Glucagonoma

Abstract

A 36 year old woman was admitted because of upper abdominal pain, fullness and weight loss. Pancreatic scintigram revealed abnormal accumulation of the radioisotope in the pancreatic head, and hepatic scintigram showed multiple filling defect in the bilateral lobe. Celiac angiogram demonstrated a tumor stain at the pancreatic head, encasement of the splenic artery and metastasis to the liver. The diagnosis of malignant glucagonoma was substantiated by high serum glucagon level of 1,100 pg/ml. Streptozotocin of 1.5g was administered intravenously once a week, totalling 9g. Thereafter, blood level of glucagon declined to the normal range, accompanied by improvement of diabetes mellitus and weight gain. At laparotomy, there was an over fist-sized mass at the body and tail of the pancreas, infiltrating the pancreatic head and periaortic region, and was found unresectable. On light microscopy, biopsied specimen was seen to be a tumor that contained glucagon. Secretary granules resembling A cell granules were observed by electron microscopy. She died of emaciation 6 years after the onset of the disease. Eighteen cases of glucagonoma reported in Japan, and 64 cases in Europe and the United States were reviewed in terms of the diagnosis and treatment.