Survival of patients with orbital and eyelid rhabdomyosarcoma treated on Children's Oncology Group studies from 1997 to 2013: A report from the Children's Oncology Group

Jonathan Metts*, Wei Xue, Zhengya Gao, Ralph Ermoian, Julie A. Bradley, Michael A. Arnold, Roshni Dasgupta, Rajkumar Venkatramani, David Walterhouse

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Orbital rhabdomyosarcoma (ORMS) commonly presents as low-risk disease (stage 1, group I–III, embryonal RMS) with excellent outcome. Long-term follow-up of patients with low-risk ORMS and outcomes of less common subgroups of ORMS treated on recent Children's Oncology Group (COG) trials have not been reported. Methods: Patients with ORMS enrolled on COG trials from 1997 to 2013 were identified. Demographic information and disease characteristics were collected. Outcomes were determined for the following subgroups: 1) low-risk ORMS, 2) resected (group I/II) low-risk ORMS, 3) non–low-risk ORMS, and 4) recurrent ORMS. Event-free survival (EFS) and overall survival (OS) were estimated using the Kaplan–Meier method. ResultsThe authors identified 218 patients with ORMS. Most tumors were embryonal/botryoid (n = 169; 77.5%), <5 cm (n = 213; 97.7%), group III (n = 170; 78.0%), and without lymph node involvement (N0; n = 215; 98.6%). For 192 patients with low-risk ORMS, the 10-year EFS and OS rates were 85.5% (95% confidence interval [CI], 77.0%–94.0%) and 95.6% (95% CI, 90.8%–100.0%), respectively. Those with group I/II low-risk ORMS (n = 5 in group I; n = 39 in group IIA) had 10-year EFS and OS rates of 88.0% (95% CI, 72.6%–100.0%) and 97.6% (95% CI, 90.0%–100.0%), respectively. Twenty-six patients with non–low-risk ORMS had 5-year EFS and OS rates of 88.5% (95% CI, 75.6%–100.0%) and 95.8% (95% CI, 87.7%–100.0%), respectively. For patients with recurrent ORMS, the 10-year OS rate from the time of recurrence was 69.4% (95% CI, 50.0%–88.8%). Conclusions: Patients with ORMS had favorable long-term survival outcomes on COG studies from 1997 to 2013, including those who had both low-risk and non–low-risk disease. A significant proportion of patients with recurrent ORMS may achieve long-term survival.

Original languageEnglish (US)
Pages (from-to)1735-1743
Number of pages9
Journalcancer
Volume129
Issue number11
DOIs
StatePublished - Jun 1 2023

Keywords

  • orbital
  • pediatric
  • rhabdomyosarcoma
  • sarcoma
  • survival

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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