Cases reported "Acromegaly"

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1/75. Kleine-Levin and Munchausen syndromes in a patient with recurrent acromegaly.

    Hypothalamic disease often affects the patients' personality and this also applies to pituitary tumors with suprasellar extension. We report on a patient with a 12-year history of recurrent acromegaly, treated with three transphenoidal operations, single field radiation therapy and bromocriptine/octreotide administration. During the course of follow-up she presented with self-inflicted anemia and kleine-levin syndrome (hypersomnia, hyperphagia and hypersexuality). Furthermore, she developed post-radiation necrosis within the right temporal lobe. Whether her neurological and personality disorders result - at least partially - from the acromegaly or the temporal lobe necrosis remains unclear.
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2/75. Preoperative short-term administration of octreotide for facilitating transsphenoidal removal of invasive growth hormone-secreting macroadenomas.

    The somatostatin analog octreotide was administered prior to transsphenoidal surgery in three patients with tumors that extended to the suprasellar space and one side of the cavernous sinus. octreotide, 100 micrograms twice a day, was subcutaneously injected for 2 weeks. octreotide administration reduced the serum growth hormone (GH) levels in these patients from 82 to 22 ng/ml, from 148 to 12 ng/ml, and from 129 to 9 ng/ml. The tumor size shrank by about 50%, and the suprasellar extension disappeared in two patients. The main tumor was sharply dissected from the normal pituitary gland at surgery. Intracavernous portions were removed using a curette. Postoperatively, GH levels were less than 5 ng/ml in two patients, and 8.5 ng/ml in one patient. Follow-up magnetic resonance imaging revealed a small residual tumor in one side of the cavernous sinus in all patients. Follow-up GH levels were less than 5 ng/ml in one patient, and less than 2 ng/ml in two patients treated with bromocriptine. Preoperative administration of octreotide for 2 weeks reduced tumor volume and allowed near-total surgical resection of invasive macroadenomas without compromising the treatment course. Residual tumor due to intracavernous extension can be managed with bromocriptine or gamma knife radiosurgery.
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keywords = sella
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3/75. Pituitary adenoma with neuronal choristoma: a report of two rare cases.

    Two rare cases of pituitary adenoma with neuronal choristoma are described. Both patients were female and presented with features of acromegaly with elevated growth hormone and prolactin levels. Radiologically, both lesions were predominantly intrasellar in location with extension into suprasellar region, but hypothalamus was not involved. Histopathological examination revealed a mixture of chromophobe pituitary adenoma cells and neuronal cells. In both cases, the adenoma component was positive for growth hormone and prolactin. Interestingly, immunopositivity for alpha-subunit, cytokeratin and prolactin was seen in the adenoma and neuronal cells in one case. Our findings support the hypothesis that the neuronal cells possibly arise from adenoma cells as a result of metaplasia.
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keywords = sella
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4/75. acromegaly with normal growth-hormone levels and pituitary histology. Case report.

    In rare cases, acromegalic patients have normal basal concentrations of growth hormone, and their acromegaly results from abnormal growth-hormone secretory patterns. A patient is reported with the clinical features of acromegaly, who had elevated somatomedin levels and an enlarged sella turcica, but whose serum growth-hormone levels on continuous monitoring were in the normal range, with levels of 2.8 to 8.9 ng/ml. Dynamic studies of growth hormone revealed normal responses to hypo- and hyperglycemia, but abnormal responses to L-dopa and thyroid-releasing hormone. At surgery, neither a pituitary adenoma nor eosinophilic hyperplasia was present. It is likely that this patient's acromegaly resulted from the presence of chronically high normal levels of growth hormone.
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ranking = 7.5140752665016
keywords = sella turcica, turcica, sella
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5/75. Sphenoidal sinus mucocele after transsphenoidal surgery for acromegaly.

