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)

Primary cardiac tumors are rare, and there are no reports of patients with a functional gastroenteropancreatic tumor syndrome caused by such a tumor. This case report describes a patient with a cardiac gastrinoma causing Zollinger-Ellison syndrome. Evidence is presented that this tumor represents a primary cardiac tumor. The exact identification of this gastrinoma in an extra-abdominal site was facilitated by the use of [111In-DTPA-DPhe1]octreotide scanning for somatostatin receptors, which these tumors characteristically possess in high numbers. The recent availability of this novel localization method may facilitate identification of extra-abdominal sites in an increasing proportion of patients with gastrinomas and related neuroendocrine functional tumors in which no intra-abdominal primary tumor is currently found.
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PMID:Primary cardiac gastrinoma causing Zollinger-Ellison syndrome. 902 11

Calcium infusion has been advocated as a provocative test for the diagnosis of some endocrine tumors of the pancreas and gastrointestinal tract (gastrinoma, insulinoma, intestinal carcinoids). The release of gastrin from gastrinoma tissue is very sensitive to alterations in the serum calcium level, and the calcium infusion test is recommended in Zollinger-Ellison syndrome when the results of secretin stimulation are equivocal. The calcium provocative test in the detection of insulinoma and carcinoid tumors is less reliable than other safer and simpler procedures. Intravenous injection of calcium followed by pentagastrin stimulates the release of somatostatin in patients with somatostatinoma and offers a reliable means for establishing the diagnosis of this tumor. Calcium administration has not proven to be useful in the diagnosis of other endocrine tumors of the digestive system.
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PMID:Use of calcium provocative test in the diagnosis of gastroenteropancreatic endocrine tumors. 906 35

The clinico-pathological features of 53 Chinese patients (27 males; 26 females) with pancreatic endocrine tumours were studied. The age range was from 14 to 78 years old (mean: 48 years) with the modal peak in the sixth decade for both sexes. Pancreatic endocrine tumours accounted for 14% of the primary pancreatic tumours operated on in Queen Mary Hospital. The autopsy incidence was 0.11%. Seventy-two per cent (38 cases) of the tumours were clinically functioning, comprising 33 insulinomas, three gastrinomas and two glucagonomas. A rare case of malignant gastrinoma associated with Cushing's syndrome was also documented. The functional tumours were seen in the younger patients. The calculated annual incidence of clinically significant tumours was approximately 0.2 per 100,000 population. There was no correlation between the site, functional status and histological patterns of the tumours. Seventy-two per cent of the tumours showed a trabecular pattern. Calcification was present in 5.7% (three cases); two such cases being gastrinomas. Amyloid was found in 25% of tumours, chiefly (92%) in the insulinomas. The main difficulty encountered in diagnosis was distinguishing between solid and cystic tumours of the pancreas. The incidence of malignancy was 15% and the histological features were poor predicative indicators of malignant potential. The metastatic pancreatic endocrine tumours were often detected in the liver and lymph nodes. Immunohistochemical stains showed evidence of multi-hormone production in 18% of cases and all tumours showed a positive reaction to at least one of the six markers, namely, neuron-specific enolase (NSE), chromogrannin (CG), synaptophysin (SYN), insulin (INS), glucagon (GLU) or somatostatin (SOM). The three panendocrine markers (NSE, SYN, CG) were satisfactory for initial screening of the endocrine nature of the tumours if used in combination, as 92% of tumours were positive for at least one of these three markers.
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PMID:Pancreatic endocrine tumour: a 22-year clinico-pathological experience with morphological, immunohistochemical observation and a review of the literature. 906 45

