Korean J Med.  2000 Oct;59(4):463-466.

A case of cholangiocarcinoma associatd with neurofibromatosis type 1

• Kim YA
• Lee EW
• Kim DH
• Kang DG
• Park H
• Kang MW
• Lim YK
• Yeo HS

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.

Keyword

Neurofibromatoses; Cholangiocarcinoma

MeSH Terms

Cafe-au-Lait Spots
Carcinoid Tumor
Cholangiocarcinoma*
Ganglioneuroma
Incidence
Leiomyoma
Neural Crest
Neurofibroma
Neurofibromatoses*
Neurofibromatosis 1*