    This report concerns one case of a sphenoid sinus mucocele occurring 17 years after transsphenoidal surgery for acromegaly. In 1979, a 51-year-old man was successfully operated by the transnasal transsphenoidal approach for a growth hormone (GH) adenoma 1 cm in diameter. In 1996, the patient was hospitalized for headaches and diplopia. He presented a loss of right visual acuity with paralysis of the right oculomotor nerve. The basal GH level was normal with a satisfactory decrease after oral glucose ingestion. Pituitary sellar radiography showed a disappearance of the posterior clinoid while magnetic resonance imaging revealed the existence of a bilocular, circular, homogeneous lesion of the sphenoid sinus 3 cm in diameter with a posterior and lateral extension. The diagnosis of mucocele was confirmed by surgical treatment, allowing drainage of the mucocele through a transsphenoidal approach. The drained material was composed of sinus epithelium containing many polynuclear and resorptive cells. Postoperatively, the symptoms decreased dramatically, leading to full recovery of visual function and disappearance of the headaches. Apart from the tumor recurrence, the mucocele of the sphenoid sinus can be evoked as a possible long term complication of transsphenoidal surgery for pituitary adenoma.
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keywords = sella
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6/75. Double adenoma of the pituitary: a somatotroph adenoma colliding with a gonadotroph adenoma.

    Pituitary collision tumors are rare. They may create difficult diagnostic problems and their histogenesis is not clear. We report here an unusual case of a somatotroph adenoma colliding with a gonadotroph adenoma.The 64-year-old man had clinical acromegaly. His blood growth hormone level was elevated and magnetic resonance imaging demonstrated a pituitary tumor. The surgically removed sellar mass was investigated by histology, immunocytochemistry and electron microscopy. Morphologic study revealed a collision tumor; one was a somatotroph adenoma, the other a gonadotroph adenoma. Authors call attention to the difficulties in clinical, imaging and pathological diagnosis. Detailed morphologic studies are needed to establish the presence of two distinct tumors composed of two different cell types.
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keywords = sella
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7/75. 'Gangliocytomas' of the pituitary: a heterogeneous group of lesions with differing histogenesis.

    Hamartomatous or neoplastic ganglion cells in the sella turcica are an unusual cause of symptoms. They have been reported in association with a functioning or nonfunctioning pituitary adenoma, with pituitary cell hyperplasia, and occasionally as masses unassociated with an adenoma, again with variable endocrinologic findings. Fewer than 50 cases of intrasellar ganglion cell lesions have been reported in the literature, only six of them associated with Cushing's syndrome. We describe the clinicopathologic features of another eight patients, three of whom presented with acromegaly, four with apparently nonfunctioning adenohypophyseal masses, and one with Cushing's syndrome. On histology, six of them were found to have sparsely granulated growth hormone (GH)-producing adenomas with ganglion cell areas, one appeared to have a gangliocytoma not associated with an adenoma, whereas the eighth had a ganglion cell lesion in the posterior pituitary. The morphologic and immunohistochemical findings suggest that the ganglion cell component of seven of these tumors has resulted from neuronal differentiation in a GH-producing adenoma, despite the lack of demonstrable adenoma in one case. A true sellar "gangliocytoma" or hamartoma of ectopic hypothalamic-type neurons appears to be a rarer explanation for the presence of ganglion cells in a pituitary biopsy.
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ranking = 9.5140752665016
keywords = sella turcica, turcica, sella
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8/75. acromegaly associated with a granular cell tumor of the neurohypophysis: a clinical and histological study. Case report.

