Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: Results from the Intergroup Rhabdomyosarcoma Study IV

K. Scott Baker, James R. Anderson, Michael P. Link, Holcombe E. Grier, Stephen J. Qualman, Harold M. Maurer, John C. Breneman, Eugene S. Wiener, William M. Crist

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Abstract

Purpose: To compare failure-free survival (FFS) and survival for patients with local or regional embryonal rhabdomyosarcoma treated on the Intergroup Rhabdomyosarcoma Study (IRS)-IV with that of comparable patients treated on IRS-III. Patients and Methods: Patients were retrospectively classified as low- or intermediate-risk. Low-risk patients were defined as those with primary tumors at favorable sites, completely resected or microscopic residual, or orbit/eyelid primaries with gross residual disease and tumors less than 5 cm at unfavorable sites but completely resected. Intermediate-risk patients were all other patients with local or regional tumors. Results: Three-year FFS improved from 72% on IRS-III to 78% on IRS-IV for patients with intermediate-risk embryonal rhabdomyosarcoma (P = .02). Subset analysis revealed two groups that benefitted most from IRS-IV therapy. FFS at 3 years for patients with resectable node-positive or unresectable (group III) embryonal rhabdomyosarcoma arising at certain favorable sites (head and neck [not Orbit/eyelid or parameningeal] and genitourinary [not bladder or pro state]) improved from 72% on IRS-III to 92% on IRS-IV (P = .01). Similarly, 3-year FFS for patients with completely resected tumor or with only microscopic disease remaining (group I or II) at unfavorable sites improved from 71% on IRS-III to 86% on IRS-IV (P = .04). Only patients with unresectable embryonal rhabdomyosarcoma (group III) at unfavorable sites had no improvement in outcome on IRS-IV (3-year FFS for IRS-III and IRS-IV, 72% and 75%, respectively; P = .31). Conclusion: IRS-IV therapy benefitted certain subgroups of patients with intermediate-risk embryonal rhabdomyosarcoma. A doubling of the intensity of cyclophosphamide (or ifosfamide equivalent) dosing per cycle between IRS-III and IRS-IV is thought to be a key contributing factor for this improvement. (C) 2000 by American society of Clinical Oncology.

Original languageEnglish (US)
Pages (from-to)2427-2434
Number of pages8
JournalJournal of Clinical Oncology
Volume18
Issue number12
DOIs
StatePublished - Jun 2000

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ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Baker, K. S., Anderson, J. R., Link, M. P., Grier, H. E., Qualman, S. J., Maurer, H. M., Breneman, J. C., Wiener, E. S., & Crist, W. M. (2000). Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: Results from the Intergroup Rhabdomyosarcoma Study IV. Journal of Clinical Oncology, 18(12), 2427-2434. https://doi.org/10.1200/JCO.2000.18.12.2427