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A combined glucagonoma and VIPoma syndrome. First pathologic and clinical report. 1988

P Cavallo-Perin, and M De Paoli, and G Guiso, and A Sapino, and M Papotti, and R Coda, and G Pagano
Institute of Internal Medicine, University of Turin, Italy.

We report a case of pancreatic tumour metastatic to the liver in a patient with insulin-treated diabetes, anaemia, cheilitis, necrolytic migratory erythema, hypokalemia and chronic watery diarrhea, a picture suggesting combined glucagonoma and VIPoma syndromes. Immunocytochemistry of a biopsied hepatic metastatic nodule revealed both glucagon and vasoactive intestinal peptide (VIP) positive cells. Increased plasma glucagon and VIP levels were detected (values of 900 pmol/l and 277 pmol/l respectively). This is the first reported case showing not only immunocytochemical, but also clinical evidence of the combined secretion of these hormones.

UI MeSH Term Description Entries
D007516 Adenoma, Islet Cell A benign tumor of the pancreatic ISLET CELLS. Usually it involves the INSULIN-producing PANCREATIC BETA CELLS, as in INSULINOMA, resulting in HYPERINSULINISM. Islet Cell Tumor,Islet of Langerhans Tumor,Nesidioblastoma,Pancreatic Islet Cell Tumors,Island Cell Tumor,Adenomas, Islet Cell,Island Cell Tumors,Islet Cell Adenoma,Islet Cell Adenomas,Islet Cell Tumors,Langerhans Tumor Islet,Nesidioblastomas,Tumor Islet, Langerhans,Tumor, Island Cell,Tumor, Islet Cell,Tumors, Island Cell,Tumors, Islet Cell
D008113 Liver Neoplasms Tumors or cancer of the LIVER. Cancer of Liver,Hepatic Cancer,Liver Cancer,Cancer of the Liver,Cancer, Hepatocellular,Hepatic Neoplasms,Hepatocellular Cancer,Neoplasms, Hepatic,Neoplasms, Liver,Cancer, Hepatic,Cancer, Liver,Cancers, Hepatic,Cancers, Hepatocellular,Cancers, Liver,Hepatic Cancers,Hepatic Neoplasm,Hepatocellular Cancers,Liver Cancers,Liver Neoplasm,Neoplasm, Hepatic,Neoplasm, Liver
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010190 Pancreatic Neoplasms Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA). Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D003969 Vipoma A tumor that secretes VASOACTIVE INTESTINAL PEPTIDE, a neuropeptide that causes VASODILATION; relaxation of smooth muscles; watery DIARRHEA; HYPOKALEMIA; and HYPOCHLORHYDRIA. Vipomas, derived from the pancreatic ISLET CELLS, generally are malignant and can secrete other hormones. In most cases, Vipomas are located in the PANCREAS but can be found in extrapancreatic sites. Diarrheogenic Tumor,Pancreatic Cholera,Verner-Morrison Syndrome,WDHA,WDHH,Watery Diarrhea Syndrome,Watery Diarrhea with Hypokalemic Alkalosis,Diarrheogenic Islet Cell Tumor,Pancreatic VIPoma,VIP-Secreting Tumor (VIPoma),Vasoactive Intestinal Peptide (VIP) Tumor,Vasoactive Intestinal Peptide-Producing Tumor,Vipoma Syndrome,WDHA Syndrome,Watery Diarrhea, Hypokalemia, and Achlorhydria Syndrome,Cholera, Pancreatic,Diarrheogenic Tumors,Pancreatic VIPomas,Syndrome, Verner-Morrison,Syndrome, Vipoma,Tumor, Diarrheogenic,Tumor, VIP-Secreting (VIPoma),Tumors, Diarrheogenic,Tumors, VIP-Secreting (VIPoma),VIP Secreting Tumor (VIPoma),VIP-Secreting Tumors (VIPoma),VIPoma, Pancreatic,VIPomas, Pancreatic,Vasoactive Intestinal Peptide Producing Tumor,Verner Morrison Syndrome,Vipomas,WDHA Syndromes
D005935 Glucagonoma An almost always malignant GLUCAGON-secreting tumor derived from the PANCREATIC ALPHA CELLS. It is characterized by a distinctive migratory ERYTHEMA; WEIGHT LOSS; STOMATITIS; GLOSSITIS; DIABETES MELLITUS; hypoaminoacidemia; and normochromic normocytic ANEMIA. Adenoma, alpha-Cell,alpha-Cell Tumor,Glucagonoma Syndrome,Adenoma, alpha Cell,Adenomas, alpha-Cell,Glucagonoma Syndromes,Glucagonomas,Syndrome, Glucagonoma,Syndromes, Glucagonoma,Tumor, alpha-Cell,Tumors, alpha-Cell,alpha Cell Tumor,alpha-Cell Adenoma,alpha-Cell Adenomas,alpha-Cell Tumors
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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