Neuroendocrine tumours can form in any part of the gastrointestinal tract. The most common types are the ECL cell tumours of the oxyntic mucosa of the stomach, G cell tumours of the duodenum, argentaffin, EC cell tumours of the small intestine and L cell tumours of the large bowel. The only well-defined clinical syndromes associated with hormone hypersecretion are ZES, resulting from duodenal gastrinomas, and carcinoid syndrome, caused by malignant argentaffin tumours. Genetic predisposition has been demonstrated for some tumour types, e.g. duodenal gastrinoma in MEN 1 and duodenal somatostatin cell tumours in MEN 2. Other factors predisposing to the genesis of these lesions include circulating hormone levels and the maintenance of chronic inflammatory states. As with most neuroendocrine tumours, malignant potential is difficult to assess on the basis of histology alone and prognostic evaluation depends more on size and evidence of local invasion and/or distant metastases.
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PMID:Gastrointestinal endocrine tumours. Pathology. 911 12

Gastrinomas in dogs are difficult to diagnose, localise and treat. In humans, somatostatin analogues have improved localisation and treatment of gastrinomas. The somatostatin analogues pentetreotide and octreotide were evaluated for the detection and treatment of gastrinoma in a dog. 111indium-pentetreotide scintigraphy revealed multiple areas of activity in the patient's mid-ventral abdomen which were consistent with masses in the pancreas and liver at laparotomy. Immunohistochemistry, electron microscopy and binding of 125I-[Tyr3]-octreotide in vitro confirmed the lesion as a gastrinoma which expressed somatostatin receptors. Octreotide at doses of 2, 4 and 8 micrograms/kg caused transient decreases in circulating gastrin. Plasma somatostatin peaked at one hour after octreotide and was still detectable at four and six hours after administration of octreotide. Combination therapy with famotidine, omeprazole, sucralfate and increasing doses of octreotide allowed patient survival for 14 months.
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PMID:Evaluation of somatostatin analogues for the detection and treatment of gastrinoma in a dog. 923 29

Somatostatin (SST) and its analogues are candidates for use as endocrine agents in the treatment of pancreatic neoplasm. To determine whether the status of SST receptors in the human pancreatic tumors differs from that in the tumor-free pancreata of the human and whether pancreatic adenocarcinoma expresses the same subgroup of SST receptors as found in gastrinomas, this study visualized and characterized SST receptors in human control pancreata (n = 10) as well as pancreatic cancers (n = 12) and gastrinomas (n = 8) with storage phosphor autoradiography. Both pancreatic adenocarcinoma and gastrinoma expressed specific SST receptors. The binding capacity (Bmax, 35.4 +/- 7.6 fmol/mg protein) and the affinity (Kd, 0.32 +/- 0.27 nM) of SST receptors in gastrinomas were significantly higher than in pancreatic cancers (Bmax, 15 +/- 2.5 fmol/mg protein; Kd, 2.16 +/- 0.4 nM). No specific SST receptors were detected in the human control pancreata. Octreotide showed similarly high potencies of inhibition of 125I-SST-28 binding as SST-28 in gastrinomas. Unlike gastrinomas, little competitive binding of 125I-SST-28 was found with octreotide in pancreatic cancers. In conclusion, compared with the control pancreas, an up-regulation of SST receptors was present in both pancreatic cancer and gastrinoma. The subgroup of SST receptors in pancreatic cancers differs from that in gastrinomas.
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PMID:Expression of somatostatin receptors in human pancreatic tumor. 966 24

1,4,7,10-tetraazacyclododecane-N,N',N",N'''-tetraacetic acid (DOTA)-lanreotide is a universal somatostatin (SST) receptor subtype ligand that binds to a large variety of human tumors. We report the case of a patient with metastatic gastrinoma who was treated with 90Y-DOTA-lanreotide. Before treatment, dosimetry with 111In-DOTA-lanreotide (150 MBq, 10 nmol) indicated a dose of 5.8 mGy/MBq for the recurrent abdominal gastrinoma, and a mean dose of approximately 1.0 mGy/MBq for liver metastases (i.e., 56 and approximately 10 mGy/MBq for 90Y-DOTA-lanreotide, respectively). After four infusions of 90Y-DOTA-lanreotide (each 1 GBq, approximately 30 nmol) over a 6-mo period, the 111In-DOTA-lanreotide scintigraphy of the liver had returned to a nearly normal condition and a remarkably decreased uptake by the recurrent gastrinoma was calculated (approximately 5 mGy/MBq for 90Y-DOTA-lanreotide). The imaging results were well-correlated with a 25% regression of the liver metastases as indicated by CT. Blood, urine and whole-body clearances of 111In-DOTA-lanreotide and 90Y-DOTA-lanreotide were very similar. The DOTA-lanreotide promises to be useful for functional tumor diagnosis (111In-DOTA-lanreotide) and receptor-mediated tumor radiotherapy (90Y-DOTA-lanreotide).
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PMID:Response to treatment with yttrium 90-DOTA-lanreotide of a patient with metastatic gastrinoma. 986 48

