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  • Title: [Cholangiocarcinoma associated with Caroli's disease. Apropos of a case. Review of the literature].
    Author: Etienne JC, Bouillot JL, Alexandre JH.
    Journal: J Chir (Paris); 1987 Mar; 124(3):161-4. PubMed ID: 3034936.
    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.
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