojosa, Alfredo
Harvey Cushing's monograph The Pituitary Body and Its Disorders describes Case XXXII, a 36-year-old man who presented with gigantism in 1910. The detailed post-mortem exam findings are prefaced with a cryptic statement, describing "inauspicious circumstances" surrounding the autopsy. Although contemporary biographies of Cushing have offered insight into these circumstances, the original surgical file for Case XXXII has not been previously reviewed. The original Johns Hopkins Hospital surgical records were reviewed, and the case of John Turner, who Cushing identified by name in his monograph The Pituitary Body and Its Disorders, was selected for further review. A review of the original surgical file revealed a typewritten note by Dr. Crowe, one of the surgeons who performed the post-mortem exam, with a handwritten addendum by Dr. Cushing. This document provides detail regarding the "inauspicious circumstances" surrounding the autopsy. Namely, the autopsy was conducted without permission of the family, during the funeral service, following a payment to the undertaker. The new information regarding the autopsy of John Turner offers insight into the previously incompletely described circumstances surrounding the autopsy. Additionally, the case illuminates the obligations and ethical quandaries that physician-scientists face.
Pereira, Bernardo Dias; Raimundo, Luísa; Mete, Ozgur; Oliveira, Ana; Portugal, Jorge; Asa, Sylvia L
Thyrotropin (TSH)-secreting pituitary adenomas are exceedingly rare at the pediatric age and no cases of co-secretion with other pituitary hormones in these tumors have been described in this age range. We present a case of a monomorphous plurihormonal pituitary adenoma that co-secreted TSH and GH in a pediatric patient. A 13-year-old male presented with increasing height velocity (17.75 cm/year, 9.55SD), weight loss, and visual impairment. Initial biochemical evaluations revealed secondary hyperthyroidism. A giant pituitary tumor compressing the surrounding structures was detected by magnetic resonance, and a transsphenoidal surgery was initially performed. Pathological examinations revealed an atypical, monomorphous plurihormonal Pit-1 lineage tumor with mixed features of silent subtype 3 adenoma and acidophil stem cell adenoma. In the postoperative period, secondary hyperthyroidism recurred with high levels of both GH and IGF1. In addition, due to tumor re-growth, a multimodality treatment plan was undertaken including surgery, somatostatin analogs, and radiotherapy. We report the first pediatric case of a plurihormonal TSH- and GH-secreting pituitary adenoma, further expanding the clinical manifestations of pediatric pituitary tumors. Comprehensive pathological evaluation and close follow-up surveillance are crucial to the prompt delivery of the best therapeutic options in the context of this particularly aggressive pituitary tumor.
Iacovazzo, Donato; Korbonits, Márta
X-linked acrogigantism (XLAG) is a recently identified condition of early-onset GH excess resulting from the germline or somatic duplication of the GPR101 gene on chromosome Xq26.3. Thirty patients have been formally reported so far. The disease affects mostly females, occurs usually sporadically, and is characterised by early onset and marked overgrowth. Most patients present with concomitant hyperprolactinaemia. Histopathology shows pituitary hyperplasia or pituitary adenoma with or without associated hyperplasia. XLAG-related pituitary adenomas present peculiar histopathological features that should contribute to raise the suspicion of this rare condition. Treatment is frequently challenging and multi-modal. While females present with germline mutations, the sporadic male patients reported so far were somatic mosaics with variable levels of mosaicism, although no differences in the clinical phenotype were observed between patients with germline or somatic duplication. The GPR101 gene encodes an orphan G protein-coupled receptor normally expressed in the central nervous system, and at particularly high levels in the hypothalamus. While the physiological function and the endogenous ligand of GPR101 are unknown, the high expression of GPR101 in the arcuate nucleus and the occurrence of increased circulating GHRH levels in some patients with XLAG, suggest that increased hypothalamic GHRH secretion could play a role in the pathogenesis of this condition. In this review, we summarise the published evidence on XLAG and GPR101 and discuss the results of recent studies that have investigated the potential role of GPR101 variants in the pathogenesis of pituitary adenomas. Copyright © 2016 Elsevier Ltd. All rights reserved.
Almeida Magalhaes, Alvaro C. de; Uehara, Karla C.; Iezzi, Denise
We compare 1,5 T magnetic resonance (MR) image findings in 193 patients with congenital pituitary congenital insufficiency. One hundred and thirty nine of the MR studies were obtained in patients who had isolated growth hormone deficiency. Other fifth - four patients had multiple pituitary hormone deficiency. On MR images, normal anterior and posterior lobes of the pituitary glands can be clearly differentiated because the posterior lobe has a characteristic high intensity on TI-weighted images. In fifty-four patients, the high- intensity of the posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in fifty-one patients. this high- intensity area is the newly formed ectopic posterior lobe, which also secrets anti-diuretic hormone just as the posterior lobe would. MR imaging can demonstrate the transection of the pituitary stalk and the formation of the ectopic lobe, revealing to be a useful diagnostic tool in the definition of the type of alteration in growth defects of endocrine origin. (author)
Giavoli, Claudia; Tagliabue, Claudia; Profka, Eriselda; Senatore, Laura; Bergamaschi, Silvia; Rodari, Giulia; Spada, Anna; Beck-Peccoz, Paolo; Esposito, Susanna
A number of studies of adults have shown that pituitary deficiencies can develop in a considerable proportion of subjects during the acute phase of meningitis or years after the infection has disappeared. The results of the very few studies of the impact of pediatric meningitis on hypothalamic-pituitary function are conflicting. In order to determine the incidence of pituitary dysfunction in children with central nervous system infection, we evaluated pituitary function and anthropometric parameters in 19 children with meningitis of different etiologies (15 males; mean age ± standard deviation [SD] at pituitary evaluation, 5.9 ± 4.0 years; mean time from the acute event ± SD, 18 ± 10 months). All of the subjects had a normal stature and growth velocity for their age and gender, and none of them was obese. On the basis of Tanner's reference charts, 17 subjects (13 boys and all four girls) were pre-pubertal; two boys were in Tanner stage 2. None of the subjects had central hypothyroidism. All of the patients had normal serum of insulin growth factor (IGF)-I and prolactin. Their sex steroid and gonadotropin levels were concordant with their age and pubertal status. Early morning urine osmolality and serum electrolyte levels showed no signs of diabetes insipidus. All of the patients had normal plasma adrenocorticotropic hormone (ACTH) levels. Peak cortisol responses to the standard dose Synacthen test (SDST) were normal in all cases. The results showed that hypopituitarism following infectious meningitis appears to be infrequent in childhood and children's pituitary glands seem to be less vulnerable to damage than those of adults.
