A case of cholangiocarcinoma associatd with neurofibromatosis type 1 / 대한내과학회지

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.