VONRECKLINGHAUSENS-DISEASE ASSOCIATED WITH DUODENAL SOMATOSTATINOMA -CONTRAST OF DUODENAL VERSUS PANCREATIC SOMATOSTATINOMAS

A case of Von Recklinghausen's disease with duodenal somatostatinoma i s reported. A search of the world's literature revealed 27 patients wi th Von Recklinghausen's disease associated with an immunohistologicall y proved duodenal somatostatinoma. Twenty-nine cases of duodenal somat ostatinoma not associated with Von Recklinghausen's disease and 32 cas es of pancreatic somatostatinomas have been identified for comparison. While their histology may be similar in many respects, the duodenal a nd pancreatic somatostatinomas show significant differences, especiall y in hormonal and growth behaviors. In contrast to its pancreatic coun terpart, the duodenal somatostatinoma is frequently associated with Vo n Recklinghausen's disease, is seldom associated with a recognizable ' 'somatostatin syndrome,'' often contains psammoma bodies, and is less frequently associated with demonstrable metastases at the time of oper ation. (C) 1995 Wiley-Liss, Inc.