[Somatostatin-producing endocrine pancreatic tumor in Recklinghausen's neurofibromatosis. Case report and literature review]

Abstract

Somatostatin-producing tumors of the pancreas were first described in 1977. In 1983 a syndrome involving multiple endocrine neoplasias (MEN) was named type III A. This syndrome consists of carcinoid of the duodenum, often producing somatostatin, and von Recklinghausen's disease (neurofibromatosis) or pheochromocytoma. The case is reported of a 62-year-old man with familial neurofibromatosis and a tumor of the head of the pancreas spreading into pars II of the duodenum. After Whipple's duodenopancreatectomy the patient exhibited no further symptoms. Immunohistochemistry served to prove the production of somatostatin and small amounts of calcitonin in the tumor.