A case of cholangiocarcinoma associatd with neurofibromatosis type 1.
- Author:
Yun Ah KIM
1
;
Eun Woo LEE
;
Do Hyun KIM
;
Dong Goo KANG
;
Hyeuk PARK
;
Myung Won KANG
;
Yeun Keun LIM
;
Hyang Soon YEO
Author Information
1. Department of Internal Medicine, KwangJu Christian Hospital, Kawang Ju, Korea.
- Publication Type:Original Article
- Keywords:
Neurofibromatoses;
Cholangiocarcinoma
- MeSH:
Cafe-au-Lait Spots;
Carcinoid Tumor;
Cholangiocarcinoma*;
Ganglioneuroma;
Incidence;
Leiomyoma;
Neural Crest;
Neurofibroma;
Neurofibromatoses*;
Neurofibromatosis 1*
- From:Korean Journal of Medicine
2000;59(4):463-466
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Neurofibromatosis is characterized by multiple cutaneous neurofibromas, cafe-au-lait spots and a broad spectrum of clinical, pathologic and radiologic finding. The incidence of malignant neoplasms complicating neurofibromatosis has never been satisfactorily estabilished ; estimates range from 3% to 15%. The malignant tumors are usually derived from neural crest tissue. Gastrointestinal involvement appears to be rare and usually consists of neurofibromas, ganglioneuromas and leiomyomas. Hepatobiliary involvement in neurofibromatosis is rare and mostly located at the periampullary region. Most of theses tumors are carcinoids or less frequently neurofibromas. The authors report a case of peripheral cholangiocarcinoma associated with neurofibromatosis type 1.
