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Pituitary turnors with large suprasellar extensions are a difficult surgical challenge. A series of 11 patients with giant pituitary adenomas is reported. Seven men and four women (mean age 54.1 years). were diagnosed following a mean duration of symptoms of 60 months, Common presenting symptoms included visual disturbances, headache, personality changes, and panhypopituitarism. A single patient presented with rapid onset of coma and oculomotor nerve palsy. Eight patients underwent a transsphenoidal approach, and three patients underwent a craniotomy as the initial surgical procedure. A total of 16 surgical procedures were performed, resulting in complete or near complete resection in seven patients, and partial removal in four. Six patients had a good outcome and one patient in poor condition prior to surgery was unchanged postoperatively. One patient was worse following surgery, and there were two operative deaths. These tumors have a consistency and a propensity to adhere to neurovascular structures, making complete surgical resection difficult. Management should be individualized and should be based upon the radiographic and clinical features of the tumor. We feel that most leslons are best approached initially transsphenoidally, unless there is significant lateral extension. In many patients, aggressive surgery is not indicated and limited subtotal transsphenoidal resection followed by irradiation is recommended. Surgical decision making and strategy is discussed in relation to our recent experience with giant pitnitary adenomas.
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PMID:Microsurgical management of giant pituitary tumors. 1717 Sep 49

Gangliogliomas are uncommon central nervous system (CNS) tumors composed of a mixture of glial and neuronal elements. Although these tumors can occur in any portion of the central nervous system, involvement of the ventricular system is rare. We herein report on two cases of gangliogliomas in the third ventricle in a 34-year-old woman and in a 52-year-old man. One patient presented only with headaches, and the other presented symptoms associated with panhypopituitarism and diabetes insipidus. In the first case the tumor in the middle portion of the third ventricle was successfully removed by a transcallosal subchoroidal approach. In the second case the hemorrhagic tumor was located in the anterior floor of the third ventricle and was removed by an anterior inter-hemispheric trans-lamina terminalis approach. To date, follow-ups of both patients have involved no adjuvant therapy, and there have been no signs of tumor recurrence on magnetic resonance images. The nature, radiological findings, and treatments of these tumors are discussed.
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PMID:Ganglioglioma in the third ventricle: report on two cases. 1749 19

We encountered a rare case of neurohypophyseal germinoma with a prominent granulomatous reaction, which invaded the right cavernous sinus. The neuroimaging and histopathology features in this case were unique, distinguishing it from other types of suprasellar lesions. A 13-year-old boy presented with loss of appetite and polyuria; both symptoms were present for 1 year, and headache, general fatigue and blurred vision present for the prior 2 months. On admission, neurological examination indicated bitemporal hemianopsia and optic atrophy. Endocrinological exam showed panhypopituitarism. Tumor markers such as alpha-fetoprotein, human growth hormone, carcinoembryonic antigen, and placental alkaline phosphatase were negative. Brain CT revealed a suprasellar tumor with calcification. MR T(1)-weighted and T(2)-weighted images showed the tumor to be isointense to normal brain parenchyma and to be enhanced densely. The tumor also involved the right cavernous sinus, so that a biopsy was performed by the transsphenoidal approach. On pathologic examination of the specimen, typical large tumor cells with lymphocytic cell infiltration and prominent granulomatous reaction were observed. Neurohypophyseal granulomatous germinoma was diagnosed. Radiotherapy was performed with a total dose of 51 Gy and the tumor shrank remarkably. The patient returned to school under hormone replacement therapy.
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PMID:Neurohypophyseal granulomatous germinoma invading the right cavernous sinus: case report and review of the literature. 1762 46

Pituitary apoplexy (PA), which is one of the most serious life-threatening complications of pituitary adenoma, is characterized by abrupt onset of headache, nausea, vomiting, visual disturbances and oculomotor paresis. Combination of oculomotor cranial nerve paralysis with normal visual fields is very rare in PA. We report a 60-year-old acromegalic man presented with panhypopituitarism and bilateral total opthalmoplegia without a visual field defect. At initial evaluation his clinical findings were compatible with adrenal crisis and eye examination revealed total opthalmoplegia, bilateral ptosis and normal vision. MRI showed a large heterogeneous mass in the pituitary fossa. Although clinical findings due to adrenal crisis improved after glucocorticoid therapy there was no improvement in opthalmoplegia and ptosis. The patient underwent transsphenoidal excision of the pituitary mass. Histological examination revealed an adenoma with large areas of hemorrhagic infarction and most of the cells were positive for GH in immunohistochemical analysis. Although opthalmoplegia was severe at presentation, total recovery was achieved 3 months after transsphenoidal surgery. Therefore the presented case clearly demonstrates that opthalmoplegia without a visual field defect due to PA has a good prognosis and early diagnosis and treatment including surgical decompression are crucially important.
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PMID:Complete surgical resolution of bilateral total opthalmoplegia without visual field defect in an acromegalic patient presented with pituitary apoplexy. 1778 21

