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)

Familial occurrence of malignant carcinoid is rare (about 3%). Authors describe occurrence of the malignant carcinoid in two brothers. In the older one the diagnosis was estimated in 1991. He had multiple intestinal carcinoid tumor with multiple liver metastases histological type III by Soga classification. Patient is intermittently treated with somatostatin analogue--lanreotid and with interferon alfa. By this therapy the disease is stabile. In the younger of brothers the diagnosis was estimated in 1999. The disease had rapid progression and in ten months patient died despite of the therapy. Definitive diagnosis was a malignant neuroendocrine tumor of pancreas-mixed low differentiated carcinoid with calcitonin overproduction. (Fig. 4, Ref. 15.)
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PMID:Malignant carcinoid in two brothers. 1172 74

Carcinoids are rare tumors of enterochromaffin cells. The carcinoid-syndrome most often occurs with hepatic metastases of carcinoids and is evoked by release of serotonin and other vasoactive substances, leading to typical symptoms such as hyper- or hypotension, bronchospasm, tachycardia, diarrhoe, and flushing. A lethal perioperative "carcinoid-crisis" may occur. We report on a patient with carcinoid-syndrome due to liver metastases undergoing hemihepatectomy. For prophylaxis, the patient preoperatively received H 1- and H 2-histamine-receptor antagonists, corticosteroids, and a continuous somatostatin infusion. Besides monitoring cardiovascular variables we intermittently measured serotonin- and catecholamine concentrations. Initially increased serotonin concentration decreased during the course of anaesthesia. However, it increased again during liver resection despite Pringle's manoeuvre and was associated with a decrease in arterial pressure, systemic vascular resistance, and central venous pressure. Hypotension was treated by volume and noradrenaline infusion. Thus, despite somatostatin infusion serotonin release is still possible, especially during surgical manipulation.
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PMID:[Anaesthetic management for hemihepatectomy in a patient with carcinoid-syndrome]. 1174 74

Motilin-secreting neuroendocrine tumours have been rarely described. Immunohistochemical, biochemical and motility investigations were performed in a 62-year-old man with liver and bone metastases of a motilin-secreting neuroendocrine tumour originating from a rectal polyp removed 14 years previously. Symptoms related to liver metastases were reduced by a right hepatectomy whereas plasma motilin levels were decreased. The patient also underwent two operations for spinal cord decompression and survived 6 more years under medical treatment, mainly octreotide. Immunohistochemistry revealed predominant expression of motilin-containing cells, with rare cells expressing somatostatin and pancreatic polypeptide, and staining for only one panendocrine marker, neurone-specific enolase. A liver tumour extract contained 17.9 microg motilin per gram of tissue, which permitted to isolate and characterize human motilin, which was identical to porcine motilin. Plasma column gel chromatography revealed a main peak corresponding apparently to porcine motilin. The patient had no symptoms of disturbed motility. Gastric emptying and gastroduodenojejunal motility were found within normal limits. The absence of alterations of gut motility was perhaps related to sustained autonomous motilin production. The long evolution of this type of tumour suggests that plasma motilin determination should be added to the investigations for neuroendocrine tumours.
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PMID:An unusual metastatic motilin-secreting neuroendocrine tumour with a 20-year survival. Pathological, biochemical and motility features. 1184 83

An 18-year-old male presented in 1979 with a gastrinoma of unknown primary origin. Massive upper-digestive haemorrhage led to total gastrectomy, at which histology evidenced liver metastases, confirmed 9 months later at reoperation for an intestinal occlusion. Postoperative morphological evidence of liver metastases was repeatedly negative using abdominal ultrasound and computerized tomography (CT) scans and magnetic resonance imaging (MRI), but a recent somatostatin-receptor-specific scintigraphy (Octreoscan) was positive only in the liver area. Twenty-two years after diagnosis, the primary tumour has not been identified, the patient leads a normal life, and his circulating gastrin levels, although still elevated at 317-550 pg/ml (normal < 127 pg/ml), have fallen over recent years from > 1000 pg/ml. We discuss the relevance of the described prognostic factors.
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PMID:Twenty-two years' survival of metastatic gastrinoma evidenced recently by somatostatin-receptor-specific scintigraphy. 1195 3

