Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
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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

We report the morphological characteristics of 30 cases of sclerosing hemangioma (SH) of the lung and explore the histological origin of the major cells in these tumors. In addition to routine light and electron microscopy, immunohistochemistry was performed by using 12 monoclonal primary and 5 polyclonal primary antibodies. These included surfactant protein B (SP-B), thyroid transcription factor-1 (TTF-1), mast cell trypsin, CD68, epithelial antigen markers (high molecular weight cytokeratin, low molecular weight cytokeratin [CK-L], epithelial membrane antigen [EMA], cancer embryonic antigen), mesothelial antigen, neuroendocrine markers (neuron-specific enolase [NSE], chromogranin A, synaptophysin, calcitonin, adrenocorticotropic hormone, human growth hormone [hHG]), vimentin, and CD34. Surface cuboidal cells have short microvilli and have lamellar bodies in their cytoplasm. They can sometimes merge into multinuclear giant cells. Immunohistochemical results showed that these cells are strongly positive for SP-B, TTF-1, CK-L, EMA, and cancer embryonic antigen, whereas polygonal cells, previously also described as round or pale cells, were strongly positive for vimentin and TTF-1, and positive or weakly positive for 2 to 3 kinds of neuroendocrine markers. Sparse neuroendocrine granules and abundant microfilaments were observed in their cytoplasm. Some cell clusters in the solid regions were positive for SP-B and EMA. Mast cells existed sparsely in almost every field. Both cuboidal and polygonal cells were negative to CD34 and mesothelial antigen staining. We conclude that cuboidal cells of SH originate from reactive proliferating type II pneumocytes, which can fuse into multinuclear giant cells. Polygonal cells, as true tumor cells, likely originate from multipotential primitive respiratory epithelium and possess the capability for multipotential differentiation. The antibodies of SP-B, TTF-1, vimentin, and CK-L are very helpful to diagnosis and differential diagnosis of SH.
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PMID:Immunohistochemical and ultrastructural markers suggest different origins for cuboidal and polygonal cells in pulmonary sclerosing hemangioma. 1511 33

A 13-month-old Korean female presented with Cushing disease and diabetes insipidus. On MRI, a 3.5-cm, focally cystic, contrast-enhancing, sellar and suprasellar mass was noted. Aside from blood adrenocorticotropin (ACTH) and cortisol elevation, other pituitary hormone blood levels were normal or markedly reduced. The subtotally resected lesion consisted of synaptophysin-immunoreactive lobules of (a) large, polygonal, amphophilic, PAS-positive cells immunoreactive for ACTH, beta-endorphin, alpha melanocyte stimulating hormone (MSH), and keratin (CAM5.2) in some cells showing Crooke hyaline change, (b) less frequent acidophilic, growth hormone (GH) immunoreactive cells, and (c) rare luteinizing hormone (LH) and/or alpha subunit immunopositive cells. Also conspicuous were smaller cells resembling Rathke-type epithelium forming rosettes to sizable glands immunoreactive for EMA, keratin, S-100 protein, galectin-3 and rarely for synaptophysin and/or one of the above-noted adenohypophysial hormones. Transcription factors, including Neuro-D1 and Pit-1, were present in ACTH- and GH-producing cells, respectively, but only in occasional Rathke-type cells. The MIB-1 labeling index (LI) was 1.5% in secretory cells and 39% in Rathke-type epithelium. Ultrastructurally, the tissue resembled fetal pituitary of 10-12 weeks gestation and contained fully differentiated corticotrophs and somatotrophs, scant cells of glycoprotein-hormone producing type with small secretory granules, and glandular epithelial cells consistent with committed, but largely undifferentiated Rathke-type epithelium. We consider the tumor as a pituitary blastoma, a lesion composed of multiple cell types common to the development of the affected organ based upon (a) prominence of primitive Rathke-type epithelium, (b) disposition of secretory cells in lobules rather than acini, (c) the limited range of secretory cells represented, (d) the presence of their corresponding transcription factors, and (e) ultrastructural features indicating orderly development of the 10- to 12-week embryonic stage.
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PMID:Pituitary blastoma. 1855 Dec 99