Full Text Available Mary-Anne Doyle, Heather A Lochnan Division of Endocrinology, University of Ottawa, Ottawa, ON, Canada Objective: “The pseudomalabsorption of thyroxine” has been used to describe patients with hypothyroidism who fail to comply with their treatment. We describe a unique case of a 32-year-old with hypothyroidism who developed pituitary hyperplasia and hyperprolactinemia secondary to the pseudomalabsorption of thyroxine. Investigations and treatment: After baseline thyroid-function tests were performed, the patient was administered levothyroxine 0.5 mg under the supervision of a registered nurse. Thyroid function testing was repeated at 30, 60, 120, and 180 minutes. Arrangements were made for further daily supervised loading of levothyroxine 0.1 mg. Results: With the administration of 0.5 mg levothyroxine, free thyroxine levels increased by 120 minutes, and with daily supervised dosing of 0.1 mg there was normalization of the thyroid hormone levels and a reduction of thyroid-stimulating hormone levels. Maintenance of thyroid-stimulating hormone < 15 mU/L for 2 weeks led to a reduction in prolactin levels and regression in the size of the pituitary on magnetic resonance imaging. Conclusion: If left untreated, these patients face significant morbidity and are at risk of developing pituitary hyperplasia, complications from an increase in pituitary size, hyperprolactinemia, and potentially myxedema coma. Recognizing pituitary hyperplasia and hyperprolactinemia as a complication from the pseudomalabsorption of levothyroxine may prevent the potential of a misdiagnosis of a prolactinoma leading to unnecessary investigations and inappropriate treatment. Patient awareness of this serious complication and the rapid, demonstrable resolution with adequate thyroid hormone replacement may provide motivation to comply with supervised dosing of levothyroxine. It has also been suggested that supervised treatment enables the individual to maintain their patient
Brandao, Rafael Augusto Castro Santiago; Dellaretti Filho, Marcos Antonio, E-mail: email@example.com [Hospital Santa Casa de Belo Horizonte, MG (Brazil); Nunes, Tadeu Wilker; Totola, Paolo Victor Fernandes; Fonseca, Vinicius Silveira; Souza, Warley Cristiano de [Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG (Brazil). Faculdade de Medicina
Neurocysticercosis (NCC) is the most common parasitic infection of the nervous system. Considered a global epidemic, the parasite presents, mainly, as asymptomatic. Usually treatment is clinical, but in some selected cases surgical intervention is a good treatment option. In our case, keeping in view the magnitude of intracranial injury, surgery was the therapy chosen. A female patient, aged 54, Mulatto, native of Novo Cruzeiro (MG) and raised in Belo Horizonte. Presented with intense frontal headache, which progressed to right hemiparesis and seizure. Ten days after onset of symptoms, sought the service of emergency care. Tomography (CT) revealed a hypodense area in left frontoparietal region with characteristics of cystic lesion.
Daems, Tania; Verhelst, Johan; Michotte, Alex; Abrams, Pascale; De Ridder, Dirk; Abs, Roger
Silent pituitary adenomas are a subtype of adenomas characterized by positive immunoreactivity for one or more hormones classically secreted by normal pituitary cells but without clinical expression, although in some occasions enhanced or changed secretory activity can develop over time. Silent corticotroph adenomas are the classical example of this phenomenon. A series of about 500 pituitary adenomas seen over a period of 20 years were screened for modification in hormonal secretion. Biochemical and immunohistochemical data were reviewed. Two cases were retrieved, one silent somatotroph adenoma and one thyrotroph adenoma, both without specific clinical features or biochemical abnormalities, which presented 20 years after initial surgery with evidence of acromegaly and hyperthyroidism, respectively. While the acromegaly was controlled by a combination of somatostatin analogs and growth hormone (GH) receptor antagonist therapy, neurosurgery was necessary to manage the thyrotroph adenoma. Immunohistochemical examination demonstrated an increase in the number of thyroid stimulating hormone (TSH)-immunoreactive cells compared to the first tissue. Apparently, the mechanisms responsible for the secretory modifications are different, being a change in secretory capacity in the silent somatotroph adenoma and a quantitative change in the silent thyrotroph adenoma. These two cases, one somatotroph and one thyrotroph adenoma, are an illustration that clinically silent pituitary adenomas may in rare circumstances evolve over time and become active, as previously demonstrated in silent corticotroph adenomas.
