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)

A 43-yr-old-man with metastatic VIPoma in whom the conventional measures of surgery, chemotheraphy, and hepatic artery embolization ultimately failed to control his severe diarrhea, resulting from vasoactive intestinal polypeptide hypersecretion, was treated with a new long-acting somatostatin analogue, SMS 201-995, for 14 mo. SMS 201-995 not only controlled the diarrhea without side effects but appeared to have possibly induced a reduction in metastatic tumor size.
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PMID:Long-term treatment of a VIPoma with somatostatin analogue resulting in remission of symptoms and possible shrinkage of metastases. 285 77

Since the description of the watery diarrhea syndrome by Verner and Morrison 29 years ago, clinical and experimental observations have elucidated the pathophysiology of this disease. Vasoactive intestinal polypeptide (VIP) is produced and released by a tumor of the pancreatic islets or by a tumor of neural crest origin such as a ganglioneuroma. Under normal conditions, current evidence suggests that VIP is a neurotransmitter in the central and peripheral nervous systems and particularly in the peptidergic nervous system. The low VIP plasma concentration observed in healthy subjects is viewed as a neuronal overflow since it has been impossible to ascertain any endocrine role for circulating VIP. Markedly elevated VIP plasma levels in the VIPoma syndrome lead to intestinal secretion with severe secretory diarrhea, resulting in hypovolemia, hypokalemia, and acidosis. These symptoms subside after successful tumor removal. Approximately 50 percent of patients have metastatic spread at the time of diagnosis. For these patients, a new and promising therapeutic modality is available in the form of a subcutaneously administered somatostatin analogue that relieves symptoms through potent inhibition of VIP release from tumor tissue.
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PMID:VIPoma syndrome. 303 22

Two cases of neuroendocrine tumor in the liver, positive for VIP, without evidence of a primary tumor outside the liver is presented. One patient had a VIPoma syndrome with diarrhea, hypokalemia, and hypercalcemia, all symptoms were reversible after treatment consisting of somatostatin analogue and arterial liver embolization followed by liver resection. The other patient showed no endocrine symptoms. To the best of our knowledge, VIPomas apparently primary in the liver have not been previously described.
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PMID:Liver VIPoma: report of two cases and literature review. 795 Aug 21

It was the aim of the present study to examine whether 111In-pentetreotide, a somatostatin analogue with predominantly renal excretion, is a suitable receptor agonist for scintigraphic imaging of endocrine gastro-entero-pancreatic (GEP) tumors, and to evaluate the contribution of the usual imaging times 4 and 24 h p.i. In 36 patients, planar scintigrams obtained 4 h, and 24 h after i.v. injection of 111 or 222 MBq 111In-pentetreotide were compared to the results of other imaging procedures and of surgery. Single photon emission computed tomography (SPECT) was also performed 24 h p.i. Positive scintigraphies were obtained in 32 out of 36 patients (18/19 patients with carcinoid syndrome, 8/9 with non hormone-producing endocrine GEP tumors, 2/4 with gastrinomas, 1/1 with glucagonoma, 1/1 with a VIPoma, 2/2 with paragangliomas). In 9 patients tumor manifestations previously not detected by conventional imaging procedures were disclosed by 111In-pentetreotide scintigraphy. 24-h images yielded significantly more true positive findings than 4-h images. In 4 patients liver metastases missed on planar scans were detected by SPECT. A discrepancy between patient-based and organ-based analysis of the results was encountered thus indicating a possible intraindividual heterogeneity in somatostatin receptor expression. In conclusion, 111In-pentetreotide is a suitable somatostatin analogue for scintigraphic in vivo demonstration of somatostatin receptors and for imaging of most tumor manifestations in patients with endocrine GEP tumors. Further studies will have to evaluate whether or not a positive receptor scintigraphy predicts response to treatment with long-acting somatostatin analogues.
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PMID:111In-pentetreotide (somatostatin analogue) scintigraphy as an imaging procedure for endocrine gastro-entero-pancreatic tumors. 797 60

A case report is presented of a man with Verner-Morrison syndrome of extreme severity, caused by an unresectable pancreatic VIPoma. The pathological role of vasoactive intestinal polypeptide (VIP) is discussed in the pathogenesis of Watery Diarrhoea, Hypokalaemia, Achlorhydria (WDHA) syndrome. The authors describe the typical symptoms of the syndrome and provide a diagnostic and therapeutic strategy. Plasma level of VIP was determined by the authors' own VIP RIA method. Administration of a long acting somatostatin analogue, octreotide (Sandostatin, Sandoz) at a dose of 100 micrograms daily, decreased the plasma level of VIP from about 55 to 38 fmol/ml, which was associated with complete regression of the diarrhoea. Due to the 'escape phenomenon' the dose of Sandostatin was gradually increased and finally completed with streptozotocin (Zanosar, Upjohn) administration, which was repeated every 8 weeks. The combination of Sandostatin and streptozotocin resulted in complete regression of diarrhoea and substantial diminution of the tumour mass. The patient displayed a weight gain and returned to normal life.
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PMID:[Diagnosis of Verner-Morrison syndrome (VDHA) and its treatment with sandostatin an streptozocitin]. 824 22

