Synchronous Biliary Tract Cancers in a Patient with von Recklinghausen Disease

ABSTRACT

Von Recklinghausen's disease (VRD) is known to be associated with various neoplasms of both neural crest and nonneural crest origin. Approximately 25% of all patients with VRD have documented gastrointestinal involvement. However, most of these tumors are benign neurofibromas. Less commonly, leiomyomas, sarcomas, ganglioneuromas and carcinoids have been reported. We report two cases of synchronous biliary cancer (gallbladder & ampulla of Vater) in patients with von Recklinghausen Disease with jejunal stromal tumors. One case (F/39) was diagnosed as an adenocarcinoma of Vater's ampulla and adenoma of the gallbladder. She underwent a pancreaticoduodenectomy. Another patient (M/55) was diagnosed as an adenocarcinoma of the Vater's ampulla and squamous cell carcinoma of the gallbladder. He underwent a right lobectomy of the liver and a pancreaticoduodenectomy (HPD).