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

The cDNA microarray gene profile of gastrointestinal stromal tumors (GISTs) revealed that DOG1 (TMEM16A) gene was mostly expressed in these neoplasms. Immunohistochemically, DOG1 protein was found positive in a significant proportion of GISTs. However, normal tissues' expression of DOG1 is not yet completely studied. Our study intended to identify the DOG1 protein expression in normal adult and fetal tissues, in comparison with that of GISTs, using an anti-DOG1 polyclonal serum. Fourteen CD117/CD34-positive GIST cases were tested for DOG1. Tissue samples from autopsies of 15 human fetuses and 11 adults were tested immunohistochemically on simple or double staining with antibodies raised against: DOG1, insulin, glucagon, somatostatin, NK1, PGP9.5, chromogranin A, and synaptophysin. All the tested GISTs were positive for DOG1, with a membranous and cytoplasmic location. The normal tissues showed a distinct positivity for DOG1 only in the endocrine pancreas, in both fetal and adult ones. The other tissues tested showed a weak or negative reaction. The DOG1 staining pattern in the pancreas islets was granular, like that of neuroendocrine markers. The location of DOG1 expression in pancreatic islets was partly similar to neuroendocrine markers chromogranin A, PGP9.5, and synaptophysin. The positive cells were situated centrally, in the vicinity of insulin-bearing cells as seen on double staining. DOG1 positivity in fetal and adult pancreatic islets suggests the strong antibody affinity for neuroendocrine cells. Before making a final conclusion regarding the suitability of DOG1 as a new neuroendocrine marker, a large survey of neuroendocrine lesions must be undertaken, including carcinoid tumors of various sites and pancreatic endocrine tumors. To the best of our knowledge, this particular localization has not been reported yet for DOG1.
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PMID:Pancreatic expression of DOG1: a novel gastrointestinal stromal tumor (GIST) biomarker. 1941 27

Despite comprising at least 75% of the length of the gastrointestinal tract, the small bowel only accounts for 3 to 6% of all its neoplasms. Forty different tumor subtypes arise from the small bowel; the commonest is adenoma, and malignant lesions include gastrointestinal stromal tumor neuroendocrine tumor lymphoma, and adenocarcinoma. Small bowel tumors typically cause either non-specific symptoms or none at all, which explains both the frequent delay in diagnosis and the wide range of potential investigations. The relative inaccessibility of the small bowel to endoscopic assessment is being challenged by the increased use of both capsule and double balloon endoscopy. Advances in endoscopic assessment are mirrored by improved sensitivity of radiological and nuclear imaging. Operative resection provides the mainstay of treatment for malignant disease (and symptomatic benign lesions), with oncological agents and somatostatin analogues providing useful adjuncts for inhibiting tumor growth and relieving symptoms. Survival reflects underlying tumor subtype, but is generally poor for malignant disease.
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PMID:Small bowel tumors: pathology and management. 2470 41

Both multiple endocrine neoplasia type 1 (MEN1)-related gastrinomas and gastrointestinal stromal tumors (GISTs) are rare neoplasms, and their association has been rarely reported. We describe an unusual association between a GIST and a MEN1-related gastrinoma. A 44-year-old man had undergone surgical removal of a pancreatic gastrinoma in 2004 and was then administered long-term somatostatin analogs, and diagnosed as having MEN1 syndrome. Following an uneventful follow-up, in April 2009, an upper gastrointestinal tract endoscopy showed esophageal narrowing, with evidence of a 2-cm solid mass on endoscopic ultrasonography. Histology revealed a tumor composed of elongated cells with plump cytoplasm arranged in a storiform pattern. The immunophenotype of the lesion was CD117 and Platelet Derived Growth Factor (PDGF) positive, whereas alpha-1 muscle actin and S-100 protein were negative. Due to morphological and immunohistochemical results, a final diagnosis of esophageal GIST was made. The association between GISTs and MEN1 could be casual, although a single case of the coexistence of a GIST and a MEN1-related gastrinoma has already been reported. A role of the MEN1 gene in the pathogenesis of GISTs could be hypothesized.
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PMID:An esophageal gastrointestinal stromal tumor in a patient with MEN1-related pancreatic gastrinoma: an unusual association and review of the literature. 2502 20

Somatostatin receptors (SSTRs) already act as important roles in gastroenteropancreatic neuroendocrine tumors (GEP-NETs) with high expression levels for prognosis predicting and octreotide LAR treatment purposes but less noticed in gastrointestinal stromal tumors (GISTs). Our study aims to fully evaluate the expression levels and prognostic values of SSTRs in GIST patients. For SSTRs expression detection, qPCR were used in 25 fresh GIST specimens, and then, 453 GIST samples (405 GISTs with operation only and 48 with imatinib adjuvant therapy after surgery) were collected for tissue microarrays (TMAs) construction and confirmed by immunohistochemistry (IHC). Clinicopathological data were confirmed by pathological diagnosis and clinical recorders, recurrence-free survivals (RFS) were evaluated in 453 GIST patients. With IHC performed, SSTR1 and SSTR2 present high positive proportion (81.9% and 87.6%) in 453 GISTs in our study, and positive expression rates of SSTR3, SSTR4 and SSTR5 are 56.1%, 8.8% and 47.2%, respectively. SSTR2 and SSTR5 negative expression are associated with decreased RFS when compared to positive cases by Kaplan-Meier survival analyses with log-rank test and univariate analysis in GISTs, furthermore, SSTR2 was an independent prognostic indicator for GISTs by multivariate analysis. In our study, detection of SSRT2 and SSTR5 expression helps to predict different prognosis in GIST patients. SSTR2 is a novel independent prognostic biomarker for GISTs. With high expression performance of SSTRs in GISTs, new therapeutic strategies such as octreotide or pasireotide LAR could be taken into consideration in selected advanced GIST patients.
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PMID:Somatostatin receptors in gastrointestinal stromal tumors: new prognostic biomarker and potential therapeutic strategy. 2562 93

A 57-year-old woman with von Recklinghausen's disease presented with epigastralgia. Gastroduodenoscopy revealed swelling of the ampulla of Vater in the ventral and caudal direction, forming a hard, elastic mass. She was diagnosed with a tumor of the ampulla of Vater, and a subtotal stomach-preserving pancreaticoduodenectomy and D2 lymph node dissection were performed. The isolated specimen showed an intra-ampullary tumor of the ampulla of Vater and a submucosal tumor in the descending duodenum, which were diagnosed as a somatostatin-producing neuroendocrine tumor and gastrointestinal stromal tumor, respectively, on pathological examination. We believe that the neuroendocrine tumor of the ampulla of Vater and gastrointestinal stromal tumor of the duodenum are common gastrointestinal lesions in von Recklinghausen's disease.
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PMID:[Neuroendocrine Tumor of the Ampulla of Vater and Gastrointestinal Stromal Tumor of the Duodenum in a Patient with Von Recklinghausen's Disease]. 2813 23