A Case of Multiple Endocrine Neoplasia Type 1 Pancreatic Glucagonoma Presenting Zollinger-Ellison syndrome by Gastrin-Producing Hepatic Tumor

Seiichi Shinji, Noritake Tanaka, Kiyonori Furukawa, Kimiyoshi Yokoi, Tomoko Seya, Takayuki Aimoto, Yoshikazu Kanazawa, Masato Yoshioka, Yoshiharu Oaki¹⁾ and Takashi Tajiri²⁾

Department of Surgery and Department of Pathology¹⁾, Nippon Medical School, Chiba Hokusoh Hospital
Department of Surgery, Nippon Medical School²⁾

We report the case of a patient with multiple endocrine neoplasia type 1 (MEN-1), with an gastrin-producing hepatic tumor and pancreatic glucagonoma. A 55-year-old woman admitted for abdominal pain and nausea was found to have a severe duodenal ulcer and elevated gastrin, glucagon, parathyroid hormone, and calcium. Abdominal computed tomography (CT) showed a high-density area suggesting a 4 cm neuroendocrine neoplasm in the pancreatic tail and a high-density area suggesting a 2 cm metastatic lesion in the left lateral hepatic segment. Neck CT showed a parathyroid tumor. Based on a preoperative diagnosis of MEN-1 with Zollinger-Ellison syndrome and pancreatic gastrinoma with liver metastasis, she underwent distal pancreatectomy and left lateral segment hepatectomy with splenectomy and cholecystectomy. The pathological diagnosis was gastrin-producing hepatic tumor and pancreatic glucagonoma. Both tumors showed different hormone production profiles and there were many microadenoma-like lesions in the pancreas. Genetic examination showed a point mutation in MEN-1.

Key words
multiple endocrine neoplasia type 1, gastrinoma, glucagonoma

Reprint requests
Seiichi Shinji Department of Surgery, Chiba-Hokusoh Hospital, Nippon Medical School
1715 Kamagari, Inbamura, Inba-gun, 270-1694 JAPAN

Accepted
December 19, 2007

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