Embryonal rhabdomyosarcoma within a choledochal cyst.

The authors describe a 6-year-old girl in whom they found an embryonal rhabdomyosarcoma in a choledochal cyst; they found no previous reports of such an association. The patient presented with recurrent jaundice and loss of weight and appetite. A large abdominal mass was palpable. Ultrasonography and computed tomography revealed a large subhepatic mass, which contained multiple septations and mural masses delineating cystic areas. The common hepatic duct opened directly into the mass, and the intrahepatic radicles were dilated. Intraoperative cholangiography revealed that the cysts in the mass were continuous with the hepatic and intrahepatic radicles. The mass was completely excised, and a Roux-en-Y hepaticojejunostomy was performed. Postoperative adjunct chemotherapy and radiotherapy were instituted.