1. Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease.
- Author
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Patel VG, Henderson VJ, Fairweather DA, Fortson JK, Weaver WL, Martin DM, Lyons R, and Hamami A
- Subjects
- Cholangiopancreatography, Endoscopic Retrograde, Comorbidity, Duodenal Neoplasms diagnostic imaging, Duodenal Neoplasms pathology, Female, Humans, Middle Aged, Neurofibromatosis 1 diagnostic imaging, Somatostatinoma diagnostic imaging, Somatostatinoma pathology, Duodenal Neoplasms epidemiology, Neurofibromatosis 1 epidemiology, Somatostatinoma epidemiology
- 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.
- Published
- 2003