Synchronous Biliary Tract Cancers in a Patient with von Recklinghausen Disease.
- Author:
Tae Sung SOHN
1
;
Yong Il KIM
;
Jong Riul LEE
;
Jae Hyung NOH
;
Seong Ho CHOI
;
Byung Boong LEE
;
Young Lyun OH
Author Information
1. Departments of General Surgery, Samsung Medical Center, College of Medicine, Sung Kyun Kwan University.
- Publication Type:Original Article
- Keywords:
Neurofibromatosis;
von Recklinghausen disease;
Adenocarcinoma;
Biliary cancer
- MeSH:
Adenocarcinoma;
Adenoma;
Ampulla of Vater;
Biliary Tract Neoplasms*;
Biliary Tract*;
Carcinoid Tumor;
Carcinoma, Squamous Cell;
Gallbladder;
Ganglioneuroma;
Humans;
Leiomyoma;
Liver;
Neural Crest;
Neurofibroma;
Neurofibromatoses;
Neurofibromatosis 1*;
Pancreaticoduodenectomy;
Sarcoma
- From:Journal of the Korean Surgical Society
1998;54(4):587-594
- CountryRepublic of Korea
- Language:Korean
-
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).
