Abstract
We report a primary duodenal adenocarcinoma associated with von Recklinghausen's disease (VRD). A 64-year-old woman visiting our hospital due to appetite loss and epigastralgia in April 1999 was diagnosed with nonampullary primary duodenal cancer in radiological studies, upper gastrointestinal endoscopy and histological diagnosis. We conducted pancreatoduodenectomy. Histological examination showed moderately differentiated adenocarcinoma. Tumors of the neural crest origin are known to occur frequently in patients with VRD. The concurrence of intestinal cancer in this present case, however, is very rare in the Japanese literature.
