Malignant Duodenal Somatostatinoma Presenting in Association with von Recklinghausen Disease

Patel, Vijaykumar G.; Henderson, Vernon J.; Fairweather, David A.; Fortson, James K.; Weaver, William L.; Martin, David M.; Lyons, Ralph; Hamami, Amir
December 2003
American Surgeon;Dec2003, Vol. 69 Issue 12, p1077
Academic Journal
Somatostatinomas are extremely rare periampullary malignant neuroendocrine tumors that may be associated with von Recklinghausen disease or type-I neurofibromatosis. Duodenal somatostatinomas are distinguished from pancreatic somatostatinomas by their frequent association with type-I neurofibromatosis and typically absence of somatostatinoma syndrome. We report a very rare and a typical case of malignant duodenal somatostatinoma presenting with somatostatinoma syndrome in association with type-I neurofibromatosis.


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