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  • Title: Periampullary neoplasms in von Recklinghausen's disease.
    Author: Klein A, Clemens J, Cameron J.
    Journal: Surgery; 1989 Nov; 106(5):815-9. PubMed ID: 2510333.
    Abstract:
    A case of neurofibromatosis is reported in a patient who was initially seen with obstructive jaundice caused by a carcinoid tumor originating from the ampulla of Vater. An extensive review of the literature suggests that patients with von Recklinghausen's disease are at significant risk for periampullary neoplasms of neural-crest and non-neural-crest origin. The tendency of those tumors to arise from the ampulla of Vater has diagnostic and therapeutic implications.
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