Embryonal rhabdomyosarcoma with metastases confined to the lungs: Report from the CWS Study Group

Background Embryonal rhabdomyosarcoma [RME] is the most common pediatric soft tissue sarcoma. Whereas the prognosis of localized rhabdomyosarcoma has improved, it remains poor for metastatic disease. Methods We analyzed RME‐patients with isolated pulmonary metastases [PRME] treated in four consecutive CWS‐trials. Treatment included multiagent chemotherapy and local treatment of the primary tumor. Therapy of lung metastases after induction chemotherapy depended on response and individual decisions. Results Twenty‐nine patients 5 cm. In addition to conventional chemotherapy, seven patients received high‐dose treatment and eight patients oral metronomic chemotherapy. The lung metastases were in remission after induction chemotherapy in 22 individuals. 19 patients received no local treatment of metastases; 3 patients had pulmonary metastasectomy and lung radiation was administered to 9 individuals. In total, 24/29 patients achieved a complete remission [CR]. Actuarial 5‐year event‐free and overall survival for all patients was 37.9 ± 18% and 48.7 ± 18%, respectively; it was 45.8 ± 20% and 58.3 ± 20% for the 24 patients who achieved a CR. Local treatment of metastases had no impact on the failure pattern. Younger age, good response, achievement of CR and maintenance‐treatment were favorable prognostic factors in univariate analysis. Conclusions Children with PRME have a fair prognosis. Local treatment of metastases did not improve outcome in our sample. Metronomic treatment may be an attractive option for PREM‐patients. [correction made here after initial online publication]. Pediatr Blood Cancer 2011;56:725–732. © 2010 Wiley‐Liss, Inc.