Hubertus Maximilian Mehdorn
Full Text Available A rare case of infundibulo-neurohypophysitis mimicking a pituitary adenoma is presented. A 69-years-old female patient developed polyuria and polydipsia. Laboratory analysis revealed central diabetes insipidus. No hormonal abnormalities. Cranial-magnetic resonance imaging (MRI showed a left sided mass in the adenohypophysis presuming a pituitary adenoma. The mass had contact to both internal carotids. Admission to our department for neurosurgical treatment followed. Ophthalmo - logic examination and neurological examination yielded normal findings. A second MRI focussing on the sellar-region showed a leftsided (T2-MRI.hyperintense, distended adenohypophysis, without contrast enhancement in T1. The stalk appeared thickened. T1- weighted sequences of the neurohypophysis showed loss of signal intensity. We diagnosed an infundibulo-neurohypophysitis and abstai - ned from surgical removal. The patient was discharged under treatment with corticosteroids and desmopressin. Hypophysitis is rare and shows special clinical characteristics. Despite defined radiological features to differentiate between hypophysitis and adenoma the possibility of misdiagnosis, and unnecessary surgical procedures, should always kept in mind.
Full Text Available Sheehan's syndrome is caused by pregnancy-related hemorrhage leading to ischemic necrosis of the anterior pituitary gland and hypopituitarism. Spontaneous pregnancy in Sheehan's syndrome is very rare. We report the case of a patient with Sheehan's syndrome who suffered from anterior pituitary insufficiency, but with sparing of gonadotropic function. The patient became pregnant spontaneously and, after her second delivery, thyrotropic function recovered. However, the patient's growth hormone and cortisol levels remained unresponsive to an insulin-tolerance test. This case demonstrates that pituitary function may recover from less extensive pituitary ischemia. We emphasize the importance of early identification of pregnancy in such cases. It is crucial to institute adequate hormone-replacement therapy during pregnancy, since hypopituitarism is associated with high fetal and maternal morbidity and mortality.
A possible new syndrome with growth-hormone secreting pituitary adenoma, colonic polyposis, lipomatosis, lentigines and renal carcinoma in association with familial testicular germ cell malignancy: A case report
Mai Phuong L
Full Text Available Abstract Background Germ-cell testicular cancer has not been definitively linked to any known hereditary cancer susceptibility disorder. Familial testicular cancer in the presence of other findings in affected and unaffected family members might indicate a previously-unidentified hereditary cancer syndrome. Case presentation The patient was diagnosed with a left testicular seminoma at age 28, and treated with left orchiectomy followed by adjuvant cobalt radiation. His family history is significant for testicular seminoma in his son, bladder cancer in his sister, and lipomatosis in his father. His evaluation as part of an etiologic study of familial testicular cancer revealed multiple colon polyps (adenomatous, hyperplastic, and hamartomatous first found in his 50 s, multiple lipomas, multiple hyperpigmented skin lesions, left kidney cancer diagnosed at age 64, and a growth-hormone producing pituitary adenoma with associated acromegaly diagnosed at age 64. The patient underwent genetic testing for Cowden syndrome (PTEN gene, Carney complex (PRKAR1A gene, and multiple endocrine neoplasia syndrome type 1 (MEN1 gene; no deleterious mutations were identified. Discussion The constellation of benign and malignant neoplasms in the context of this patient's familial testicular cancer raised the possibility that these might be manifestations of a known hereditary susceptibility cancer syndrome; however, genetic testing for the three syndromes that were most likely to explain these findings did not show any mutation. Alternatively, this family's phenotype might represent a novel neoplasm susceptibility disorder. This possibility cannot be evaluated definitively on the basis of a single case report; additional observations and studies are necessary to investigate this hypothesis further.
Binoy Kumar Mohanty
Full Text Available BACKGROUND Pituitary tumours are relatively common endocrine tumours. They can present with symptoms related to hormone excess or hormone deficiency. They can also present with compressive symptoms like visual problems and headache. OBJECTIVE To study the various clinical presentations and endocrine profile of patients presenting with pituitary tumours to a tertiary care hospital. DESIGN Cross sectional study. MATERIAL AND METHODS We collected and analysed the clinical data including hormonal status of 33 consecutive patients who presented to our department from March 2014 to February 2016 for evaluation of pituitary tumours. RESULTS Majority of the subjects studied belonged to 40-50 years group (33.34%.The most common type of pituitary tumour in our population was non-functioning pituitary tumours (45.45%. The next common cause was somatotroph adenoma (27.27% followed by prolactinoma (15.15% and corticotroph adenomas (12.13%. There was significant male predominance (60.60% among total cases. Among all patients, headache (54.54% was most common presentation followed by features related to hormone excess (51.51%. CONCLUSIONS Pituitary tumours can present with variety of symptoms. A detailed endocrine workup is essential in each case to reach at correct diagnosis. In our cohort, non-functioning pituitary tumour was the most common tumour subtype.
Liebert, W.; Szyfter, W.; Tokarz, F.; Szmeja, Z.; Paprzycki, W.
