Do children and adolescents with completely resected alveolar rhabdomyosarcoma require adjuvant radiation? A report from the Children's Oncology Group
- 2 March 2020
- journal article
- research article
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 67 (5), e28243
- https://doi.org/10.1002/pbc.28243
Abstract
Background The role of adjuvant radiotherapy (RT) remains unclear in patients with localized, completely resected (group I) alveolar rhabdomyosarcoma (ARMS). Procedure Patients with group I ARMS enrolled on any one of three prior Children's Oncology Group (COG) clinical trials (D9602, D9803, or ARST0531) were analyzed. All patients received systemic chemotherapy and 36 Gy adjuvant RT (if given) to the primary site at week 12 or week 4 for D9602/D9803 and ARST0531, respectively. Results Thirty‐six patients with group I ARMS were treated on D9602 (n = 6), D9803 (n = 17), or ARST0531 (n = 13), of whom 24 (67%) were male. The median age was 4.1 years (range, 0.8‐45.8). Twenty (56%) patients had an unfavorable primary site, and 10 (28%) had tumors > 5 cm. FOXO1‐fusion status was negative, positive, and unknown in 10 (28%), 15 (42%), and 11 (30%) tumors, respectively. Twenty‐two (61%) patients received RT. Overall, the four‐year event‐free survival (EFS) and overall survival (OS) were 70.8% and 88.3%, respectively. Patients with FOXO1 positivity who received RT had superior EFS compared with those who did not (77.8% vs 16.7%; P = 0.03). Among 10 patients who were FOXO1 negative, the outcome was similar with or without RT. Conclusions Although limited by a small sample size, data from this study support the routine use of adjuvant RT in patients with FOXO1‐positive disease even after complete resection. Additionally, omitting adjuvant RT is rational for patients with FOXO1‐negative ARMS and will be prospectively investigated in the current COG trial ARST1431.Funding Information
- St. Baldrick's Foundation
This publication has 40 references indexed in Scilit:
- Local Control With Reduced-Dose Radiotherapy for Low-Risk Rhabdomyosarcoma: A Report From the Children’s Oncology Group D9602 StudyInternational Journal of Radiation Oncology*Biology*Physics, 2012
- Effect of Radiotherapy Techniques (IMRT vs. 3D-CRT) on Outcome in Patients With Intermediate-Risk Rhabdomyosarcoma Enrolled in COG D9803—A Report From the Children’s Oncology GroupInternational Journal of Radiation Oncology*Biology*Physics, 2012
- Results of the Intergroup Rhabdomyosarcoma Study Group D9602 Protocol, Using Vincristine and Dactinomycin With or Without Cyclophosphamide and Radiation Therapy, for Newly Diagnosed Patients With Low-Risk Embryonal Rhabdomyosarcoma: A Report From the Soft Tissue Sarcoma Committee of the Children's Oncology GroupJournal of Clinical Oncology, 2011
- Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984–1997: A report from the Children's Oncology GroupPediatric Blood & Cancer, 2010
- Judgement requiredPediatric Blood & Cancer, 2010
- Fusion Gene-Negative Alveolar Rhabdomyosarcoma is Clinically and Molecularly Indistinguishable from Embryonal RhabdomyosarcomaJournal of Clinical Oncology, 2010
- Vincristine, Actinomycin, and Cyclophosphamide Compared With Vincristine, Actinomycin, and Cyclophosphamide Alternating With Vincristine, Topotecan, and Cyclophosphamide for Intermediate-Risk Rhabdomyosarcoma: Children's Oncology Group Study D9803Journal of Clinical Oncology, 2009
- Chronic Health Conditions in Adult Survivors of Childhood CancerThe New England Journal of Medicine, 2006
- Group II Rhabdomyosarcoma and Rhabdomyosarcomalike Tumors: Is Radiotherapy Necessary?Journal of Clinical Oncology, 2004
- A Proportional Hazards Model for the Subdistribution of a Competing RiskJournal of the American Statistical Association, 1999