Aggressive Pediatric Adrenal Cortical Carcinoma With a Novel Translocation t(20;22) and intergenic fusion of MN1 and ZNF341: A Case Report and Literature Review

Caroli disease involves several different entities in contrast to Caroli syndrome. We herein report two cases of congenital localized dilatation of the intrahepatic bile ducts exhibiting a unique morphology distinct from other types of Caroli disease. Specifically, the dilatation was limited to the second- and third-order intrahepatic bile ducts, and the dilated bile ducts abruptly connected to normal-sized ducts on both the upstream and downstream sides. Both cases are prenatally diagnosed and have been asymptomatic for nearly a decade. Nine other cases were identified in the English and Japanese literature, suggesting that this is a specific subtype of Caroli disease.