Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease.
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 atypical case of malignant duodenal somatostatinoma presenting with somatostatinoma syndrome in association with type-I neurofibromatosis.
Patel
Vijaykumar G
VG
Department of Surgery, Morehouse School of Medicine, Atlanta, Georgia 30310, USA.
Henderson
Vernon J
VJ
Fairweather
David A
DA
Fortson
James K
JK
Weaver
William L
WL
Martin
David M
DM
Lyons
Ralph
R
Hamami
Amir
A
D002760:Cholangiopancreatography, Endoscopic Retrograde; D015897:Comorbidity; D004379:Duodenal Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000000981:diagnostic imaging / Q000453:epidemiology*; D013005:Somatostatinoma / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000473:pathology