In the years 1976-1993 we have performed 90 transsphenoidal approaches for pituitary adenomas. This paper presents a detail report concerning 23 cases, operated in the last 2 years, with CT and NMR documentation. A preoperative visual deficit was very common in this group of patients. In the postoperative period the dramatic improvement of the vision was observed in 86% of cases. In our opinion the transsphenoidal approach is very useful for the operation of the tumors with suprasellar expansion. (author)
Hagen, C; Schroeder, H D; Hansen, S
with TMZ. One tumour was initially a macroprolactinoma that developed into a mixed GH- and prolactin-secreting carcinoma (patient A). To our knowledge, this is the first published in English literature. Two adenomas, a macroprolactinoma (patient B) and a clinically non-functioning pituitary adenoma...... sizes were significantly reduced, hormone levels normalized and symptoms of mass effects decreased in all three cases. The carcinoma was treated from 2004 to 2006 (23 months). Three years after the terminating treatment, the tumour has not regrown and hormone levels are normalized. Immunohistochemical...
T cell lymphoblastic lymphoma/leukemia within an adrenocorticotropic hormone and thyroid stimulating hormone positive pituitary adenoma: A cytohistological correlation emphasizing importance of intra-operative squash smear.
Gupta, Rakesh K; Saran, Ravindra K; Srivastava, Arvind K; Jagetia, Anita; Garg, Lalit; Sharma, Mehar C
We present a rare case of primary pituitary T cell lymphoma/leukemia (T-LBL) in association with adrenocorticotropic hormone (ACTH) and thyroid stimulating hormone (TSH) expressing pituitary adenoma in a 55-year-old woman highlighting the importance of intra-operative squash smears examination. The patient presented with complaints of headache, diminution of vision and recent onset altered sensorium. MRI revealed a mass lesion in the sellar-suprasellar region with non-visualization of pituitary gland separately, extending to involve adjacent structures diagnosed as invasive pituitary macroadenoma. Intra-operative tissue was sent for squash smear examination. The cytology showed a tumor comprising of sheets of immature lymphoid cells intermixed with clusters of pituitary acinar cells with many mitoses and tingible body macrophages. A diagnosis of presence of immature lymphoid cells within the pituitary was offered and differentials of infiltration by lymphoma cells from systemic disease versus primary central nervous lymphoma-like lymphoma arising in the pituitary adenoma were considered. Later paraffin section examination and immunohistochemistry corroborated with the squash findings and a final diagnosis of primary pituitary T cell lymphoma/leukemia in association with ACTH and TSH expressing pituitary adenoma was made. To date, only six cases of primary pituitary T cell lymphomas, including three T-LBL cases, have been reported. This is the seventh case and first one additionally describing cytohistological correlation and importance of intra-operative cytology. © 2017 Japanese Society of Neuropathology.
In this study, the effects from postoperative radiotherapy of parasellar or suprasellar adenomas of the pituitary were analysed to provide more detailed information about the value of radiation treatment, when used as a secondary measure after surgery or even in the primary treatment of pituitary adenomas. The analysis was based on 75 patients showing parasellar or suprasellar tumours of the pituitary. Radiation was in most cases preceded by surgery and carried out in the Radiological Department of Munich's University Hospital after the year 1975, when computed tomography had been made available to the Neurosurgical Department of this clinic. (orig./MG) [de
Bancalari, Rodrigo E; Gregory, Louise C; McCabe, Mark J; Dattani, Mehul T
The embryonic development of the pituitary gland involves a complex and highly spatio-temporally regulated network of integrating signalling molecules and transcription factors. Genetic mutations in any of these factors can lead to congenital hypopituitarism in association with a wide spectrum of craniofacial/midline defects ranging from incompatibility with life to holoprosencephaly (HPE) and cleft palate and septo-optic dysplasia (SOD). Increasing evidence supports a genotypic overlap with hypogonadotrophic hypogonadal disorders such as Kallmann syndrome, which is consistent with the known overlap in phenotypes between these disorders. This chapter reviews the cascade of events leading up to the successful development of the pituitary gland and to highlight key areas where genetic variations can occur thus leading to congenital hypopituitarism and associated defects. Copyright © 2012 S. Karger AG, Basel.
Mitrofanova, Lubov B.; Konovalov, Petr V.; Krylova, Julia S.; Polyakova, Victoria O.; Kvetnoy, Igor M.
