Botryoid rhabdomyosarcoma of the biliary tract

We report the clinical and pathologic features of five children wih a botyroid rhabdomyosarcoma of the biliary tract. There were three boys and two girls ranging in age from 11/2 to 51/2 years at diagnosis. Obstructive jaundice was the usual presenting sign, often with fever and hepatomegaly. Of four tumors involving the common bile ducts, two extended into porta hepatis and adjacent liver parenchyma; the fifth tumor arose in the wall of the cystic duct. Each had a typical botryoid (grape-like) configuration with a cambium layer of tumor cells beneath biliary epithelium. Rhabdomyoblasts were identified in all cases and confirmed by ultrastructure in one. Our data, along with review of other cases reported in the English literature, indicate a rather guarded prognosis. Although metastases have developed in 40% of cases, death was usually due to the effects of local invasion by tumor. Aggressive adjuvant therapy currently in use for childhood rhabdomyosarcomas may lessen the high mortality associated with this tumor.