Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P61278 (somatostatin)
 22,083 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes the concomitant occurrence of a somatostatin-rich duodenal carcinoid, a medullary thyroid carcinoma and a diffuse adrenal medullary hyperplasia in a patient with von Recklinghausen's disease. A 50-year-old Japanese man died from lung metastasis of a malignant schwannoma. In addition to extensive viscero-cutaneous neurofibromatosis, two different types of neuroendocrine tumors were found in the duodenum and thyroid gland at autopsy. The duodenal tumor, which was located in the second portion, showed the histologic appearance of a carcinoid tumor with glandular differentiation and psammoma-bodies. Immunohistochemically the tumor cells were intensely positive for somatostatin. The thyroid tumor was composed of nests of tumor cells arranged in an endocrine pattern, and showed immunoreactivity for calcitonin. A review of the literature revealed no previously reported case of concomitant occurrence of duodenal somatostatinoma and medullary thyroid carcinoma in a single patient with von Recklinghausen's disease. Morphometric analysis of adrenal glands disclosed the presence of diffuse medullary hyperplasia. Thus, the present case exhibited a similarity in some respects with multiple endocrine neoplasia (MEN) syndrome, Type IIa or IIb.
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PMID:von Recklinghausen's disease associated with somatostatin-rich duodenal carcinoid (somatostatinoma), medullary thyroid carcinoma and diffuse adrenal medullary hyperplasia. 168 37

The glandular peripheral nerve sheath tumor is a rare variant of nerve sheath neoplasms in which the focally occurring glands are lined by cells showing divergent differentiation. The vast majority of the reported nerve sheath tumors harboring these glands have been malignant. We herein present a case of benign glandular peripheral nerve sheath tumor in a 43-year-old woman who had no evidence of von Recklinghausen's disease. Histologically, the tumor is composed of spindle cell component and collections of glandular component. The glandular component occupied the central two-thirds of the lesion and was lined by a single layer of nonciliated cuboidal or columnar cells. No mitotic figures were recognized in the spindle cell area. This spindle cell area had neurofibroma-like features rather than schwannoma. Many of the spindle cells had positive reaction products for S-100 protein. The glandular lining epithelium were positive for cytokeratins (CAM 5.2, AE1/AE3, PKK1) and EMA. Some epithelial cells were immunoreactive for CEA, chromogranin, somatostatin and Leu-7. These immunohistochemical findings support the neuroendocrine differentiation of the epithelial element from the schwannian component.
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PMID:Benign glandular peripheral nerve sheath tumor. A case report. 752 35

Among all the neurogenic tumours, the benign intrathoracic schwannoma is a rare tumour. Most often it is discovered by accident in the course of a routine X-ray examination of the thorax. Symptoms appear only when size becomes important. The surgical removal of an intrathoracic schwannoma may result in the development of a chylothorax. According to the literature, its treatment remains controversial. The originality of the case we report here is on the one hand the exceptional size of the tumour and the success over the long term of conservative treatment with complete parenteral alimentation, and on the other hand, the inefficacy of the subcutaneous administration of the analogue of somatostatin in reducing a chylothorax with high outflow.
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PMID:Benign thoracic schwannoma and postoperative chylothorax: case report and review of the literature. 801 50

We recently demonstrated that cultured malignant schwannoma (MS)-derived cells can support human skin mast cell (HSMC) survival in vitro. Cultured HSMCs were spindleshaped in close contract with MS-derived cells, suggesting cell to cell interaction. To elucidate the mechanism of the enhanced HSMC survival in coculture with MS-derived cells and the cellular interactions between HSMC and MS-derived cells, we examined the immunocytochemical characteristics of MS-derived cells using immunofluorescence. Morphologically, cultured MS-derived cells were polygonal with abundant cytoplasm and resembled perineurial cells. The cultured cells immunoreacted positively with vimentin, fibronectin, laminin and collagen IV, but negatively with anti-S100 protein, anti-neuron specific enolase, and anti-neurofilament (68 kd, 145 kd, 200 kd) antibodies. MS-derived cells were distinct from Schwann cells in their lack of S100 protein and also distinguishable from endoneurial fibroblasts that produce fibronectin, but never expressed laminin or collagen IV. MS-derived cells thus possess the characteristics of perineurial cells in their general morphology and their immunocytochemical properties. Immunoreactivity for substance P (SP) and neurokinin A (NKA) was found in the cytoplasm of these cells, particularly around the nuclei. Vasoactive intestinal peptide, somatostatin, and calcitonin gene related peptide were negative. From these findings, we characterized the MS-derived cell's in vitro properties and concluded that it is similar to a perineurial cell. The extracellular matrix protein, laminin, and fibronectin expressed in the MS-derived cell might contribute to HSMC survival and morphology through cell to matrix adhesion. Neuropeptides such as SP and NKA, expressed in the MS-derived cell, might play some role in enhanced HSMC survival in vitro.
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PMID:Immunocytochemical characterization of malignant schwannoma-derived cells in culture. 904 33