Introduction plurihormonality of pituitary adenomas is an ability of adenoma cells to produce more than one hormone. After the immunohistochemical analysis had become a routine part of the morphological study, a great number of adenomas appeared to be multihormonal in actual practice. We hypothesize that the same cells of a normal pituitary gland releases several hormones simultaneously. Objective To analyse a possible co-expression of hormones by the cells of the normal anterior pituitary of adult humans in autopsy material. Materials and methods We studied 10 pituitary glands of 4 women and 6 men with cardiovascular and oncological diseases. Double staining immunohistochemistry using 11 hormone combinations was performed in all the cases. These combinations were: prolactin/thyroid-stimulating hormone (TSH), prolactin/luteinizing hormone (LH), prolactin/follicle-stimulating hormone (FSH), prolactin/adrenocorticotropic hormone (ACTH), growth hormone (GH)/TSH, GH/LH, GH/FSH, GH/ACTH, TSH/LH, TSH/FSH, TSH/ACTH. Laser Confocal Scanning Microscopy with a mixture of primary antibodies was performed in 2 cases. These mixtures were ACTH/prolactin, FSH/prolactin, TSH/prolactin, ACTH/GH, and FSH/GH. Results We found that the same cells of the normal adenohypophysis can co-express prolactin with ACTH, TSH, FSH, LH; GH with ACTH, TSH, FSH, LH, and TSH with ACTH, FSH, LH. The comparison of the average co-expression coefficients of prolactin, GH and TSH with other hormones showed that the TSH co-expression coefficient was significantly the least (9,5±6,9%; 9,6±7,8%; 1,0±1,3% correspondingly). Conclusion Plurihormonality of normal adenohypophysis is an actually existing phenomenon. Identification of different hormones in pituitary adenomas enables to find new ways to improve both diagnostic process and targeted treatment. PMID:28418929
Mitrofanova, Lubov B; Konovalov, Petr V; Krylova, Julia S; Polyakova, Victoria O; Kvetnoy, Igor M
plurihormonality of pituitary adenomas is an ability of adenoma cells to produce more than one hormone. After the immunohistochemical analysis had become a routine part of the morphological study, a great number of adenomas appeared to be multihormonal in actual practice. We hypothesize that the same cells of a normal pituitary gland releases several hormones simultaneously. To analyse a possible co-expression of hormones by the cells of the normal anterior pituitary of adult humans in autopsy material. We studied 10 pituitary glands of 4 women and 6 men with cardiovascular and oncological diseases. Double staining immunohistochemistry using 11 hormone combinations was performed in all the cases. These combinations were: prolactin/thyroid-stimulating hormone (TSH), prolactin/luteinizing hormone (LH), prolactin/follicle-stimulating hormone (FSH), prolactin/adrenocorticotropic hormone (ACTH), growth hormone (GH)/TSH, GH/LH, GH/FSH, GH/ACTH, TSH/LH, TSH/FSH, TSH/ACTH. Laser Confocal Scanning Microscopy with a mixture of primary antibodies was performed in 2 cases. These mixtures were ACTH/prolactin, FSH/prolactin, TSH/prolactin, ACTH/GH, and FSH/GH. We found that the same cells of the normal adenohypophysis can co-express prolactin with ACTH, TSH, FSH, LH; GH with ACTH, TSH, FSH, LH, and TSH with ACTH, FSH, LH. The comparison of the average co-expression coefficients of prolactin, GH and TSH with other hormones showed that the TSH co-expression coefficient was significantly the least (9,5±6,9%; 9,6±7,8%; 1,0±1,3% correspondingly). Plurihormonality of normal adenohypophysis is an actually existing phenomenon. Identification of different hormones in pituitary adenomas enables to find new ways to improve both diagnostic process and targeted treatment.
Full Text Available This article takes the “island” as a key trope in tourism studies, exploring how ideas of culture and nature, as well as those of paradise (lost are central to its interpretation for tourists and tourist industries alike. Increasingly, however, island tourism is blurring the line between geographies of land and water, continent and archipelago, and private and public property. The case of ‘The World’ islands mega project off the coast of Dubai (UAE is used to chart the changing face and future of island tourism, exploring how spectacle, branding and discourses of the gigantic, miniature, and fake, particularly alongside technological mediations on a large-scale, reflect the postmodern neoliberal world of tourism and the liquid times in which we live. Artificial island complexes such as this one function as cosmopolitan ‘non-places’ at the same time that they reflect a resurgence in (British nascent nationalism and colonial nostalgia, all the whilst operating in a sea of ‘junkspace’. The shifting cartography of ‘the island’ is thus mapped out to suggest new forms of place-making and tourism’s evolving relationship to these floating islandscapes.
Lazarus, S. M.; Splitt, M. E.; Brownlee, James; Spiva, Nicholas; Liu, Ningyu
This paper presents a meteorological analysis of a storm that produced two jets, four gigantic jets (GJ), and a starter, which were observed by two radars as well as the Kennedy Space Center 4-Dimensional Lightning Surveillance System on 3 August 2013 in Central Florida. The work is the first application of dual polarization data to a jet-producing storm and is the fifth case related to a tropical disturbance. The storm environment is consistent with the moist tropical paradigm that characterizes about three quarters of the surface and aircraft observed jet and GJ events. The most unstable (MU) convective available potential energy is not unusual for Florida summer convection and is below the climatological mean for these events. An unusual speed shear layer is located near the storm equilibrium level (EL) and the storm exhibits a tilted structure with CGs displaced upshear. The turbulence, as measured by the eddy dissipation rate, is extreme near the storm top during the event window, consistent with the GJ mixing hypothesis. The individual events are collocated with, and track along, the center axis of the divergent outflow at the EL and occur within the region of the coldest GOES IR temperatures—placing the events within the overshoot. The dual polarization data indicate a deep graupel column, extending above the mixed phase layer, to a 13 km altitude.
van der Velde, Oscar A.; Bór, József; Li, Jingbo; Cummer, Steven A.; Arnone, Enrico; Zanotti, Ferruccio; Füllekrug, Martin; Haldoupis, Christos; Naitamor, Samir; Farges, Thomas
At 2336:56 UTC on 12 December 2009, a bright gigantic jet (GJ) was recorded by an observer in Italy. Forty-nine additional sprites, elves, halos and two cases of upward lightning were observed that night. The location of the GJ corresponded to a distinct cloud top (-34°C) west of Ajaccio, Corsica. The GJ reached approximately 91 km altitude, with a "trailing jet" reaching 49-59 km, matching with earlier reported GJs. The duration was short at 120-160 ms. This is the first documented GJ which emerged from a maritime winter thunderstorm only 6.5 km tall, showing high cloud tops are not required for initiation of GJs. In the presence of strong vertical wind shear, the meteorological situation was different from typical outbreaks of fall and winter thunderstorms in the Mediterranean. During the trailing jet phase of the GJ, a sprite with halo triggered by a nearby cloud-to-ground lightning flash occurred at a relatively low altitude (origins in the cloud (i.e., a positive cloud-to-ionosphere discharge, +CI), with a large total charge moment change of 11600 C km and a maximum current of 3.3 kA. Early VLF transmitter amplitude perturbations detected concurrently with the GJ confirm the production of large conductivity changes due to electron density enhancements in the D-region of the ionosphere.
