Mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with Zollinger-Ellison syndrome: an autopsy case with a 24-year survival period |
| |
Authors: | T Terada Y Matsunaga H Maeta K Endo S Horie T Ohta |
| |
Institution: | (1) Second Department of Pathology, Faculty of Medicine, Tottori University, Nishi-cho 86, Yonago 683-8503, Tottori, Japan Fax: +81-859-348348, JP;(2) Department of Surgery (II), Kanazawa University School of Medicine, Kanazawa, Japan, JP |
| |
Abstract: | We report an autopsy case of mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with Zollinger-Ellison
syndrome. A 38-year-old Japanese male was found to have Zollinger-Ellison syndrome and pancreatic gastrinoma, and gastrectomy
and resection of the pancreatic tumor were performed. However, hypergastrinemia persisted, and the patient died of disseminated
carcinomatosis at 62 years of age, 24 years after the onset of Zollinger-Ellison syndrome. At autopsy, the main tumor was
present in the residual pancreas, and metastases were noted in many organs. In the pancreas and other organs, ductal and endocrine
carcinoma areas were mixed and there was a gradual transition between the two. No acinar differentiation was noted. The ductal
elements were positive for mucins and carcinoembryonic antigen but negative for neuroendocrine markers, while endocrine elements
were positive for chromogranin A and synaptophysin and to a lesser extent for gastrin, but negative for mucins and carcinoembryonic
antigen. The ductal elements comprised about 30% of the tumor cells, and endocrine elements 70%. According to the revised
World Health Organization classification, our case was diagnosed as mixed ductal-endocrine carcinoma. Our case is rare because
the tumor manifested as gastrinoma with Zollinger-Ellison syndrome and the patient survived for 24 years. To the best of our
knowledge, no such case has been reported. Our case suggests that pancreatic endocrine tumors may evolve into mixed ductal-endocrine
carcinomas.
Received: 14 April 1999 / Accepted: 7 July 1999 |
| |
Keywords: | Mixed ductal-endocrine carcinoma Pancreas Gastrinoma Immunohistochemistry Zollinger-Ellison syndrome |
本文献已被 PubMed SpringerLink 等数据库收录! |
|