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Randomized Controlled Trial
. 2020 May;67(5):e28243.
doi: 10.1002/pbc.28243. Epub 2020 Mar 2.

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

Jamie M Aye  1 , Yueh-Yun Chi  2 , Jing Tian  2 , Erin R Rudzinski  3 , Odion T Binitie  4 , Roshni Dasgupta  5 , Suzanne L Wolden  6 , Douglas S Hawkins  7 , Abha A Gupta  8
Affiliations
Randomized Controlled Trial

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

Jamie M Aye et al. Pediatr Blood Cancer. 2020 May.

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.

Keywords: FOXO1; radiation; rhabdomyosarcoma.

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Conflict of interest statement

CONFLICTS OF INTEREST

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Outcomes by use of radiotherapy
FIGURE 2
FIGURE 2
Event-free survival by FOXOl-fusion status and use of radiotherapy
See this image and copyright information in PMC

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