A Case of von Recklinghausen's Disease Complicated by Carcinoma of the Papilla of Vater and Multiple Gastrointestinal Stromal Tumors of the Jejunum

Shin-ichi Ikuta, Chiaki Yasui, Tsukasa Aihara, Hidenori Yoshie, Toshihiro Bandoh, Masao Mitsunobu, Ayako Sugihara* and Naoki Yamanaka

Department of Surgery and Department of Pathology*, Meiwa General Hospital

A 71-year-old man with von Recklinghausen's disease and referred for general fatigue was found in gastroduodenoscopy and biopsy specimen pathology to have adenocarcinoma of the papilla of Vater, necessitating pylorus-preserving pancreatoduodenectomy and partial jejunal resection due to multiple tumors found on the serosal surface of the jejunum. Immunohistologically, the jejunal tumors were diagnosed as uncommitted gastrointestinal stromal tumors (GISTs). Von Recklinghausen's disease frequently accompanies neurogenic tumors, but is rarely associated with nonneurogenic tumors. Carcinoma of the papilla of Vater and jejunal GISTs associated with von Recklinghausen's disease is rare.

Key words
von Recklinghausen's disease, carcinoma of the papilla of Vater, gastrointestinal stromal tumor

Reprint requests
Shin-ichi Ikuta Department of Surgery, Meiwa General Hospital
4-31 Agenaruo-cho, Nishinomiya, 663-8186 JAPAN

Accepted
October 25, 2006

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