We have evaluated survival and tumor-related symptoms in the presence of mesenteric lymph node and liver metastases in relation to surgical procedures in 314 patients (148 women, mean age at diagnosis 61 years; 249 with liver metastases) treated for midgut carcinoid tumors. Of the operated patients, 46% presented with severe abdominal pain and intestinal obstruction and were operated on before the diagnosis. Medical treatment (somatostatin analogs, interferon-a) was initiated in 67% and 86%, respectively. Surgical attempts included small intestine or ileocecal/right-sided colon resection with excision of mesenteric lymph node metastases. Most of the patients (n = 286) had mesenteric lymph node metastases; 33% of them had unresectable mesenteric lymph node metastases and underwent surgery without mesenteric dissection. Patients who underwent resection for the primary tumor had a longer survival than those with no resection (median survival 7.4 vs. 4.0 years; p <0.01). Patients who underwent successful excision of mesenteric metastases had a significantly longer survival than those with remaining lymph node metastases. Patients operated on for a primary tumor but with remaining lymph nodes but no liver metastases and who subsequently received interferon and somatostatin analog treatment had a median survival of 7.4 years. Resection of the primary tumor and the mesenteric lymph node metastases led to a significant reduction in tumor-related symptoms. Surgery to remove the primary intestinal tumor including mesenteric lymph node metastases is supported by the present results, even in the presence of liver metastases. Liver metastases and significant preoperative weight loss are identified as major negative prognostic factors for survival.
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PMID:Effect of surgery on the outcome of midgut carcinoid disease with lymph node and liver metastases. 1201 80

Patients with clinically evident medullary thyroid cancer should have a total extracapsular thyroidectomy with bilateral central neck dissection and an ipsilateral prophylactic or therapeutic modified (functional) radical neck dissection when the primary tumor is greater than 1 cm and when the central neck nodes are positive. A prophylactic contralateral neck dissection should be done when the primary tumor is bilateral and when there is extensive lymphadenopathy on the side of the primary tumor. Patients who have gross, unresectable residual medullary thyroid cancer should receive postoperative external radiotherapy. Patients who are carriers of germ-line RET proto-oncogene point mutations or have an elevated (basal or stimulated) calcitonin levels on screening should have a prophylactic total thyroidectomy before age 6 years. In patients with an elevated basal or stimulated plasma calcitonin level and an intrathyroidal nodule on ultrasound, a total thyroidectomy and central neck lymph node dissection should be done. Patients with persistent or recurrent medullary thyroid cancer should have a complete thyroidectomy (if not done initially) and bilateral central and modified radical neck dissection, including upper mediastinal lymphadenectomy. Patients who are symptomatic from distant medullary thyroid cancer metastases (diarrhea, flushing, weight loss, or bone pain) should be treated with somatostatin analogs. Bone metastases should be resected if possible, and symptomatic lesions that are unresectable should be treated with external radiotherapy. Cytoreductive procedures such as radiofrequency ablation or cryoablation for liver metastases should be considered in symptomatic patients to reduce tumor burden. Localized pulmonary metastases should be resected. Chemotherapy or radioactive immunotherapy (iodine 131 labeled carcinoembryonic antigen monoclonal antibody) protocols should be considered in patients with nonoperative widely metastatic progressing medullary thyroid cancer.
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PMID:Medullary thyroid cancer. 1205 61

The aim of this study was to investigate the role and function of somatostatin, glucagon and gastrin in 35 colorectal adenocarcinoma and 25 liver metastases. The investigation was carried out at the electron immunocytochemistry level. Our results showed that well-differentiated tissues have the highest somatostatin levels compared to poorly-differentiated tumors while glucagon levels remain high. We also found gastrin immunoreactivity in cell membranes of poorly-differentiated primary adenocarcinoma tissues and in liver metastases. Low somatostatin expression, high glucagon expression and the presence of gastrin were found in tumors of poor grading and therefore poor prognosis.
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PMID:Immunoelectron study of somatostatin, gastrin and glucagon in human colorectal adenocarcinomas and liver metastases. 1217 92

