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  • Title: Malignant duodenal somatostatinoma presenting in association with von Recklinghausen disease.
    Author: Patel VG, Henderson VJ, Fairweather DA, Fortson JK, Weaver WL, Martin DM, Lyons R, Hamami A.
    Journal: Am Surg; 2003 Dec; 69(12):1077-82. PubMed ID: 14700294.
    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.
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