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

Abstract

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.