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      Periampullary neoplasms in von Recklinghausen's disease.

      Surgery
      Adult, Ampulla of Vater, Carcinoid Tumor, diagnosis, etiology, surgery, Common Bile Duct Neoplasms, Diagnosis, Differential, Humans, Male, Neurofibromatosis 1, complications

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          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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