Cholangiocarcinomes sur syndrome des microhamartomes biliaires multiples

Two cases of cholangiocarcinoma arising in a background of multiple bile duct hamartomas are reported. In each case, the clinical and radiological investigations showed a liver tumor with no other concomitant disease. Tumors were resected, and microscopic examination revealed in one case a hilar cholangiocarcinoma, in the other case a peripheric cholangiocarcinoma, both being well-differentiated. It revealed also numerous bile duct hamartomas scattered throughout the liver. In one case, some bile duct hamartomas showed a gradual morphologic transition from benign to dysplastic and neoplastic epithelium. Patients with multiple bile duct hamartomas may have an increased risk of developing cholangiocarcinomas.