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)

Somatostatin is a peptide synthesized in many tissues that can act as a neurotransmitter, a systemic hormone, or a local hormone, and inhibits the secretion of hormones or other cell products. A long-acting synthetic analogue of somatostatin (SMS 201-995) has been developed which when administered subcutaneously has a biologic half-life of 90 to 120 minutes and can be administered 2 or 3 times per day. SMS 201-995 can lower plasma concentrations of growth hormone and somatomedin-C in patients with pituitary acromegaly, but no controlled trials to assess symptomatic response or change in tumor size have been done. In patients with pituitary thyrotropin-producing pituitary tumors, SMS 201-995 has been remarkably effective in producing biochemical and clinical responses and is the drug of first choice in this syndrome when tumor resection is not possible. In patients with the carcinoid syndrome, SMS 201-995 effectively reduces diarrhea, is the best available drug for treatment of carcinoid flush (effective in approximately 90% of cases), and is useful in treating carcinoid crisis. Eighty-five percent of patients with pancreatic islet cell tumors that produce vasoactive intestinal peptide will respond to SMS 201-995 with a reduction in diarrhea that often has been resistant to all other therapy. SMS 201-995 may also be useful in treating the symptoms in some patients with glucagonomas, growth hormone releasing hormone-producing tumors and insulinomas. Whether SMS 201-995 has a significant effect on gut neuroendocrine tumor growth remains uncertain. Certain nonmalignant diseases of the gut respond to somatostatin, including secretory diarrhea and fistulas of unknown cause. In general, SMS 201-995 has proved safe with few significant side effects, but whether the long-term use of the drug will result in an iatrogenic form of the somatostatinoma syndrome is uncertain.
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PMID:NIH conference. Somatostatin and somatostatin analogue (SMS 201-995) in treatment of hormone-secreting tumors of the pituitary and gastrointestinal tract and non-neoplastic diseases of the gut. 253 88

This case involves a 51-year-old female. In 1983, a gastroscopic examination disclosed a submucosal tumor in the duodenum. In 1986, during follow-up observation, on her visit to hospital an early gastric carcinoma at the antrum was found. Thus, a distal gastrectomy, which entailed the complete removal of the Group 2 lymph nodes and the hepatoduodenal, intra-mesenteric lymph nodes, and Billroth I reconstructive surgery were both performed. A histopathological examination revealed that the antral lesion was a carcinoma, sig. m. ly0. v0. n(-), and that a lesion at the duodenal bulb was carcinoid, with cell that were positive to Grimelius staining and to somatostatin serotonin by an immunological staining. A metastases to the lymph nodes but only along the upper trunk of the common hepatic artery, also was found.
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PMID:[A case of duodenal carcinoid associated with early gastric carcinoma]. 253 35

We report the immunohistochemical and ultrastructural features of a case of duodenal carcinoid (somatostatinoma) combined with cutaneous-type von Recklinghausen's disease in a 65-year-old woman. The duodenal tumor located at the 2nd portion was composed of tumor cells arranged in a trabecular, glandular or nest-like pattern, occasionally associated with eosinophilic globules in the glandular structures. The tumor cells mostly showed strong immunoreactivity with anti-somatostatin antibody. Electron microscopy revealed that every tumor cell contained intracytoplasmic granules with electron-dense cores, 100-300 nm in size, in varying numbers, and intracytoplasmic lumina were rarely detected. The present case including identical cases reported so far suggest that the disease entity is likely to be a combination of duodenal somatostatinoma and von Recklinghausen's disease and/or phaeochromocytoma. This is the tenth case of duodenal carcinoid associated with von Recklinghausen's disease and/or phaeochromocytoma in the world literature and the third case reported as duodenal somatostatinoma.
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PMID:Duodenal carcinoid (somatostatinoma) combined with von Recklinghausen's disease. A case report and review of the literature. 254 98

A patient with ectopic Cushing's syndrome secondary to a malignant thymic carcinoid tumour was treated with the somatostatin analogue SMS 201-995. The administration of the drug subcutaneously in increasing doses over a 34 day period failed to control the ACTH or glucocorticoid excess.
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PMID:Failure of somatostatin analogue SMS 201-995 to control Cushing's syndrome due to ectopic release of ACTH from a bronchial carcinoid. 255 18

Various endocrine-related tumours contain large numbers of high-affinity somatostatin receptors. 123I-labelled tyr-3-octreotide (tyr-3-SMS 201-995, a synthetic derivative of somatostatin) was used to localise such tumours in vivo with a gamma-camera. Positive scans were obtained for two meningiomas, two gastrinomas, and one carcinoid; negative scans were obtained for one insulinoma (in which unlabelled octreotide had no effect on insulin levels), one phaeochromocytoma, one adrenal carcinoma (octreotide had no effect on cortisol levels), and three medullary thyroid carcinomas (octreotide had no effect on calcitonin levels). Thus radioiodinated tyr-3-octreotide can label somatostatin receptors in endocrine-related tumours in vivo and can therefore be used for tumour localisation.
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PMID:Localisation of endocrine-related tumours with radioiodinated analogue of somatostatin. 256 13

