Rhabdomyosarcoma of the biliary tree: the case for aggressive surgery

Embryonal rhabdomyosarcoma of the bile ducts is associated with a poor prognosis because complete resection rarely is considered possible. We reviewed the literature and report our experience with the management of 3 consecutive patients. The patients were 1, 4, and 9 yr old at presentation, and had jaundice, cachexia, and an abdominal mass. The tumor arose in the common hepatic duct in 2 patients, and in the left hepatic duct in the other. All had tumor extension into both lobes of the liver and residual disease was noted after resection. Two patients were given tumor bed irradiation and all received multiple-drug chemotherapy. Two patients are alive, jaundice free, and with no evidence of disease at 9 mo and 14 yr follow-up. The other patient was a long-term survivor, but developed local recurrent tumor after cessation of chemotherapy and expired 33 mo after diagnosis. The traditional anatomic criteria of hepatic tumor resectability may not apply to botryoid sarcoma of the bile ducts. Aggressive surgery combined with the new adjuvant therapies appears to provide the best chance for a longer survival.