Do children and adolescents with completely resected alveolar rhabdomyosarcoma require adjuvant radiation? A report from the Children's Oncology Group Journal Article


Authors: Aye, J. M.; Chi, Y. Y.; Tian, J.; Rudzinski, E. R.; Binitie, O. T.; Dasgupta, R.; Wolden, S. L.; Hawkins, D. S.; Gupta, A. A.
Article Title: Do children and adolescents with completely resected alveolar rhabdomyosarcoma require adjuvant radiation? A report from the Children's Oncology Group
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. © 2020 Wiley Periodicals, Inc.
Keywords: cancer survival; child; clinical article; controlled study; event free survival; cancer surgery; overall survival; cancer combination chemotherapy; drug dose reduction; cancer patient; radiation dose; topotecan; radiation; tumor volume; randomized controlled trial; cyclophosphamide; vincristine; rhabdomyosarcoma; adjuvant radiotherapy; transcription factor fkhr; surgical patient; low risk patient; foxo1; human; male; female; priority journal; article; pediatric patient
Journal Title: Pediatric Blood and Cancer
Volume: 67
Issue: 5
ISSN: 1545-5009
Publisher: Wiley Periodicals, Inc  
Date Published: 2020-05-01
Start Page: e28243
Language: English
DOI: 10.1002/pbc.28243
PUBMED: 32124549
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 1 May 2020 -- Source: Scopus
Altmetric
Citation Impact
MSK Authors
  1. Suzanne L Wolden
    453 Wolden