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Nasopharyngeal extension of a craniopharyngioma is very rare and usually presents with headache, nasal obstruction and visual disturbances. We present a case of a 4 year old girl, who became symptomatic with visual deterioration. MRI showed a huge supra - and infrasellar cystic craniopharyngioma with extension into the sphenoid sinus. Primary treatment was a transnasal puncture of the cyst followed by a subfrontal approach with removal of the tumour preserving the chiasm and optic nerves. The visual acuity postoperatively improved while she needed hormone replacement due to panhypopituitarism. Follow-up 12 months after the operation showed no recurrence. This is the youngest patient of about 27 patients reported so far in the literature.
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PMID:Nasopharyngeal extension of a craniopharyngioma in a 4 year old girl. 1181 Mar 94

Inflammatory diseases of the pituitary gland constitute a group of interest because of their scarce frequency, because the disorder presents with symptoms of hypopituitarism and expanding sellar mass and because of their therapeutics implications. We present one case of idiopathic granulomatous hypophysitis, in a 55-years-old patient with daily headaches, panhypopituitarism and a sellar mass lesion. Granulomatous hypophysitis is characterized by granulomas with epithelioid histiocytes and multinucleated giant cells but also shows lymphocyte collections. With respect to immunohistochemistry our results show histiocytes (CD68+) and an heterogeneous inflammatory infiltrate (CD45RO+ y CD20+). We analyze the differential diagnosis with another granulomatous processes, infectious or not infectious, and with the histiocytosis. We examine the possible relation with the lymphocytic hypophysitis.
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PMID:[Idiopathic granulomatous hypophysitis. Morphological and immunohistochemical study of a case]. 1205 6

Lymphocytic hypophysitis is a rare inflammatory disorder which is caused by autoimmune destruction of the pituitary gland. Almost all reported cases have been in women and the disease is often associated with pregnancy. We describe here the first male case of lymphocytic hypophysitis in Korea. The patient presented with headache, impotence, decreased libido, and deteriorated vision. Endocrinologic studies showed panhypopituitarism, and pituitary MRI imaging revealed a homogeneously enhanced pituitary mass with a thickened stalk. Treatment with prednisolone and thyroid hormone for five months was ineffective. Transsphenoidal resection of the pituitary mass was performed successfully with normalization of the visual field defect. Histologic examination revealed diffuse lymphocytic infiltration with dense collagenous fibrosis, consistent with lymphocytic hypophysitis. Lymphocytic hypophysitis should be considered in differential diagnosis even in men with hypopituitarism and an enlarged pituitary gland.
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PMID:First male case of lymphocytic hypophysitis in Korea. 1269 33

We report here two cases of pituitary apoplexy or pseudoapoplexy revealing a gonadotroph adenoma. A 69-year-old man, who had just started antiandrogen treatment (Gn-RH agonist) for prostatic cancer, was admitted to neurosurgery emergency because of increasing headache and visual impairment. The CT-scan disclosed the presence of a large pituitary mass with lateral invasion of the left cavernous sinus. Hormonel testing showed panhypopituitarism. A few days later, diabetes insipidus appeared. The patient first received corticosteroid therapy and underwent surgical adenomectomy. Immunostaining of the tumor tissue was positive for FSHbeta, confirming the diagnosis of gonadotroph adenoma. Three months after surgery, the endocrine evaluation showed pituitary insufficiency. An 81-year-old man complained of mnemonic disorders. The CT-scan revealed a pituitary mass without extension. The Ophthalmological examination showed left temporal upper quadranopsia. Endocrinological tests with administration of GN-HR triggered headache and vomiting. A second CT-scan was unchanged. Hormone testing revealed increased serum levels of FSH and decreased serum levels of LH. Surgical management of the primary tumor was undertaken due to the visual field alteration. Immunohistochemical studies confirmed the diagnosis of gonadotroph FSHbeta adenoma.
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PMID:[Two cases of non-functional gonadotroph adenoma pituitary apoplexy following GnRH-agonist treatment revealing gonadotroph adenoma and pseudopituitary apoplexy after GnRH administration]. 1291 66

Pituitary apoplexy is a rare and underdiagnosed clinical syndrome. It results from hemorrhagic infarction of the pituitary gland. In its classical form it is characterized by acute headache, ophthalmoplegia, visual loss and pituitary insufficiency. Meningeal irritation signs, clinically indistinguishable from infectious meningitis, are considered rare and have not been reported as presenting signs. We report a 53-yr-old man who was admitted to hospital following acute headache, fever, neck stiffness and paresis of the left oculomotor and abducent nerves. A lumbar puncture revealed an increased number of polymorphs but with a sterile cerebral spinal fluid. Magnetic resonance imaging (MRI) showed an intrasellar mass with central necrosis in an enlarged sella. Endocrinological evaluation demonstrated insufficient thyroid, adrenocortical, and gonadal function. Necrosis within a chromophobe adenoma was found upon surgical decompression of the sella. After surgery anterior panhypopituitarism did not recover, while ophthalmoplegia subsided. The patient is now in good health under appropriate hormonal replacement therapy.
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PMID:Acute sterile meningitis as a primary manifestation of pituitary apoplexy. 1466 31