We report a case of a 29-yr-old man with a rectal carcinoid that metastasized to the liver, secreting somatostatin. The patient first presented with an abdominal mass and mild diabetes, and, subsequently, rectal tumor and hypersomatostatinemia. Microscopic examination of the rectal tumor showed characteristic features of carcinoid. Multiple liver metastases were recognized. The plasma level of somatostatin was remarkably high (2,839 pg/ml). Plasma gut glucagon and pancreatic polypeptide (PP) increased with progress of the disease. He died of hepatic failure 20 months after his first admission. Immunoperoxidase staining showed that rectal tumor cells contained somatostatin-like-immunoreactivity. Electron microscopy of tumor tissue showed neurosecretory (D-cell) granules. Somatostatin content of the rectal tumor was very high (5,629 pg/mg).
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PMID:Somatostatinoma of the rectum. 257 69

Five patients with external pancreatic fistulas were treated with a synthetic peptide that mimics the action of somatostatin (Sandostatin, Sandoz; East Hanover, NJ). Four of the patients developed fistulas after drainage of pancreatic pseudocysts and one developed a fistula following resection of a pancreatic carcinoid. One day after initiation of therapy, the mean fistula output for the group decreased by 52 per cent. By three days, fistula output decreased by 70 per cent. All fistulas closed in 7 to 44 days. Adverse reactions to the drug included diarrhea in one patient and transient hyperglycemia in another. Sandostatin is effective in decreasing drainage from external pancreatic fistulas and may, therefore, facilitate their closure. No serious adverse reactions to the drug were noted. Further studies will better define the role of Sandostatin in the treatment of pancreatic fistulas.
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PMID:Treatment of external pancreatic fistulas with somatostatin. Second place winner: Conrad Jobst award. 257 57

We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).
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PMID:Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin. 257 47

SMS 201-995 (Sandoz Pharmaceuticals, East Hanover, NJ) is a synthetic peptide analog of native somatostatin that has been used to relieve symptoms caused by neuroendocrine tumors. Reports have described an insulin suppressive effect of SMS 201-995 that results in elevations of blood glucose. We report a patient with a metastatic small bowel carcinoid and renal failure in whom mild symptomatic hypoglycemia occurred 30 to 60 minutes after SMS 201-995 administration. No increase in insulin or decreases in glucagon, cortisol, or catecholamines were observed during these hypoglycemic episodes. Elevated levels of growth hormone fell gradually following SMS 201-995 administration and did not temporally correspond to the 30- to 60-minute nadir of blood glucose. However, SMS 201-995 levels peaked during this 30- to 60-minute period. As clinical experience with this drug broadens, patients whose glucose control is dependent on counter-regulatory hormones should be monitored for the possibility of hypoglycemia.
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PMID:Hypoglycemia after administration of somatostatin analog (SMS 201-995) in metastatic carcinoid. 260 30

The increased knowledge of the pathobiology of gastrointestinal carcinoid (neuroendocrine) tumours and the improved therapeutic possibilities have brought a demand for more precise diagnosis. Although the carcinoid tumours can often be tentatively recognized in routinely processed microscopic slides, their more accurate identification requires additional diagnostic procedures. General neuroendocrine markers such as the argyrophil reaction of Grimelius and immunohistochemistry with application of antibodies against chromogranin A and of neuron-specific enolase are discriminatory staining methods which are used to reveal the neuroendocrine origin of almost all highly differentiated carcinoid tumours of the gastrointestinal tract. Mid-gut carcinoids, which predominate among these tumours almost unexceptionally contain serotonin. This biogenic amine can be demonstrated by the argentaffin reaction of Masson, serotonin immunoreactively or by formalin-induced fluorescence. The characteristic staining pattern of mid-gut carcinoids is almost invariably preserved in the metastatic deposits and consequently the staining methods for identifying serotonin can also be used on metastases to reveal a primary mid-gut carcinoid. The enterochromaffin-like (ECL) cell carcinoids of the body and fundic area of the stomach often seen in association with pernicious anaemia are argyrophil with the Sevier-Munger silver stain. Other neuroendocrine tumours, viz. antral, duodenal and rectal carcinoids should be studied by a battery of relevant peptide hormone antisera for adequate diagnosis. During the last decade new peptide hormones have been found in circulation in patients with carcinoid tumours, but serotonin and urinary 5-HIAA are still the most important markers for carcinoids of the mid-gut origin. Other clinically useful tumour markers are chromogranin A + B, pancreatic polypeptide, human chorionic gonadotropin alpha and beta subunits. For localizing procedures, angiography is the most reliable investigative method for primary tumours in the gut, whereas CT-scan and ultrasound investigations are good for detection of liver metastases. During the last five years, the therapy for malignant carcinoid tumours has been considerably improved. Chemotherapy has only revealed objective response rates in about 10-30% of the patients giving median survivals from start of therapy of about 10 months. Recently treatment with alpha interferons and the new somatostatin analogue octreotide have given objective responses in 50-75% of patients with malignant mid-gut carcinoid tumours. These patients have now a median survival from start of therapy of 70 months when treated with alpha interferons. In the future new therapies will come into use such as monoclonal antibodies and perhaps also agents blocking different growth factors.
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PMID:Gastrointestinal carcinoid tumours. Histogenetic, histochemical, immunohistochemical, clinical and therapeutic aspects. 266 60


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