J Chir (Paris) 1987 Mar;124(3):161-4

Cholangiocarcinoma associated with Caroli's disease. Apropos of a case. Review of the literature

Etienne JC, Bouillot JL, Alexandre JH

Abstract
This is a report of a rarely observed occurrence of complication in Caroli's
disease, i.e. the emergence of a cholangiocarcinoma in the course of the
disease. There are 20 observations reported in the literature, and the
hypotheses of pathogenesis set forth are reviewed. The frequency of a malignant
transformation during the course of Caroli's disease is compared to the
frequencies of other hepatic-biliary congenital cystic tumours. The excision of
the cyst resulted in an appreciable period of survival for the patient