There have been several reports describing the cases of acromegaly, which show reduction in size of tumor in due to pituitary apoplexy or lymphocytic hypophysitis. We have encountered a patient of acromegaly, who developed panhypopituitarism after suffering from meningitis and showed the reduction of tumor in size. The results of imaging examination suggested the presence of pituitary apoplexy and lymphocytic hypophysitis. The patient was a 27-year-old woman, who visited a local physician with complains of headache and fever. After performing lumbar puncture, she was diagnosed as viral meningitis, and conservative therapy was initiated. The results of biochemical test of blood revealed hyponatremia. Because facial appearance of the patient was similar to that of acromegaly, endocrine dysfunction was suspected. The result of pituitary hormone tests showed high levels of growth hormone (GH) and somatomedin C (IGF-1) and low levels of the other hormones. At the same time, sign of diabetes insipidus was noted, and the patient was referred to our hospital. In the examination at the admission, GH and IGF-1 showed the trends to decrease, and the reduction in size of tumor was also detected. From the results of imaging examination, pituitary apoplexy and lymphocytic hypophysitis were suspected. Operation was performed, and pathological examination revealed inflammation of pituitary adenoma.
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PMID:[Spontaneous remission of acromegaly after meningitis: a case report]. 1472 37

We studied a series of 93 patients diagnosed with craniopharyngioma during a 15-year period with respect to presenting symptom, clinical course and management. The majority (62%) of patients were men, and had presented with neurological symptoms (75%), with headaches (82%) being the most common presenting symptom. The incidence of certain endocrine and ophthalmic symptoms varied little from that in the literature, on the other hand, the incidence of certain other symptoms did differ markedly from the literature. For example, loss of libido and amenorrhoea were seen at a much lower frequency than that stated in the literature. Hypertension, sensorimotor symptoms and urinary incontinence were not seen at all in our patients. In most cases diagnosis was made by a cranial CT scan, which is more sensitive than plain radiography for detection of enlarged sella turcica (69% vs. 24%; P < 0.001). In over 90% of cases, therapy consisted of removal of a variable portion of the tumour, with or without radiotherapy. Post-operative mortality was substantially reduced in cases treated by radiotherapy (P < 0.05). The most common post-operative complications in all cases were recurrence of disease, panhypopituitarism and diabetes insipidus.
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PMID:Presentation and outcome of 93 cases of craniopharyngioma. 1496 70

Although intrinsic pituitary lesions are the most common cause of hypopituitarism, suprasellar masses can produce similar symptoms. The differential diagnosis of a suprasellar mass includes cystic lesions, tumors, granulomatous disease, and infection. The etiology is not always obvious, and despite extensive work-up, may remain elusive. A 28-year-old Mexican man presented with complaints of headache and weakness for two weeks duration. He became increasingly lethargic and an MRI revealed a two centimeter suprasellar mass. Testing of the hypothalamic-pituitary axis suggested panhypopituitarism. He was prescribed treatment with hydrocortisone, DDAVP, and levothyroxine. Open craniotomy and biopsy of the hypothalamus revealed marked inflammation with plasma cells, histiocytes, and small lymphocytes. Light microscopy revealed macrophage-contained leishmania-like organisms although results were not immediately available. Pathological data was consistent with acute infection by Trypanasoma cruzi. Despite supportive efforts, the patient expired two months after presentation. This case illustrates the difficulty of diagnosing and the potential rapid mortality of a suprasellar mass. Because of the wide consideration of etiologies, a tissue diagnosis is needed. However, as this case illustrates, a definitive tissue diagnosis is not always possible, even following biopsy during open craniotomy.
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PMID:Acute chagas' disease presenting with a suprasellar mass and panhypopituitarism. 1576 61

We report the case of a 29-yr-old woman who first presented an aseptic meningitis at the beginning of a pregnancy. She was admitted one month later with headaches and vomiting. Panhypopituitarism with diabetes insipidus was diagnosed. Magnetic resonance imaging (MRI) data suggested the existence of lymphocytic infundibulohypophysitis, with inflammation of the suprasellar area. No new symptoms were noticed until 6 months later when this patient pointed out troubles of the visual field, due to a compression of the optic chiasma. Three boluses of 1 g methylprednisolone were prescribed, with no effects. After delivery, the defects of the visual field increased. A neurosurgical intervention was decided. Diagnosis of Rathke's cleft cyst (RCC) was made. We concluded that this patient presented a rupture of a RCC, which occurred at the beginning of pregnancy, associated later with panhypopituitarism with diabetes insipidus, due to a probable hypophysitis. The end of the pregnancy was marked by consequences of an increased volume of the RCC. To our knowledge, this case is the first described during pregnancy.
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PMID:Hypophysitis associated with a ruptured Rathke's cleft cyst in a woman, during pregnancy. 1669 3

Intrasellar arachnoid cyst (IAC) is a very rare pathological lesion occurring in 5 of 1000 autopsy cases, and constitutes 9% of all arachnoid cysts. As a space-occupying mass, IAC may cause headaches, visual disturbances, hypopituitarism, precocious puberty, and the "bobble-head doll" syndrome. The pathogenesis of IAC remains controversial. Magnetic resonance imaging (MRI) is the neurodiagnostic tool of choice to evaluate IAC. The authors presented a 38 year-old woman suffering from severe chronic headaches, dysmenorrhea, and visual disturbance. MRI revealed an intrasellar cystic lesion that had compressed the optic chiasma. Preoperative endocrinological assessment revealed hyperprolactinemia and hypogonadotropic hypogonadism. The patient underwent transsphenoidal surgery. The cyst membrane was opened and clear, serous fluid was evacuated. The postoperative course was complicated by CSF leakage, which was corrected by an autologous fat graft placement. Visual field defects improved immediately after surgery but a transient panhypopituitarism and diabetes insipidus occurred. Postoperative MRI revealed no recurrence of the lesion during the four-year follow-up.
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PMID:[Intrasellar arachnoid cyst. A case report and review of the literature]. 1696 58


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