Carcinoid tumours are relatively rare neoplasms that often present as diagnostic dilemmas due to obscure or non-specific symptomatology. The ability of carcinoid tumours to cause clinical symptoms by secretion of hormones or biogenic amines is best recognised in the form of the carcinoid syndrome. Although generally slow growing, a significant proportion demonstrates aggressive tumour growth and may be difficult to manage. Ten percent of carcinoids are part of the spectrum of multiple endocrine neoplasia type 1, which should be considered in the investigation and management of these patients. A further 10% of carcinoid tumours are associated with other noncarcinoid tumours of various histological types. This review discusses recent improvements in biochemical diagnosis with the introduction of plasma chromogranin A, and puts into context the use of the imaging modalities, including Indium-111 Octreotide scintigraphy, and newer developing techniques, such as positron emission tomography. The therapeutic options are reviewed, including the use of somatostatin analogues as the treatment of choice in the control of hormone-mediated symptoms, the role of surgery, the use of chemotherapy, biotherapy using interferon, and the exciting new field of receptor-targeted therapy. In addition, the challenging interventional management of liver metastases is discussed, including the role of hepatic-artery embolisation and chemo-embolisation, radiofrequency ablation and the place of orthotopic liver transplantation in selected patients. We conclude that with the increasing number of investigative procedures and therapeutic options available to diagnose and treat carcinoid tumours, a multidisciplinary approach is needed. Furthermore, additional scientific research and controlled clinical trials are needed to determine the efficacy of the many treatment options, which for these rare tumours can only be achieved by collaboration.
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PMID:Carcinoid tumour. 1241 Jan 71

Our objective was to report the outcome in patients with liver metastasis from endocrine tumors who underwent transarterial chemoembolization (TACE) as first-line non-surgical treatment. From January 1990 to December 2000, 14 patients with progressive unresectable liver metastases from digestive neuroendocrine tumor were treated with TACE (mean of 3.6 sessions) before any non-surgical treatment (somatostatin analogue, chemotherapy or interferon). Liver involvement was less than 50% in 11 patients. Size of the largest lesion ranged from 1.5 to 10 cm. Ten patients presented with carcinoid symptoms. The TACE was performed with Doxorubicin emulsified in Lipiodol and gelatin sponge particles. Symptomatic response upon flushes and/or diarrhea was complete in 7 of 10 cases and partial in 2 of 10 cases. An objective morphologic response was noted in 12 of 14 cases. The 5- and 10-year survival rate from diagnosis was 83 and 56%, respectively. Six patients were alive at the end of the study after 27-100 months from first TACE and 38-142 months from diagnosis. Three of them were successfully palliated for 55, 69, and 100 months with only TACE as treatment. Long-term palliation is possible in unresectable liver metastases from digestive neuroendocrine tumors with a few sessions of TACE as first-line and eventually exclusive treatment.
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PMID:Trans-catheter arterial chemoembolization as first-line treatment for hepatic metastases from endocrine tumors. 1254 Nov 21

In June 1999, a 62-year-old man is hospitalised to evaluate the sonographic suspicion of liver metastases. The biopsy of the liver shows a malignant neuroendocrine tumour. Further diagnostic investigation including gastroscopy, colonoscopy, enteroclysis, thoracal and abdominal CT and somatostatin-receptor-scintigraphy does not localise the primary tumour. In the absence of clinical symptoms a wait and see procedure with clinical and imaging controls at regular intervals is arranged. Beginning in spring of 2001--nearly two years after the initial diagnosis--the patient suffers from progredient diarrhoea and weight loss leading to hospitalisation in September 2001. The existence of secretory diarrhoea, hypokalaemia and hypercalcaemia arouses suspicion of vipoma. This is proven by a remarkably elevated plasma concentration of vasoactive intestinal peptide (VIP). Once more, an accurate investigation is started but no primary tumour can be discovered despite extensive liver metastases. A vipoma is a rare differential diagnosis of secretory diarrhoea. This case report describes the remarkable constellation of liver metastases of a malignant neuroendocrine neoplasm without a primary tumour and the clinical presentation of a W.D.H.A. syndrome (watery diarrhoea, hypokalaemia and hypo- or achlorhydria). Despite extensive disease, therapy with octreotide and prednisolone provides a good clinical response.
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PMID:[W.D.H.A. Syndrome due to occult neuroendocrine malignancy with concomitant liver metastases]. 1259 2


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