Glucagonoma syndrome: report of one case

A 60-year-old male patient was admitted due to protracted systemic pruritus and erythema for 3 years without an obvious dermatologic cause, which exacerbated in the past 3 months, along with weight loss and diarrhoea. He had significant fasting hyperglycemia and dramatically elevated serum glucagon level in biochemical examination. Elevated chromogranin A (CgA) and neuron-specific enolase (NSE) levels were also manifested, whereas carbohydrate antigen 19-9 (CA-199) and carcinoembryonic antigen (CEA) were unremarkable. His skin presented with necrolytic migratory erythema (NME), anemia and other morphologies. Both ultrasound and computed tomography imaging revealed a space-occupying lesion in the distal pancreas and inferior vena caval thrombosis. Preoperative preparations included parenteral nutrition support, somatostatin analog treatment and inferior vena cava filter placement. Then, the patient had performed distal pancreatectomy with splenectomy with regional lymph node dissection to achieve primary R0/R1 resection. Pathology indicated a neuroendocrine tumor in distal pancreas, with spleen involved and regional lymph nodes metastases. Immunohistochemistry revealed that neuroendocrine tumor areas were diffusely positive for SSR2, SSR5, SYN and CgA. Postoperatively, skin symptoms disappear and cured without reoccurrence and blood glucose levels returned to the normal range. The postoperative surveillance was indicated after surgery for their high relapse rate and malignant behavior.