[Localized Caroli's disease associated with mucosecreting papillary adenocarcinoma. Value of hepatic resection]

A new case of carcinoma arising in Caroli's disease in a 48-year old male patient is reported. Since dilatation of the bile ducts was localized to the left lobe, left hepatectomy was performed. Histological examination showed that the tumour was a papillary mucoid adenocarcinoma. There was no recurrence of cholangitis while the patient survived. This case and a review of the literature shows that left hepatectomy suppresses repeated attacks of cholangitis in patients with Caroli's disease restricted to the left lobe, and that the incidence of carcinoma in Caroli's disease is higher than was formerly believed, which should encourage early surgical treatment when the disease is localized in the left lobe.