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 language | English (US) |
|---|---|
| Pages (from-to) | 1735-1743 |
| Number of pages | 9 |
| Journal | cancer |
| Volume | 129 |
| Issue number | 11 |
| DOIs | |
| State | Published - Jun 1 2023 |
Funding
This work was supported (in part) by grants from St Baldrick's Foundation, Summer's Way Foundation, Maddie's Promise Foundation, Sebastian Strong Foundation, and Friends of TJ Foundation. Trials D9803 and ARST0531 were supported by National Clinical Trials Network–Children’s Oncology Group Operations Grant U10CA180886 and National Clinical Trials Network Statistics and Data Center Grant U10CA180899. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The authors thank Douglas Hawkins and Carola Arndt for their expertise on data reported from trials D9803 and ARST0531. This work was supported (in part) by grants from St Baldrick's Foundation, Summer's Way Foundation, Maddie's Promise Foundation, Sebastian Strong Foundation, and Friends of TJ Foundation. Trials D9803 and ARST0531 were supported by National Clinical Trials Network–Children’s Oncology Group Operations Grant U10CA180886 and National Clinical Trials Network Statistics and Data Center Grant U10CA180899. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The authors thank Douglas Hawkins and Carola Arndt for their expertise on data reported from trials D9803 and ARST0531.
Keywords
- orbital
- pediatric
- rhabdomyosarcoma
- sarcoma
- survival
ASJC Scopus subject areas
- Oncology
- Cancer Research
