Local control for intermediate-risk rhabdomyosarcoma: Results from D9803 according to histology, group, site, and size: A report from the children's oncology group Journal Article


Authors: Wolden, S. L.; Lyden, E. R.; Arndt, C. A.; Hawkins, D. S.; Anderson, J. R.; Rodeberg, D. A.; Morris, C. D.; Donaldson, S. S.
Article Title: Local control for intermediate-risk rhabdomyosarcoma: Results from D9803 according to histology, group, site, and size: A report from the children's oncology group
Abstract: Purpose To determine local control according to clinical variables for patients with intermediate-risk rhabdomyosarcoma (RMS) treated on Children's Oncology Group protocol D9803. Patients and Methods Of 702 patients enrolled, we analyzed 423 patients with central pathology-confirmed group III embryonal (n=280) or alveolar (group III, n=102; group I-II, n=41) RMS. Median age was 5 years. Patients received 42 weeks of VAC (vincristine, dactinomycin, cyclophosphamide) or VAC alternating with VTC (T = topotecan). Local therapy with 50.4 Gy radiation therapy with or without delayed primary excision began at week 12 for group III patients. Patients with group I/II alveolar RMS received 36-41.4 Gy. Local failure (LF) was defined as local progression as a first event with or without concurrent regional or distant failure. Results At a median follow-up of 6.6 years, patients with clinical group I/II alveolar RMS had a 5-year event-free survival rate of 69% and LF of 10%. Among patients with group III RMS, 5-year event-free survival and LF rates were 70% and 19%, respectively. Local failure rates did not differ by histology, nodal status, or primary site, though there was a trend for increased LF for retroperitoneal (RP) tumors (P=.12). Tumors ≥5 cm were more likely to fail locally than tumors <5 cm (25% vs 10%, P=.0004). Almost all (98%) RP tumors were ≥5 cm, with no difference in LF by site when the analysis was restricted to tumors ≥5 cm (P=.86). Conclusion Local control was excellent for clinical group I/II alveolar RMS. Local failure constituted 63% of initial events in clinical group III patients and did not vary by histology or nodal status. The trend for higher LF in RP tumors was related to tumor size. There has been no clear change in local control over RMS studies, including IRS-III and IRS-IV. Novel approaches are warranted for larger tumors (≥5 cm). © 2015 Elsevier Inc.
Keywords: child; event free survival; cancer surgery; excision; primary tumor; survival rate; treatment failure; major clinical study; cancer recurrence; cancer growth; drug dose reduction; cancer radiotherapy; topotecan; follow up; local therapy; cyclophosphamide; vincristine; oncology; histology; distant metastasis; childhood cancer; electron therapy; infant; tumors; newborn; cancer size; dactinomycin; brachytherapy; retroperitoneal tumor; local control; cancer control; rhabdomyosarcoma; patient treatment; embryonal rhabdomyosarcoma; intermediate risk patient; phase 2 clinical trial (topic); local failure; intermediate risks; radiation dose reduction; human; priority journal; article; clinical groups; group protocols
Journal Title: International Journal of Radiation Oncology, Biology, Physics
Volume: 93
Issue: 5
ISSN: 0360-3016
Publisher: Elsevier Inc.  
Date Published: 2015-12-01
Start Page: 1071
End Page: 1076
Language: English
DOI: 10.1016/j.ijrobp.2015.08.040
PROVIDER: scopus
PUBMED: 26581144
PMCID: PMC5147527
DOI/URL:
Notes: Export Date: 2 December 2015 -- Source: Scopus
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  1. Suzanne L Wolden
    560 Wolden