Long term survival in two children with rhabdomyosarcoma of the biliary tract.

BACKGROUND: Due to low incidence, rhabdomyosarcoma (RMS) of the biliary tract poses numerous complex management problems especially in diagnosis and local therapy. PATIENTS: The two presented patients were diagnosed by biopsy, performed by laparotomy and endoscopic retrograde cholangiopancreatography (ERCP) respectively. Nearly complete tumor regression was achieved by chemotherapy and irradiation according to the CWS-protocol. Subsequent radical resection followed directly in one patient and after local relapse in the other. Both patients are in remission 13 resp. 4 years after diagnosis with a good quality of life. CONCLUSIONS: Even in well responding biliary rhabdomyosarcomas, surgery after chemotherapy and radiotherapy seems to be necessary. Adjuvant chemotherapy should be continued after hepatic lobectomy.