Medical treatment is the elective therapy for patients with gastrinoma when the tumor is not found at surgery or is unresectable or when there is a metastatic disease. H2-blockers and omeprazol are able to control gastric acid secretion and, in addition, somatostatin analogues decrease gastrin levels. A new long-acting and slow release formulation of a somatostatin analogue (lanreotide, SR-L) has been developed. We treated two patients suffering from gastrinoma, total gastrectomy and hepatic metastases with 30 mg intramuscular injections of SR-L every 15 and 10 days, respectively, for a seven-month period. After the treatment, gastrin levels decreased from 35,494 and 15,086 ng/l to 3,211 and 167 ng/l (92 and 98% below pre-treatment levels) in case 1 and 2 respectively, with a relief of symptoms and no side effects.
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PMID:Short- and long-term effect of a long-acting somatostatin analogue, lanreotide (SR-L) on metastatic gastrinoma. 1019 83

Somatostatin receptor scintigraphy is the best imaging method to identify the presence of neuroendocrine gastroenteropancreatic tumours. Nevertheless, a well structured surgical approach incorporating specific intra-operative methods can localize those tumours that cannot be readily detected by this imaging technique. In the case of gastrinoma, standard palpation allows duodenal tumour detection in approximately 60% of cases, endoscopic transillumination, in more than 80%. Furthermore, adding duodenotomy, 95-97% duodenal tumours can be localized. Intraoperative ultrasound, instead, does not add much to standard palpation in duodenal gastrinoma localization. For insulinoma detection, among the intra-operative methods, inspection gives the poorest results, identifying the lesion in only 20% of cases. Palpation offers better results, localizing 60-80% of insulinomas. The introduction of intra-operative ultrasound has revolutionized the ability to find pancreatic insulinoma, allowing the surgeon to identify the insulinoma in nearly every patient.
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PMID:Intra-operative procedures to localize endocrine tumours of the pancreas and duodenum. 1060 29

Disseminated neuroendocrine tumours are difficult to treat and are generally not responsive to radiotherapy or chemotherapy. Nuclear medicine techniques using a radiolabelled somatostatin analogue, 111In-Octreotide, have been used for the diagnosis of neuroendocrine tumours. It has been suggested that high activities of such an agent may have a therapeutic effect. The aims of this study were to assess toxicity and to determine if there had been evidence of efficacy. Eight patients with known disseminated neuroendocrine tumours were enrolled in the study; six had carcinoid tumours, one had a medullary cell carcinoma of the thyroid and one patient had a malignant gastrinoma. Between 1.3 and 4.6 GBq of 111In-Octreotide were administered to each patient for up to five administrations over 12 months. A total of 23 administrations were given. Tests of vital signs, renal, liver and endocrine function as well as haematological markers were taken before and after treatment. The treatment was well tolerated with only one patient suffering from a sensation of flushing during the infusion but no changes in vital sings. There was a transient (up to 48 h) drop in circulating lymphocytes in four patients and platelets in two patients; no supportive therapy was needed. One patient with severe renal impairment had a slight reduction in glomerular filtration rate. We conclude that high-activity 111In-Octreotide is well tolerated with low toxicity and can be considered for use in patients with disseminated neuroendocrine tumours. Further work is now being performed to assess efficacy.
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PMID:Toxicity of high-activity 111In-Octreotide therapy in patients with disseminated neuroendocrine tumours. 1071 9


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