A Successful Case of Neuroendocrine Tumor of the Pancreas Treated by Streptozotocin

A 49-year-old woman was admitted because of nausea and epigastralgia. Esophagogastrointestinal fiberscopy revealed a gastric varix located in the fundus and a moderate amount of coagulum in the stomach without evidence of active bleeding. On the 2nd hospital day, she went into hypovolemic shock due to rupture of the gastric varix. CT scanning demonstrated a pancreatic body tumor involving the splenic vein, which resulted in left-sided portal hypertension causing the gastric varix, along with multiple liver metastases. An emergency proximal partial gastrectomy was performed. A wedge biopsy of the pancreatic tumor suggested the diagnosis of a solid and papillary epithelial neoplasm. After her recovery, distal pancreatectomy with splenectomy was performed for debulking of the tumor. Intraarterial infusion of anti-cancer drugs including doxorubicin and cisplatin failed to improve the liver metastases. A preoperative biochemical study revealed an elevated serum level of gastrin (1, 560pg/ml), which increased markedly with growth of the metastatic liver tumors (>10, 000pg/ml). She frequently experienced hypoglycemic attacks due to hyperinsulinemia (150μU/ml). On histological review of surgical specimens, a final diagnosis of an islet cell tumor was established. The hyperinsulinemia could not be controlled by the administration of either Ca blockers or somatostatin analogs. Intraarterial infusion of streptozotocin was initiated, with marked improvement in hormone levels and her clinical condition. We report this case focusing on 3 major topics including the specific symptoms found at the onset of the disease, the process to the final pathological diagnosis and the treatment strategies for liver metastasis of neuroendocrine tumors.