Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Glucagon-like peptides (GLP) 1 and 2 are hormones derived from the post-translational processing of proglucagon in the intestinal L cells that influence intestinal motility and small bowel growth, respectively. We describe a patient with a neuroendocrine tumor of unknown primary origin with peritoneal carcinomatosis and diffuse liver metastases, who presented with constipation and nocturnal itching for over 3 years. Small bowel follow-through showed decreased small intestinal motility and marked intestinal hypertrophy. Biopsies from mesenterial lymph nodes showed, histologically, a well-differentiated neuroendocrine tumor (G1), with positive immunostaining for chromogranin A, GLP-1, GLP-2 and polypeptide YY (PYY). Jejunal biopsy demonstrated marked intestinal mucosal hypertrophy. HPLC analysis combined with RIA of tumor and serum extracts revealed that the tumor was producing and releasing fasting levels of GLP-1 of 738+/-20.7 pg/ml (normal levels (nl) <100 pg/ml), GLP-2 of 3,150+/-9 pg/ml (nl <100 pg/ml) as well as PYY 550 pg/ml (nl <100 pg/ml). Octreotide administration decreased levels of GLP-1 and GLP-2 and reduced small intestinal transit time from 150 to 50 min. However, tumor growth was not inhibited by octreotide, interferon or dacarbazine therapy and the patient died 8 months later. This is the first case report demonstrating the overproduction of GLP-1, GLP-2 and PYY from an neuroendocrine tumor, in a patient with intestinal hypertrophy and delayed intestinal transit time.
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PMID:Intestinal proliferation and delayed intestinal transit in a patient with a GLP-1-, GLP-2- and PYY-producing neuroendocrine carcinoma. 1117 2

A 75-year-old man was admitted to our hospital because of abdominal fullness. Imaging modalities revealed multiple tumors in the liver, but did not detect other tumors. Endoscopic examination revealed an ulcerated tumor in the duodenum. Biopsies were obtained from the duodenal tumor and liver tumors. The duodenal biopsies revealed malignant cells of composite carcinoid and small cell carcinoma. The carcinoid component showed trabecular and ribbon features, and mitotic figures were seen in one per high power fields, thus fulfilling the criteria of typical carcinoid. By contrast, the small cell carcinoma component showed typical small cell carcinoma features. The liver biopsies showed typical small cell carcinoma. The tumors of both organs were argyrophilic. Immunohistochemically, the tumor cells of both organs were negative for gastrin, somatostatin, serotonin, glucagon, and PP, being compatible with a non-functioning tumor. The duodenal and liver tumors were positive for pancytokeratins, epithelial membrane antigen, chromogranin, synaptophysin, CD56, and neuron-specific enolase. By contrast, they were negative for S100 protein, CD45, CD20, CD3, TTF-1, and calcitonin. Cytokeratin 7 was positive in the carcinoid component but negative in the small cell carcinoma component. Cytokeratin 20 was negative in both components and in liver tumors. The Ki-67 labeling was 8% in the carcinoid element and 34% in the small cell carcinoma element in the duodenal tumor, and 38% in the hepatic tumors. The patient died of carcinomatosis 6 months after admission. These findings demonstrate a composite carcinoid and small cell carcinoma in the duodenum. A review of the literature in English language revealed no cases of composite carcinoid and small cell carcinoma in the duodenum.
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PMID:Composite carcinoid and small cell carcinoma of the duodenum. 2064 74