![]() |
JOURNAL TOOLS |
eTOC |
To subscribe |
Submit an article |
Recommend to your librarian |
ARTICLE TOOLS |
Reprints |
Permissions |

YOUR ACCOUNT
YOUR ORDERS
SHOPPING BASKET
Items: 0
Total amount: € 0,00
HOW TO ORDER
YOUR SUBSCRIPTIONS
YOUR ARTICLES
YOUR EBOOKS
COUPON
ACCESSIBILITY
CASE REPORT
Minerva Endocrinologica 2004 March;29(1):19-24
Copyright © 2004 EDIZIONI MINERVA MEDICA
language: English
Von Recklinghausen’s neurofibromatosis associated with duodenal somatostatinoma. A case report and review of the literature
Cappelli C., Agosti B., Braga M., Cumetti D., Gandossi E., Rizzoni D., Agabiti Rosei E.
Somatostatin producing duodenal carcinoids are rare, comprising a mere 2% of small bowel carcinoids and 5-10% of all duodenal tumors. Since the 1st case described by Kaneko in 1979 more than 50 cases have been reported in the world literature. From these reports, it is gradually emerging that duodenal somatostatinomas may show a strong association with Von Recklinhausen's neurofibromatosis (VRNF) as a distinct neuroendocrine syndrome. A case of a patient affected by VRNF associated with duodenal somatostatinoma with consequent obstructive jaundice is reported. The authors discuss the characteristics of these tumors and review the literature. A total of 27 patients with Von Recklinghausen's disease associated with immunohistologically proved duodenal somatostatinoma have been identified and compared with 29 duodenal somatostatinoma not associated with VRNF, and with 32 cases of pancreatic somatostatinomas.