Worstell, M. W.; Mauel, M. E.; Roberts, T. M.
Space and laboratory plasma confined by a strong magnetic field have remarkable properties. Low frequency mixing of the plasma occurs through the interchange of long plasma-filled tubes aligned with the magnetic field. The plasma dynamics becomes two-dimensional because these tubes can only move radially or circulate around the poles of the magnetic dipole. Studies of turbulent interchange dynamics made using the Collisionless Terella Experiment (CTX) show that turbulence appears as chaotic time-varying modes with broad global mode structures that interact nonlinearly and form an inverse cascade.footnotetextB.A. Grierson, M.W. Worstell, M.E. Mauel, Phys. Plasmas 16 055902 (2009) When we drive vortex mixing through the application of electrostatic bias to multiple probes, we break the rotational symmetry of the plasma and small vortex tubes are seen to drive larger ``gigantic'' swirls. Statistical analysis of the time-evolving spectra and measurement of the bicoherence of the turbulence show an increase of three wave coupling during non-axisymmetric electrostatic drive of the probe array.
Lee, Li-Jou; Huang, Sung-Ming; Chou, Jung-Kung; Kuo, Cheng-Ling; Chen, Alfred B.; Su, Han-Tzong; Hsu, Rue-Rou; Frey, Harald U.; Takahashi, Yukihiro; Lee, Lou-Chuang
Secondary transient luminous events (TLEs) recorded by the ISUAL-FORMOSAT2 mission can either be secondary jets or secondary gigantic jets (GJs), depending on their terminal altitudes. The secondary jets emerge from the cloud top beneath the preceding sprites and extend upward to the base of the sprites at ˜50 km. The secondary jets likely are negative electric discharges with vertically straight luminous columns, morphologically resembling the trailing jet of the type-I GJs. The number of luminous columns in a secondary jet seems to be affected by the size of the effective capacitor plate formed near the base of the preceding sprites and the charge distribution left behind by the sprite-inducing positive cloud-to-ground discharges. The secondary GJs originate from the cloud top under the shielding area of the preceding sprites, and develop upward to reach the lower ionosphere at ˜90 km. The observed morphology of the secondary GJs can either be the curvy shifted secondary GJs extending outside the region occupied by the preceding sprites or the straight pop-through secondary GJs developing through the center of the preceding circular sprites. A key factor in determining the terminal height of the secondary TLEs appears to be the local ionosphere boundary height that established by the preceding sprites. The abundance and the distribution of the negative charge in the thundercloud following the sprite-inducing positive cloud-to-ground discharges may play important role in the generation of the secondary TLEs.
Ana G Ferreira
Full Text Available Sellar plasmacytomas are rare and the differential diagnosis with non-functioning pituitary adenomas might be difficult because of clinical and radiological resemblance. They usually present with neurological signs and intact anterior pituitary function. Some may already have or eventually progress to multiple myeloma. We describe a case associated with extensive anterior pituitary involvement, which is a rare form of presentation. A 68-year-old man was referred to our Endocrinology outpatient clinic due to gynecomastia, reduced libido and sexual impotence. Physical examination, breast ultrasound and mammography confirmed bilateral gynecomastia. Blood tests revealed slight hyperprolactinemia, low testosterone levels, low cortisol levels and central hypothyroidism. Sellar MRI showed a heterogeneous sellar mass (56 × 60 × 61 mm, initially suspected as an invasive macroadenoma. After correcting the pituitary deficits with hydrocortisone and levothyroxine, the patient underwent transsphenoidal surgery. Histological examination revealed a plasmacytoma and multiple myeloma was ruled out. The patient was unsuccessfully treated with radiation therapy (no tumor shrinkage. Myeloma ultimately developed, with several other similar lesions in different locations. The patient was started on chemotherapy, had a bone marrow transplant and is now stable (progression free on lenalidomide and dexamethasone. The presenting symptoms and panhypopituitarism persisted, requiring chronic replacement treatment with levothyroxine, hydrocortisone and testosterone.
Kelberman, Daniel; Rizzoti, Karine; Lovell-Badge, Robin; Robinson, Iain C A F; Dattani, Mehul T
Normal hypothalamopituitary development is closely related to that of the forebrain and is dependent upon a complex genetic cascade of transcription factors and signaling molecules that may be either intrinsic or extrinsic to the developing Rathke's pouch. These factors dictate organ commitment, cell differentiation, and cell proliferation within the anterior pituitary. Abnormalities in these processes are associated with congenital hypopituitarism, a spectrum of disorders that includes syndromic disorders such as septo-optic dysplasia, combined pituitary hormone deficiencies, and isolated hormone deficiencies, of which the commonest is GH deficiency. The highly variable clinical phenotypes can now in part be explained due to research performed over the last 20 yr, based mainly on naturally occurring and transgenic animal models. Mutations in genes encoding both signaling molecules and transcription factors have been implicated in the etiology of hypopituitarism, with or without other syndromic features, in mice and humans. To date, mutations in known genes account for a small proportion of cases of hypopituitarism in humans. However, these mutations have led to a greater understanding of the genetic interactions that lead to normal pituitary development. This review attempts to describe the complexity of pituitary development in the rodent, with particular emphasis on those factors that, when mutated, are associated with hypopituitarism in humans.