The evolution of gastrointestinal endocrinology has led to the design and application of analogs of gut peptides to treat disease. Octreotide, a long-acting analog of the inhibitory peptide somatostatin, has proven useful in the management of disorders such as carcinoid syndrome and secretory diarrhea due to VIPoma. More recent experience suggests a role for this peptide in the management of certain complications of gastrointestinal surgery. Prophylactic use of octreotide appears warranted in the prevention of carcinoid crisis in selected patients with carcinoid syndrome undergoing invasive procedures, and in the prevention of complications in selected patients undergoing pancreatic surgery. Evidence from placebo-controlled trials supports a role for octreotide in the management of dumping symptoms in severely affected patients, at least in the short term. Octreotide appears to serve a useful adjunctive role in controlling output from postoperative gastrointestinal fistulae and may hasten closure, particularly in pancreatic fistulae. Selected patients with ileostomy diarrhea and short bowel syndrome benefit from octreotide treatment, but the long-term value of the peptide in controlling stool output is less clear. Rare patients with other forms of postoperative secretory diarrhea have been successfully treated with octreotide. Finally, animal and early human experience suggests that octreotide may have a role as an adjunctive treatment of partial small bowel obstruction. In most of these conditions, the available data is sparse and further controlled trials are warranted.
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PMID:Perioperative use of octreotide in gastrointestinal surgery. 835 66

The purpose of the workshop was to critically evaluate the use of octreotide in the management of important surgical and gastroenterological conditions. The topics covered included: (1) management of functioning gut neuroendocrine tumors, (2) new approaches to localize these tumors, (3) the place of octreotide in the treatment of variceal bleedings, and (4) the use of octreotide in postoperative conditions. Octreotide therapy has been shown to be effective in the carcinoid syndrome, in which symptom control is achieved in 85% of patients, and reduction in 5-HIAA in 60%. Although tumor regression is rarely seen, prolongation of survival probably occurs. Control of diarrhea has been achieved in 84% of patients with VIPoma treated with octreotide. Similarly, octreotide has been found to provide effective control of the necrolytic, migratory dermatitis seen in glucagonoma. By contrast, insulinomas are more resistant to somatostatin agonist therapy. In the Zollinger-Ellison syndrome, octreotide is effective in alleviating symptoms and in reducing serum gastrin levels. However, its use in this syndrome has been superceded by omeprazole. Radioiodine-labelled octreotide has been very effective in in vivo imaging of neuroendocrine tumors in the abdomen, and is now considered the best available technique for localizing these tumors preoperatively. Intraoperative localization with a hand-held gamma camera is being developed. There is an exciting future possibility to use the technique to deliver therapy to tumors. Octreotide therapy has been shown to be at least as effective as and without the adverse hemodynamic effects of Pitressin in control of variceal hemorrhage. It should be regarded as one of several modalities of therapy in the condition.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Somatostatin analogue therapy in functioning neuroendocrine gut tumors. 835 71

Gastrointestinal neuroendocrine tumors are slowly growing and metastases are often limited to the liver. As a result of their favorable biological behavior these tumors have a relatively good prognosis even in metastatic stage. Due to a variety of therapeutic options patients with malignant neuroendocrine tumors may survive for extended periods of time up to ten years. Often a combination of different treatments and also alternation between the different therapeutic regimes is needed. A patient with excessive WDHA-syndrome and severe metabolic disturbances due to a pancreatic VIPoma with metastatic spread into the liver and abundant hormonal secretion is presented. Cytotoxic agents (streptozocin, 5-fluorouracil and adriamycin) were able to alleviate clinical symptoms and to control tumor growth for six years. Analogues of somatostatin (octreotide) and interferon alpha had been very useful in controlling clinical symptoms and tumor progress for 18 months. Cytotoxic agents or octreotide were not able, however, to achieve any permanent cure. Eventually, treatment failure occurred with dramatic progression of symptoms and tumor growth, unresponsive to any medical therapy. Consequently, total hepatectomy and liver transplantation together with extirpation of the pancreatic primary tumor was performed and succeeded in providing a normal life to the patient. In our opinion the overall outcome of patients with metastatic VIPoma may be improved best by maintaining the patients on medical therapy until treatment failure occurs. In case of extended hepatic metastases orthotopic liver transplantation might be considered for patients with symptomatic disease who no longer respond to conventional treatment modalities.
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PMID:Metastatic pancreatic VIPoma: deteriorating clinical course and successful treatment by liver transplantation. 957 8

We report the first case of metastatic Vipoma treated by orthotopic liver transplantation. A woman with explosive secretory diarrhea causing hypokalemic acidosis was diagnosed as having a vasoactive intestinal peptide secreting tumor with widespread hepatic metastases. The symptoms were initially controlled for 9 months with increasing doses of long-acting somatostatin analogue (Sandostatin, Sandoz Ltd, UK). Alpha interferon was not tolerated, causing an acute paranoid psychosis. Eventually orthotopic liver transplantation was performed with the removal of the primary tumor from the distal pancreas. Postoperatively, complications were associated with the distal pancreatectomy. The patient has no evidence of tumor recurrence on imaging or serum VIP level 12 months posttransplantation.
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PMID:Treatment of metastatic Vipoma by liver transplantation. 1015 88

The VIPoma syndrome is rare. It is usually caused by a neuroendocrine tumor located in the pancreas. Somatostatin analogs and interferon-a can be helpful in the symptomatic control of the disease, but the efficacy of chemotherapy in metastatic disease is limited. We report the case of a 32-yr-old patient who had a primary intestinal VIPoma with peritoneal carcinomatosis and hepatic metastases. Somatostatin analogs and conventional chemotherapy regimens were not effective on VIPoma syndrome and tumor progression. The combination of 5- fluorouracil and interferon-alpha was associated with a major clinical improvement and tumor regression. Further investigations should evaluate the place of such a combination as a first line treatment for patients with metastatic neuroendocrine tumors.
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PMID:Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil. 1063


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