Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P01350 (gastrin)
 9,683 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four cases of esophageal carcinoma arising in metaplastic Barrett's epithelium are presented in which multidirectional differentiation was demonstrated by light and/or electron microscopy and immunohistochemistry. All tumors and adjacent mucosa produced both neutral and acidic mucins, as well as one or more hormones indigenous to the gut, including gastrin, bombesin, substance P, somatostatin, and serotonin. Gastrin and somatostatin were the peptides most frequently identified in the tumors, while somatostatin and serotonin predominated in Barrett's epithelium. Ultrastructurally, neurosecretory-type granules, 80-250 nm in diameter, were present in 2 cases; squamous features also were present in one of these cases. One patient displayed hypertrophic osteoarthropathy, which disappeared after the tumor was resected. These cases represent the majority of the Barrett-associated carcinomas in our material. Compared to the "pure" esophageal adenocarcinomas not included in this report, these tumors behaved more aggressively, with wider local involvement and nodal and systemic metastases at the time of presentation. The incidence of multidifferentiation in esophageal carcinomas is not known nor is its possible significance, particularly with regard to tumors arising in metaplastic epithelium. This group may merit further study to detect true differences, if any, between these esophageal carcinomas and their apparently more common counterparts.
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PMID:Carcinoma with multidirectional differentiation arising in Barrett's esophagus. 613 8

Since the availability of the H2-receptor antagonists (1978), seven patients admitted to the Department of Emergency Surgery, University of Pisa, with Zollinger-Ellison syndrome (ZES) sustained by extrapancreatic gastrinomas underwent elective surgical treatment. There were four women and three men, with a mean age of 37.3 years (16 to 67 years of age). Preoperative localization studies included endoscopy of the upper part of the gastrointestinal tract, computed axial tomography, ultrasound, selective angiography and transhepatic portal vein sampling for gastrin and were effective in four patients. In the other three patients, the localization of the gastrinoma was made at laparotomy. Four patients had gastrinomas of the duodenal and jejunal wall. Three underwent excisional operations, whereas the remnant was treated with pancreatoduodenectomy. In three of the patients, the location of the gastrinoma was extrapancreatic and extraintestinal, and surgical treatment consisted of nodal excision (two patients) and hepatectomy. None of the patients had undergone a gastric operation in addition to tumorectomy procedures. Soon after excisional operation, ZES recurred in one patient and a second laparotomy with nodal excision resulted in a cure. On long term follow-up evaluation, ranging from 15 to 136 months (mean of 85.1 divided by 37.3 months), no patients showed a recurrence of ZES. The possibility of extrapancreatic gastrinoma should be suspected in patients with sporadic ZES. The need for emergency surgical treatment in these patients is minimized by the medical control of the gastric secretion that consents diagnostic studies for the preoperative localization of the gastrinoma. Even if only suspected, the site of the extrapancreatic gastrinoma has an increased chance to be identified at operation. This consents to remove gastrinomas mostly by a minimal excisional procedure resulting in the normalization of gastric secretion and the prevention of malignant evolution of these tumors.
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PMID:Outcome of surgical treatment for extrapancreatic gastrinomas. 834 95

Gastric neuroendocrine tumours (NETs) arise from enterochromaffin-like cells, which are located in oxyntic glands within the stomach. Type I tumours represent 70-80% of gastric NETs and are associated with hypergastrinaemia, chronic atrophic gastritis and achlorhydria. Gastrin is involved in the endocrine regulation of gastric acid production. Most type I gastric NETs are sporadic, have a good prognosis and their genetic basis are unknown. We performed an exome sequencing study in a family with consanguineous parents and 10 children, five of whom were affected by type I gastric NET. Atypical clinical traits included an earlier age of onset (around 30 years), aggressiveness (three had nodal infiltration requiring total gastrectomy and one an adenocarcinoma) and iron-deficiency rather than megaloblastic anaemia. We identified a homozygous missense mutation in the 14th exon of the ATP4A gene (c.2107C>T), which encodes the proton pump responsible for acid secretion by gastric parietal cells. The amino acid p.Arg703Cys is highly conserved across species and originates a change of one of the transmembrane domains that avoids the liberation of protons from cells to stomach. This is consistent with the achlorhydria that was observed in the affected individuals. No germline or somatic mutations in the ATP4A gene were found in sporadic gastric NET patients. Based on the results of this large family, it seems that this atypical form of gastric NET has an earlier age of onset, behaves more aggressively and has atypical clinical traits that differentiated from other studied cases.
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PMID:Exome sequencing identifies ATP4A gene as responsible of an atypical familial type I gastric neuroendocrine tumour. 2567 51

Primary lymph node gastrinoma has been defined as gastrin-producing tumor present in lymph nodes and predominantly found in well-defined anatomical region known as gastrinoma triangle. They are usually localized preoperatively with imaging, and their surgical resection results in long-term relief. The authors report a case of unresectable primary lymph nodal gastrinoma with liver metastases in a 14-year-old adolescent boy with proven histopathology detected on Ga-DOTANOC whole-body PET/CT scan followed by preoperative multiple Lu-DOTATATE cycles for cytoreduction. Subsequent surgical resection of residual mass resulted in complete response with a follow-up of around 4 years in this unusual case of Zollinger-Ellison syndrome.
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PMID:Management of Primary Lymph Nodal Gastrinoma With Liver Metastases Resulting in Zollinger-Ellison Syndrome. 3039 27