    acromegaly is usually caused by a growth hormone (GH)-secreting pituitary adenoma, and hypersecretion of GH-releasing hormone (GHRH) from a hypothalamic or neuroendocrine tumor accounts for other cases. The authors report on the unusual association of acromegaly with a granular cell tumor of the neurohypophysis. A 42-year-old woman with a 10-year history of acral enlargement, headache, and menstrual abnormalities was referred to our department for a suspected GH-secreting pituitary adenoma. The patient's basal GH levels were mildly elevated at 4.8 microg/L, were not suppressed in response to an oral glucose tolerance test, and increased paradoxically after administration of thyrotropin-releasing hormone. The patient's insulin-like growth factor-1 (IGF-1) level was elevated at 462 microg/L, whereas a magnetic resonance image of the sella turcica revealed an intra- and suprasellar lesion that was compatible with a diagnosis of pituitary adenoma. A transsphenoidal approach to remove the lesion, which was mainly suprasellar, was successful during a second operative attempt, resulting in the clinical and biochemical regression of the patient's acromegaly. Four months postoperatively, the patient's basal GH level was 0.9 microg/L and her IGF-1 level was 140 microg/L. Histological analysis of the operative specimen demonstrated a granular cell tumor of the neurohypophysis, which when stained proved negative for pituitary hormones and GHRH. This case represents the first reported association between a granular cell tumor of the neurohypophysis and acromegaly. granular cell tumor of the neurohypophysis could be added to the restricted list of neoplastic causes of acromegaly secondary to hypersecretion of a GH-releasing substance.
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ranking = 9.5140752665016
keywords = sella turcica, turcica, sella
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9/75. Glucocorticoid-dependency on GH secretion and tumor growth in a GH-producing pituitary adenoma with Cushing's syndrome.

    We report a rare case of a 40-year-old woman with Cushing's syndrome and acromegaly. At the age of 28 she was diagnosed with Cushing's syndrome due to a left adrenal tumor concomitant with a GH-producing pituitary tumor. Before adrenal surgery her basal GH levels were extremely high and CT scanning revealed a high-density mass in the sella turcica. A 28 g left adrenocortical adenoma was removed by adrenalectomy. During the four months after the adrenalectomy, basal GH levels dramatically decreased and the high-density mass detected by CT scanning had disappeared but the basal GH levels and IGF-1 had not been normalized. She gradually became acromegalic in the twelve years after the adrenalectomy. At the age of 40 CT scanning showed reappearance of the pituitary tumor. In order to examine the glucocorticoid dependency on GH secretion, we compared the GH secretion in a series of endocrinological tests before and after oral 8 mg dexamethasone administration for 7 days. There was no difference between before and after dexamethasone administration in the GH secreting pattern, but basal GH levels were apparently increased after dexamethasone treatment. Transsphenoidal surgery was done and pathological examination showed a GH-producing pituitary adenoma. in vitro, dexamethasone increased GH secretion from the cultured GH-producing adenoma cells in a dose-dependent manner. In this case, both GH secretion and pituitary tumor growth seemed to be dependent on glucocorticoid.
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ranking = 7.5140752665016
keywords = sella turcica, turcica, sella
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10/75. Apoplexy of pituitary macroadenoma after combined test of anterior pituitary function.

    pituitary apoplexy has been reported as a very rare complication of combined tests of anterior pituitary function and of TRH or gonadotropin-releasing hormone (GnRH) administration in pituitary tumor. A 34-year-old man with a GH-secreting pituitary macroadenoma and diabetes mellitus received an injection of 400 microg TRH, 100 microg GnRH, and 0.15 U/Kg regular insulin. Twenty minutes later, he complained of a severe headache and vomited. visual acuity and visual field did not change and his headache was persistent during the next 24 hours of conservative management. magnetic resonance imaging (MRI) of the sella turcica done the day after the event showed definitive elevation of the optic chiasm and slight enlargement of tumor and focal areas of mixed high signal and low signal intensities in the macroadenoma on noncontrast T1-weighted images. headache subsided markedly within a day of octreotide therapy. Transsphenoidal removal of the pituitary tumor was performed 9 days after the hormone study. Ischemic necrosis and hemorrhage were confirmed in the acidophilic adenoma with positive immunostaining for GH. Postoperative course was uneventful and his serum insulin-like growth factor-1 (IGF-1) level and blood glucose levels were normalized. Three months after the surgery the dynamic test was repeated without adverse effects. To our knowledge, this is a very rare case of apoplexy of GH-secreting pituitary adenoma after a combined stimulation test of anterior pituitary function.
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ranking = 7.5140752665016
keywords = sella turcica, turcica, sella
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