Bonafe, A.; Sobel, D.; Manelfe, C.
The relative value of a second generation CT scanner and complex motion polytomography are compared in 36 patients undergoing transphenoidal surgery due to clinical and biological evidence of pituitary hypersecretion. CT findings of focal glandular hypodensity in 17 patients and upward convexity of the sellar contens in 15 patients suggested the presence of a pituitary microadenoma. Complex motion tomographic findings suggesting a microadenoma were found in 21 of 32 cases and included bulging of the sellar floor or localized thinning of the lamina dura. Selective transsphenoidal adenomectomy was performed on 34 of the 36 patients and the preoperative location based on CT scanning data was positively correlated with surgical results in 75% of the cases. When there is a strong clinical suspicion of pituitary hypersecretion both CT and polytomography may suggest the existence and location of a microadenoma. CT should be the radiographic technique of choice since it produces accurate information as to the size and density of the pituitary gland. (orig.)
Horacio Armando Marenco
Full Text Available Over the past three decades, surgical series of elderly patients treated for pituitary adenomas have been published, all of which used the microscopic transsphenoidal or transcranial approach. The objective of this study was to retrospectively analyze the surgical results of our first 25 elderly patients with non-functioning pituitary macroadenoma (NFPM operated by the endoscopic endonasal approach (EEA. Preoperative visual loss was found in 92.8% of the cases, and 70.8% experienced visual improvement following surgery. Preoperative pituitary dysfunction was found in 69.2% of the cases and postoperative pituitary recovery occurred in 22.2% of them. Mean hospital stay was 6.7 days. The results of this study suggest that surgery remains the first line of treatment for NFPM in the elderly. Because age alone is not a barrier for surgery, patients should be selected for surgical treatment based on their symptoms and clinical condition, as defined by comorbidities.
Fuks, Z.; Glatstein, E.; Marsa, G.W.; Bagshaw, M.A.; Kaplan, H.S.
Chronic damage following external irradiation of the normal pituitary and thyroid glands, delivered incidentally during radiotherapy of neoplasms of the head and neck may be more common than has been appreciated in the past. A case of a child who developed pituitary dwarfism 5 1 / 2 years after radiation therapy had been delivered for an embryonal rhabdomyosarcoma of the nasopharynx is described. A review of similar cases from the literature is presented. Likewise, external irradiation of the normal thyroid gland produces a spectrum of radiation-induced syndromes. Clinical damage to the pituitary and thyroid glands is usually manifested months to years after treatment and is preceded by a long subclinical phase. A careful exclusion of these glands from radiation treatment fields is recommended whenever possible. An early detection of endocrine function abnormalities in patients receiving radiation to these glands is desirable, since appropriate treatment may prevent the late deleterious effects of external irradiation of the pituitary and thyroid glands
Full Text Available Introduction. Invasive tumours in Nelson’s syndrome need aggressive therapy. Recent reports have documented the efficacy of temozolomide (TMZ in the treatment of adenomas resistant to conventional management. Objective. The review of the literature concerning TMZ treatment of atypical corticotroph adenomas and a case study of 56-year-old woman who developed Nelson’s syndrome. Treatment Proceeding. The patient with Cushing’s disease underwent transsphenoidal adenomectomy followed by a 27-month-long period of remission. Due to a regrowth of the tumor, she underwent two reoperations followed by stereotactic radiotherapy. Because of treatment failures, bilateral adrenalectomy was performed. Then she developed Nelson’s syndrome. A fourth transsphenoidal adenomectomy was performed, but there was a rapid recurrence. Five months later, she underwent a right frontotemporal craniotomy. Due to a rapid regrowth of the tumour, the patient did not receive gamma-knife therapy and was treated with cabergoline and somatostatin analogue for some time. Only TMZ therapy resulted in marked clinical, biochemical, and radiological improvement. To date, this is the first case of invasive corticotroph adenoma in Nelson’s syndrome treated with temozolomide in Poland. Conclusion. In our opinion, temozolomide can be an effective treatment option of invasive adenomas in Nelson’s syndrome.
Pituitary dwarfism, associated with growth hormone deficiency, is an autosomal, recessively inherited disorder in shepherd dogs. Due to the serious nature of pituitary dwarfism and lack of efficient treatment, it is preferable to prevent dwarfs from being born by applying a correct breeding policy. However, because pituitary dwarfism is a recessively inherited disorder and carriers do not differ phenotypically from non-carriers, genetic testing is required to prevent mating of 2 carriers. But...
Moran, Amy L; Woods, H Arthur
Beginning with the earliest expeditions to the poles, over 100 years ago, scientists have compiled an impressive list of polar taxa whose body sizes are unusually large. This phenomenon has become known as 'polar gigantism'. In the intervening years, biologists have proposed a multitude of hypotheses to explain polar gigantism. These hypotheses run the gamut from invoking release from physical and physiological constraints, to systematic changes in developmental trajectories, to community-level outcomes of broader ecological and evolutionary processes. Here we review polar gigantism and emphasize two main problems. The first is to determine the true strength and generality of this pattern: how prevalent is polar gigantism across taxonomic units? Despite many published descriptions of polar giants, we still have a poor grasp of whether these species are unusual outliers or represent more systematic shifts in distributions of body size. Indeed, current data indicate that some groups show gigantism at the poles whereas others show nanism. The second problem is to identify underlying mechanisms or processes that could drive taxa, or even just allow them, to evolve especially large body size. The contenders are diverse and no clear winner has yet emerged. Distinguishing among the contenders will require better sampling of taxa in both temperate and polar waters and sustained efforts by comparative physiologists and evolutionary ecologists in a strongly comparative framework.
