Primary Malignant Hepatic Glucagonoma: An Autopsy Case

Abstract

Abstract. a 73-year-old woman was admitted to our department for treatment of diabetes (plasma glucose 289 mg/dl, hba 1C 7.1%, and glycated albumin 34.9%). she displayed the signs and symptoms of glucagonoma syndrome, including necrolytic migratory erythema (Nme), low aminoacidemia, and a marked increase of the serum glucagon level (4,940 pg/ ml). Thus, we suspected a glucagonoma causing secondary diabetes. however, we could not detect any mass in the pancreas or the gastrointestinal tract, and only found a liver lesion resembling a hemangioma. her Nme improved markedly after intravenous infusion of amino acids, and her plasma glucose was controlled reasonably well by intensive insulin therapy. however, her general condition deteriorated and she died on day 57 after hospitalization. at autopsy, the only tumor detected was the liver mass. This was a large solid tumor (8×6×5 cm) with a pattern of white and dark brown stripes located in the left lobe, while two white nodules were also found in the right lobe. based on the histopathological and immunohistochemical findings, the liver lesion was shown to be a malignant glucagonoma with intrahepatic metastases. since primary malignant hepatic glucagonoma has not been reported before, we present this extremely rare case of primary malignant glucagonoma of the liver. Correspondence to: Takuyuki kaTabami, m.d., division of metabolism and endocrinology, department of medicine, st. marianna university school of medicine, 2-16-1 sugao, miyamaeku, kawasaki 216-8511, Japan. e-mail: t2kataba@marianna-u.ac.jp NEUROENDOCRINE tumors (NeTs) are diagnosed on the basis of typical histological findings and the diagnosis is confirmed by diffuse positive staining for neuroendocrine cell markers [1]. NeTs are not only found in endocrine glands, but can occur throughout the body, including the gastrointestinal tract, lungs, and liver. Glucagonoma is one of the NeTs, and it almost always arises from the islets of the pancreas, with extrapancreatic tumors accounting for less than 1% of all cases [2]. Primary hepatic glucagonoma has not been reported before. here we present an extremely rare case of primary malignant hepatic glucagonoma that was confirmed at autopsy. Case ReportThe patient was a 73-year-old woman. at the age of 63 years, distal gastrectomy (billroth ii) was performed at another hospital, but we could not obtain any information with regard to the underlying disease. she had a history of heart failure at the age of 64 years. since then, a liver mass had been found (suspected hemangioma) and myelodysplastic syndrome had occurred at 71 years of age. diabetes mellitus had been diagnosed at our hospital when she was aged 69 years, but she had been followed without any anti-diabetic therapy. since her postprandial glucose level increased to 379 mg/dl, she was admitted to our ward for the treatment of diabetes when she was 73 years old.Physical examination on admission, she was very lean (bmi 12.0 kg/m 2 ). blood pressure was normal (116/89 mmhg), heart rate NOTE

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“…Hemorrhage and necrosis were noted. Immunohistochemically, the cells were positive for glucagon, chromogranin A., and synaptophysin (Obi et al 2009).…”

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