A 49 y.o. man was admitted for investigation of an intense fatigue associated with nausea, vomiting, weight loss and headache. Examination and work-up reveals a moderate hypercalcaemia and a panhypopituitarism attributed to a craniopharyngioma. Extensive work-up has excluded the most frequent causes of hypercalcaemia and we finally attributed this anomaly to the adrenal failure. In this article, we discuss the diagnostic approach, the etiology and physiopathology of this hypercalcemia and his association with endocrinological anomalies such as adrenal failure.
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PMID:[Adrenal insufficiency and hypercalcemia--an unusual presentation]. 1805 Jun 1

Idiopathic granulomatous hypophysitis is a rare entity. The usual clinical presentation is that of an expanding mass lesion with varying degree of hypopituitarism. This patient described presented to us with severe headache along with panhypopituitarism and post-operative diabetes insipidus.
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PMID:Granulomatous hypophysitis--an interesting and rare cause mimicking pituitary mass. 1805 39

A 23 year-old patient was referred to the endocrine outpatient clinic with the suspicion of diabetes insipidus as he complained of nycturia and polydipsia since 2-3 months. Further he presented with nausea, vomiting, loss of appetite, rapid weight loss, diffuse body pain and fatigue. No headache, blurred vision, or fever were reported. The clinical examination showed sexual infantilism (poor beard, pubic and axillary hair growth, small testis). The patient's skin was strikingly pale as well as dry and scaly. Lymph node palpation was unremarkable. Endocrine evaluation revealed diabetes insipidus as well as complete anterior pituitary insufficiency. MR imaging demonstrated contrast-enhancing mass lesions at the pineal gland, hypothalamus, and anterior horn of lateral ventricles bilaterally. The localization pattern deemed to be highly suspicious for intracranial germinoma. As beta-HCG and AFP were negative in serum and cerebrospinal fluid the diagnosis of germinoma was confirmed histologically. After radiotherapy with cranio-spinal radiation therapy with 24 Gy followed by two weeks of local tumor boost with 16 Gy, the posttherapy MRI scan indicated complete tumor removal. This case demonstrates a very rare and potentially curable tumor as the cause of panhypopituitarism in adults.
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PMID:Intracranial germinoma as a very rare cause of panhypopituitarism in a 23-year old man. 1905 25

Pituitary apoplexy indicates pituitary adenoma hemorrhage, which could result in acute pituitary insufficiency and mortality. The typical symptoms are headache, visual disturbance, nausea, vomiting, altered mental status, and panhypopituitarism. However, cortisol-induced hyperglycemia and acute delirium could be an initial presentation of a pituitary adenoma hemorrhage with stormy release of the adrenocorticotrophic hormone. A 28-year-old woman presented with severe vomiting, irritable state, and delusion. She had medical history of irregular menstrual cycles and marked body weight gain after her second childbirth 8 years ago. She was diagnosed of diabetic ketoacidosis 2 days before this visiting at local medical department. On physical examination, Cushing appearance without definite neurological deficit was disclosed. Further blood tests revealed high blood sugar, cortisol, and adrenocorticotrophic hormone levels without evidence of diabetic ketoacidosis. The brain computed tomography and magnetic resonance imaging showed pituitary macroadenoma and pituitary hemorrhage. Cushing disease with pituitary apoplexy was then diagnosed. Conservative management with delayed neurosurgery was applied. The patient became clear with normalized cortisol and blood sugar levels soon after. Follow-up computed tomography scan of the brain revealed no progression of tumor bleeding or mass effect. To our knowledge, pituitary apoplexy associated with cortisol-induced hyperglycemia and acute delirium has never been reported before. This case reminds us of pituitary apoplexy and its rare manifestations.
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PMID:Pituitary apoplexy associated with cortisol-induced hyperglycemia and acute delirium. 1909 Dec 87

Hypophysitis secondary to a ruptured Rathke's cyst is rare. We describe a 53-year-old female who presented with headache and subsequently developed aseptic meningitis and panhypopituitarism. MRI findings and concomitant cardiac arrhythmia and peripheral vasculitis led to a provisional diagnosis of neurosarcoidosis. There were no respiratory manifestations of sarcoidosis. Improvement was noted with empirical treatment with steroids. Pituitary biopsy was undertaken to confirm the diagnosis prior to treatment with long-term immunosuppression for putative neurosarcoidosis. The biopsy revealed lymphocytic hypophysitis secondary to a ruptured Rathke's cyst. This report highlights a rare pathology and the importance of a tissue diagnosis before undertaking non-surgical management of a pituitary mass.
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PMID:Hypophysitis secondary to ruptured Rathke's cyst mimicking neurosarcoidosis. 1923 Nov 95

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.
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PMID:Panhypopituitarism due to metastases to the hypothalamus and the pituitary resulting from primary breast cancer: a case report and review of the literature. 1993 72


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