title: Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease.
abstract: 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.
authors.affiliation: Department of Surgery, Morehouse School of Medicine, Atlanta, Georgia 30310, USA.
authors.forename: Vijaykumar G
authors.initials: VG
authors.lastname: Patel
authors.forename: Vernon J
authors.initials: VJ
authors.lastname: Henderson
authors.forename: David A
authors.initials: DA
authors.lastname: Fairweather
authors.forename: James K
authors.initials: JK
authors.lastname: Fortson
authors.forename: William L
authors.initials: WL
authors.lastname: Weaver
authors.forename: David M
authors.initials: DM
authors.lastname: Martin
authors.forename: Ralph
authors.initials: R
authors.lastname: Lyons
authors.forename: Amir
authors.initials: A
authors.lastname: Hamami
mesh_terms: 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