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

Citation:
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 www.tinyurl.com/y3atbevf

Abstract:

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.

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