Caroli's disease is characterized by congenital cystic dilatation of the intrahepatic bile ducts. In 7% of
casea a malignant tumor develops complicating the course of the disease.
We report the case of a 25 year-old woman in whom Caroli's disease was diagnosed at the age of 11.
From that time on, she had several episodes of cholangitis. In 1989, the abdominal ultrasound and CT
scan showed dilatation of the intrahepatic bile ducts, intracystic lithiasis and a solid mass. FNA cytology
showed a papillary adenocarcinoma. At laparotomy a tumor was found occupying both hepatic lobes,
and intraoperative US showed another two nodules in the left lobe. The tumor was considered
unresectable. Examination of the hilar lymph nodes was tumor-negative. Two weeks later, the patient
underwent an ortothopic liver transplantation (OLT). The pathological examination confirmed Caroli's
disease with adenocarcinoma. Two years after OLT, the patient is alive with normal liver function and
no evidence of disease.
To our knowledge this is the first case report of adenocarcinoma in Caroli's disease treated by OLT.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.