Vierimaa, O. (Outi)
Abstract Multiple endocrine neoplasia type 1 (MEN1) is an inherited syndrome characterized by parathyroid, gastroenteropancreatic and pituitary neuroendocrine tumours. In Northern Finland, two founder mutations of the MEN1 gene (1466del12, 1657insC) accounting for the majority of the MEN1 cases, have common ancestors born in the 18th and 19th centuries, respectively. Three small clusters of familial pituitary adenoma have also been detected, two of which could be linked by genealogy to a ...
Teller, W M
This survey deals with disorders caused by genetically disturbed function of the anterior pituitary gland. Genetic Dwarfism may be caused by isolated growth hormone deficiency (IGHD) or panpituitary diseases, such as congenital absence of the pituitary or familial panhypopituitarism. Genetic disturbances of isolated pituitary hormone secretion without dwarfism may occur as isolated gonadotropin deficiency (IGD), isolated luteinizing hormone deficiency ("fertile eunuch"), Kallmann syndrome (olfactogenital dysplasia), isolated thyrotropin deficiency (ITD) and isolated corticotropin deficiency (ICD). Pituitary dysfunction may also be associated with other genetic disease entities.
Ahmed Imran Siddiqi, MBBS, MRCP
Full Text Available We describe a patient with pituitary hyperplasia due to primary hypothyroidism. Pituitary hyperplasia and pituitary masses cannot be reliably differentiated on imaging alone, despite significant improvement in imaging quality in recent years.
Full Text Available Background The presence of ganglion cells within an endocrine pituitary adenoma in sellar region is rare, and is usually diagnosed as "mixed gangliocytoma-pituitary adenoma". Due to lack of radiological characteristics, it is very difficult to make an accurate diagnosis preoperatively. Herein we describe one case of unusual mixed gangliocytoma - growth hormone (GH secreting pituitary adenoma in sellar region and review related literatures, so as to summarize the clinicopathological characteristics and improve the diagnosis and differential diagnosis of this tumor. Methods and Results A 28 - year - old female presented with headache and blurred vision for 8 months. She also complained of acromegaly and amenorrhea. Head CT and MRI examinations showed a sellar and suprasellar mass with clear boundary compressing the optic chiasm and buttom of the third ventricle. The mass exhibited isointense signal or mild hypointensity on T1WI and mild hyperintensity on T2WI with heterogeneous enhancement on the contrast MRI. The tumor was removed totally. The histological sections demonstrated two parts of intermixed areas. One part of areas was marked by a proliferation of scattered gangliocyte - like cells arranged in a fibrillary background. Other areas were marked by a sheet - like or locally papillary proliferation of round and oval cells. Immunohistochemically, cytoplasm of gangliocyte-cells were diffusely positive for synaptophysin (Syn, and negative for adenohypophysial hormones; cytoplasm of round and oval cells were diffusely positive for Syn, and almost 30% cells were positive for GH, and negative for other neurohypophysial hormones. A final diagnosis of mixed gangliocytoma-GH secreting pituitary adenoma in sellar region (WHO grade Ⅰ was made. The patient did not receive postoperatively adjuvant therapy and was followed-up for one year, without any neurological deficit or signs of recurrence. Conclusions Mixed gangliocytoma - pituitary
McCormack, Shana E; Li, Dong; Kim, Yeon Joo; Lee, Ji Young; Kim, Soo-Hyun; Rapaport, Robert; Levine, Michael A
Pituitary stalk interruption syndrome (PSIS, ORPHA95496) is a congenital defect of the pituitary gland characterized by the triad of a very thin/interrupted pituitary stalk, an ectopic (or absent) posterior pituitary gland, and hypoplasia or aplasia of the anterior pituitary gland. Complex genetic patterns of inheritance of this disorder are increasingly recognized. The objective of this study was to identify a genetic cause of PSIS in an affected child. Whole exome sequencing (WES) was performed by using standard techniques, with prioritized genetic variants confirmed via Sanger sequencing. To investigate the effects of one candidate variant on mutant WDR11 function, Western blotting and coimmunofluorescence were used to assess binding capacity, and leptomycin B exposure along with immunofluorescence was used to assess nuclear localization. We describe a child who presented in infancy with combined pituitary hormone deficiencies and whose brain imaging demonstrated a small anterior pituitary, ectopic posterior pituitary, and a thin, interrupted stalk. WES demonstrated heterozygous missense mutations in two genes required for pituitary development, a known loss-of-function mutation in PROKR2 (c.253C>T;p.R85C) inherited from an unaffected mother, and a WDR11 (c.1306A>G;p.I436V) mutation inherited from an unaffected father. Mutant WDR11 loses its capacity to bind to its functional partner, EMX1, and to localize to the nucleus. WES in a child with PSIS and his unaffected family implicates a digenic mechanism of inheritance. In cases of hypopituitarism in which there is incomplete segregation of a monogenic genotype with the phenotype, the possibility that a second genetic locus is involved should be considered. Copyright © 2017 Endocrine Society
In this thesis, we explored pituitary functioning and extra-pituitary implications of two pituitary disorders in humans. In part A, we focused on the long-term consequences of the diagnosis and treatment of nonfunctioning pituitary macroadenoma (NFMA) on hypothalamic regulation of circadian