Caroli's Disease: a premalignant condition?

This study reports four new cases of Caroli's disease complicated by the development of cholangiocarcinoma. Caroli's disease appears to be a premalignant condition, but early diagnosis of malignancy in the cyst wall is difficult because signs of ductal stenosis develop slowly and treatment is often based on erroneous diagnosis of recurrent cholangitis. When carcinoma is present, operative intervention makes no difference in survival. In patients with Caroli's disease, adequate intraductal and hepatic biopsies should be obtained at the first operation. These patients should be followed carefully, and malignant growth should be suspected when cholangitis occurs despite patent anastomoses. Earlier diagnosis will obviate repeated operations in patients with malignant Caroli's disease.