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)

A 12-year-old, male black and white colobus monkey (Colobus guereza kikuyuensis) from a small community zoo presented with a 6-month history of mild, slowly progressive ataxia and paresis culminating in an acute episode of recumbency, depression, and seizures. The animal was humanely euthanatized. Gross post-mortem examination revealed significant abnormalities including diffuse pallor of the carcass and a firm, pale, 8-cm diameter mass, adherent to the serosa of the proximal duodenum and colon, and embedded within the pancreas and mesenteric root. Histologically, the mass had characteristics of a neuroendocrine or endocrine tumor. Immunohistochemical stains for chromogranin, synaptophysin, insulin, and glucagon were positive, confirming the diagnosis of a mixed pancreatic islet cell tumor. These tumors are rare in all species except ferrets and unreported previously in colobus monkeys.
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PMID:Spontaneous pancreatic islet cell tumor in a black and white colobus monkey (Colobus guereza kikuyuensis). 1826 22

A 65-year-old Japanese male was referred to our hospital for evaluation of a main pancreatic duct obstruction. Two months previously, he had suffered an attack of acute pancreatitis that was resolved with conservative treatment. Dynamic contrast-enhanced study by multidetector row computed tomography revealed a well-enhanced 5 x 5 mm mass in the head of the pancreas with dilatation of the main pancreatic duct in the body and tail. On endoscopic retrograde pancreatography, obstruction of the main pancreatic duct in the head of the pancreas was noted. Pancreatic juice cytology was nondiagnostic. Endoscopic ultrasonography demonstrated a well-defined hypoechoic mass, about 5 mm in size, with distal main pancreatic duct dilatation. The patient underwent elective pylorus-preserving pancreaticoduodenectomy. Pathological examination revealed a well-differentiated endocrine tumor of the pancreas of uncertain behavior, 5 mm in size. Immunohistochemically, the tumor was diffusely positive for chromogranin A and synaptophysin, and focally it was positive for insulin, glucagon, and CA19-9; it was negative for gastrin. The final diagnosis was main pancreatic duct obstruction secondary to a nonfunctioning endocrine tumor of the pancreas of uncertain behavior. Of note, a small nonfunctioning endocrine tumor of the pancreas is important in the differential diagnosis of main pancreatic duct obstruction demonstrated by radiographic examinations.
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PMID:Main pancreatic duct obstruction due to a small nonfunctioning endocrine tumor of the pancreas. 1940 May 56

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

This article describes a newly recognized highly malignant neoplastic entity in young bearded dragons (Pogona vitticeps), gastric neuroendocrine carcinomas, which readily metastasize. Ten bearded dragons with histories of anorexia (8), vomiting (3), hyperglycemia (2), and anemia (3) were included in this study. All animals had neoplastic masses in their stomach, with metastasis to the liver. Microscopically, 6 of these neuroendocrine carcinomas were well-differentiated and 4 were poorly differentiated. For further characterization, immunohistochemistry for protein gene product 9.5, neuron-specific enolase, endorphin, chromogranins A and B, synaptophysin, somatostatin, insulin, glucagon, gastrin, pancreatic polypeptide, and vasoactive intestinal peptide was performed on 5 animals. Because only immunolabeling for somatostatin was consistently observed in all neoplasms, a diagnosis of somatostatinoma was made for these 5 bearded dragons. Some neoplasms also exhibited multihormonal expression. Electron microscopy performed on 1 tumor confirmed the presence of neuroendocrine granules within neoplastic cells. Gastric neuroendocrine carcinomas, and specifically somatostatinomas, have not been previously reported in bearded dragons, or other reptiles, and may be underdiagnosed due to inconsistent, ambiguous clinical signs. In humans, pancreatic somatostatinomas are associated with a syndrome of hypersomatostatinemia, which includes hyperglycemia, weight loss, and anemia, as observed in some of these bearded dragons. Somatostatinomas in humans are commonly associated with neurofibromatosis type 1 (Von Recklinghausen's disease), caused by a mutation in the tumor suppressor gene NF1, which results in decreased expression of neurofibromin. In all 5 animals examined, neoplasms exhibited decreased neurofibromin expression compared with control tissues, suggesting that decreased functional neurofibromin may play a role in the pathogenesis of somatostatinomas in bearded dragons.
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PMID:Gastric neuroendocrine carcinomas in bearded dragons (Pogona vitticeps). 1960 3

We previously reported that Reg IV is associated with neuroendocrine (NE) differentiation in gastric cancers. The aim was to examine which NE hormone products are related to Reg IV-positive NE cells and their roles in gastric cancers. In the present study, we performed immunohistochemical analysis in a tissue microarray (TMA) of a consecutive series of 630 cases with ten different antibodies, including chromogranin A, synaptophysin and neural cell adhesion molecule (NCAM) as NE differentiation markers, and gastrin, serotonin, calcitonin, gastrin-releasing peptide (GRP), pancreatic polypeptide (PP), somatostatin and glucagon as NE hormones. In 630 cases, we identified 205 (33%) with NE differentiation and 147 (23%) positive for Reg IV. Reg IV-positive cases showed NE differentiation more frequently than Reg IV-negative cases (P < 0.0001). In 205 cases with NE differentiation, Reg IV-positive cases expressed serotonin (P= 0.0032) and somatostatin (P= 0.036) more frequently than Reg IV-negative cases. Double immunofluorescence staining revealed co-expression of Reg IV with gastrin, serotonin and PP. These results indicate that Reg IV might be a mediating factor of several NE hormones.
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PMID:Immunostaining of gastric cancer with neuroendocrine differentiation: Reg IV-positive neuroendocrine cells are associated with gastrin, serotonin, pancreatic polypeptide and somatostatin. 2040 31

