Management of Low and Intermediate Risk Adult Rhabdomyosarcoma: A Pooled Survival Analysis of 553 Patients.

Elsebaie, M. A. T., M. Amgad, A. El Kashash, A. S. Elgebaly, G. G. E. L. Ashal, E. Shash, and Z. Elsayed, "Management of Low and Intermediate Risk Adult Rhabdomyosarcoma: A Pooled Survival Analysis of 553 Patients.", Scientific reports, vol. 8, issue 1, pp. 9337, 2018 Jun 19. copy at


This is the second-largest retrospective analysis addressing the controversy of whether adult rhabdomyosarcoma (RMS) should be treated with chemotherapy regimens adopted from pediatric RMS protocols or adult soft-tissue sarcoma protocols. A comprehensive database search identified 553 adults with primary non-metastatic RMS. Increasing age, intermediate-risk disease, no chemotherapy use, anthacycline-based and poor chemotherapy response were significant predictors of poor overall and progression-free survival. In contrast, combined cyclophosphamide-based, cyclophosphamide + anthracycline-based, or cyclophosphamide + ifosfamide + anthracycline-based regimens significantly improved outcomes. Intermediate-risk disease was a significant predictor of poor chemotherapy response. Overall survival of clinical group-III patients was significantly improved if they underwent delayed complete resection. Non-parameningeal clinical group-I patients had the best local control, which was not affected by additional adjuvant radiotherapy. This study highlights the superiority of chemotherapy regimens -adapted from pediatric protocols- compared to anthracycline-based regimens. There is lack of data to support the routine use of adjuvant radiotherapy for non-parameningeal group-I patients. Nonetheless, intensive local therapy should be always considered for those at high risk for local recurrence, including intermediate-risk disease, advanced IRS stage, large tumors or narrow surgical margins. Although practically difficult (due to tumor's rarity), there is a pressing need for high quality randomized controlled trials to provide further guidance.