Cy. Mao et al., VONRECKLINGHAUSENS-DISEASE ASSOCIATED WITH DUODENAL SOMATOSTATINOMA -CONTRAST OF DUODENAL VERSUS PANCREATIC SOMATOSTATINOMAS, Journal of surgical oncology, 59(1), 1995, pp. 67-73
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.