Do Children and Adolescents with Completely Resected Alveolar Rhabdomyosarcoma Require Adjuvant Radiation? A Report from the Children's Oncology Group

Overview

Journal Pediatr Blood Cancer

Specialties Hematology
Oncology
Pediatrics

Date 2020 Mar 4

PMID 32124549

Citations 1

Authors

Jamie M Aye

Yueh-Yun Chi

Jing Tian

Erin R Rudzinski

Odion T Binitie

Roshni Dasgupta

Suzanne L Wolden

Douglas S Hawkins

Abha A Gupta

Affiliations

Soon will be listed here.

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.

Citing Articles

Clinical features and prognosis of paediatric rhabdomyosarcoma with bone marrow metastasis: a single Centre experiences in China.

Huang C, Jian B, Su Y, Xu N, Yu T, He L BMC Pediatr. 2021; 21(1):463.

PMID: 34670517 PMC: 8529763. DOI: 10.1186/s12887-021-02904-9.

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