Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine cases of symptomatic Rathke's cleft cyst are reported. Their most frequent signs and symptoms included headache, chiasmal syndrome and hypopituitarism, while one of the cases developed a sudden onset of headache and vomiting following diabetes insipidus. Endocrinological findings showed a decreased ACTH, gonadotropin and growth hormone more frequently while there were 2 cases of hyperprolactinemia and 1 case of diabetes insipidus. In a neuroradiological examination, a plain skull X-ray showed 5 cases of ballooning of the sella turcica, and a CT scan demonstrated a low to high density of the cyst and 2 cases of marginal enhancement of the cyst. MRI mostly demonstrated a well delineated mass at the sella extending mostly into the suprasellar region and a low to high intensity of the cyst in the T1-weighted image. Two cases were marginally enhanced after gadolinium DTPA administration. The pathological examination, done on 6 cases, showed either single or multiple layers of the epithelium which were mostly ciliated. The epithelium was positive in PAS and Alcian blue in all cases and a histochemical examination showed 3 cases to be positive in EMA and 2 cases positive in CEA. A resection of the cyst wall and an opening of the cyst is thus recommended in symptomatic cases. Therefore, the transsphenoidal approach should be the choice of treatment in an intra- and suprasellar extension of the cysts with sellar enlargement.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Symptomatic Rathke's cleft cyst: a clinicopathologic study of 9 cases]. 816 47

Pituicytoma is a rare neoplasm occurring in the sellar and suprasellar regions. The tumor is currently considered to arise from the pituicyte, a specialized glial cell of the posterior lobe and the stalk of the pituitary gland. The authors describe a case of pituicytoma, focusing on the ultrastructural features. The patient was a 79-year-old woman with hypopituitarism and visual disturbances. Immunohistochemically, the tumor showed strong and diffuse positivity for vimentin, and EMA and GFAP were focally detectable. Ultrastructurally, the lesion was composed of elongated cells containing in the Golgi area aggregates of intermediate filaments in a concentric pathway (fibrous body), and secretory granules. These features intermediate between a pituicytoma and a pituitary adenoma suggest that "pituicytomas" may also arise from the specialized stromal folliculo-stellate cells of the adenohypophysis, which are able to differentiate into endocrine cells.
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PMID:Pituicytoma: ultrastructural evidence of a possible origin from folliculo-stellate cells of the adenohypophysis. 1157 76