A novel combination of tumors was found in a 68 year-old female with Multiple Endocrine Neoplasia type-1 (MEN 1) that included a cystic pancreatic endocrine neoplasm (CPEN), a pituitary adenoma, and multifocal cholesterol granulomas (MCGs) in the breast, pleura, and the extremities. The pancreatic tumor displayed a single central locule surrounded by a thin rim of neoplastic parenchyma. The tumor showed heterogeneity in the architecture that included glandular, trabecular and solid patterns. The tumor cells of the pancreas were immunohistochemically positive for both endocrine and pancreatic acinar markers including chromogranin A, synaptophysin, glucagon, lipase, and reg protein. Electron microscopy revealed that there were numerous smaller dense-cored neurosecretory granules, larger zymogen-like granules and microvilli on the apical side of the tumor cells. The pancreatic tumor was diagnosed as CPEN with acinar cell features. Analysis of the DNA extracted from the tissues revealed that there is a MEN1 germline mutation in exon 10 codon 527, and somatic mutation in exon 2 codon 32 in the pancreatic tumor, and one base pair deletion in exon 2 codon 79 in the pituitary adenoma. Here, we report the case and discuss possible pathogenesis of CPEN and MCGs in a patient with MEN 1.
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PMID:Multiple endocrine neoplasia type 1-associated cystic pancreatic endocrine neoplasia and multifocal cholesterol granulomas. 2040 35

Pancreatic tumors with combined exocrine and endocrine features are rare. Most reported cases are classified as mixed exocrine and endocrine carcinoma of the pancreas. We report the first case of solitary concomitant endocrine tumor and ductal adenocarcinoma of the pancreas. A 58-year-old patient was admitted for uncontrolled diabetes mellitus and body weight loss. The tumor was fortuitously discovered in the pancreatic tail after a tumor survey panel. Grossly, the solitary tumor had a central fibrous band that clearly divided it into two parts. On microscopic examination, the tumor contained both endocrine and exocrine components distinctly separated by the central fibrous band. The exocrine part showed a poorly-differentiated adenocarcinoma. The endocrine part was strongly immunoreactive to chromogranin, synaptophysin and glucagon. We reviewed the literature on pancreatic tumors with combined exocrine and endocrine features. A simple classification for this group of neoplasms is suggested, including five types: amphicrine, mixed, collision, solitary concomitant and multiple concomitant.
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PMID:Solitary concomitant endocrine tumor and ductal adenocarcinoma of pancreas. 2051 94

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

The objectives of this study were to evaluate the frequency of carcinoid tumors of the extrahepatic biliary ducts (EHBDs) and the pathologic progression and the role of surgery in the management of this disease. We describe two cases of malignant carcinoids of the EHBDs, which presented as common bile duct tumors in two adult male patients, aged 52 and 70 years, who were diagnosed histologically on surgical resection specimens. A comprehensive review of the literature has also been performed with a focus on survival data. Microscopically, the tumors presented herein were composed of relatively small rounded cells with a trabecular or nesting pattern. Both cases were diffusely immunopositive for chromogranin and synaptophysin, and one of them was also focally reactive with somatostatin and pancreatic polypeptide. There was no expression in any of these tumors of thyroid transcription factor-1 (TTF-1), gastrin, insulin, glucagon, vasoactive intestinal peptide (VIP) and prolactin. The tumor showed transmural invasion in both cases, with lymph node metastasis and subcapsular liver tissue infiltration in one. Both patients are alive with no evidence of disease 41 months and 59 months, respectively, after surgery. Despite being extremely uncommon, with only 70 cases reported to date, carcinoids should be included in the differential diagnosis of EHBD tumors. This study emphasizes the necessity of complete surgical resection as the gold standard treatment for these lesions, and the importance of a correct pathologic diagnosis for prognostic implications.
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PMID:Well-differentiated neuroendocrine carcinoma (malignant carcinoid) of the extrahepatic biliary tract: report of two cases and literature review. 2066 35

A 14-year-old female spayed Golden Retriever was presented to the University of Florida's Veterinary Medical Center with history of lymphoplasmacytic gastroenteritis, intermittent vomiting, watery diarrhea, and weight loss for over a year. CBC, biochemical profile, and urinalysis were within reference intervals. Abdominal ultrasonographic examination revealed mesenteric and jejunal lymphadenopathy and hyperechoic hepatic nodules. Cytologic examination of the enlarged lymph nodes revealed loosely cohesive cells with moderate nuclear pleomorphism and rare punctate eosinophilic cytoplasmic granules. The cytologic interpretation was metastatic neuroendocrine neoplasia. On surgical exploration, a mass was detected in the right lobe of the pancreas. Histologic evaluation determined the mass to be an islet cell tumor. Approximately 98% of cells were positive by immunolabeling for pancreatic polypeptide (PP), and only rare cells were positive for insulin or somatostatin. All cells were negative for glucagon, gastrin, vasoactive intestinal polypeptide, protein gene product 9.5, synaptophysin, and chromogranins A and B. Pancreatic tumors that primarily produce PP are rare in dogs, and this is the first report of both the cytologic and histologic features of an islet cell tumor predominantly secreting PP. Clinical signs for these tumors are typically absent or nonspecific; signs may include watery diarrhea, as noted in this dog, although the diarrhea may have resulted from lymphoplasmacytic gastroenteritis. Additional case studies are needed to further characterize the cytomorphologic features and clinical presentation of PP-secreting islet cell tumor, or polypeptidoma, in dogs.
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PMID:Metastatic pancreatic polypeptide-secreting islet cell tumor in a dog. 2069 42


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