Journal article
Risk group accurately predicts outcome in primary extremity non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) in patients <30 years of age: Findings from Children’s Oncology Group study ARST0332
Journal of clinical oncology, Vol.36(15_suppl), pp.10546-10546
05/20/2018
DOI: 10.1200/JCO.2018.36.15_suppl.10546
Abstract
Background: Data are limited on primary extremity NRSTS in pediatric patients. Methods: This ARST0332 subset analysis evaluated the clinical features and outcomes of extremity (including shoulder/hip girdle) NRSTS in < 30 year old patients risk-stratified to receive surgery +/- radiotherapy +/- chemotherapy based on clinicopathologic characteristics including tumor size, grade, extent of surgery, and presence of metastases. Other variables analyzed included age, gender, race, tumor invasiveness, and depth. Results: Primary extremity tumors comprised 297/551 cases (100 upper and 197 lower extremity) treated with surgery only (n = 137), adjuvant radiotherapy (n = 9), adjuvant chemoradiotherapy (n = 38), or neoadjuvant chemoradiotherapy followed by surgery if feasible (n = 113). Extremity site correlated with older age (p = 0.0059), diameter < 5 cm (p = 0.0421), lower grade (p < 0.0001), superficial location (p = 0.0314), and non-invasiveness (p < 0.0001). However, among patients with extremity tumors, older patients more commonly had > 5 cm (p < 0.0001), deep (p = 0.0010), invasive (p = 0.0037) tumors, and distant metastases (p = 0.0003). Estimated 5-year cumulative incidence of local and distant recurrence was 5% and 20%, respectively. Five-year overall survival (OS) for low-, intermediate- and high-risk patients was 96.7%, 78%, and 25.4%, respectively, and was not statistically significantly different than for non-extremity tumors. Older age, size > 5 cm, invasiveness, and higher risk group predicted inferior event-free survival; only higher risk group predicted inferior OS. Conclusions: Although extremity NRSTS are more likely to have low-risk features (size < 5 cm, lower grade, superficial location, non-invasiveness) compared to non-extremity tumors, their outcomes are similar with risk-adapted therapy. Older patients had more high-risk features. Risk group was the most important predictor of overall survival.
Details
- Title: Subtitle
- Risk group accurately predicts outcome in primary extremity non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) in patients <30 years of age: Findings from Children’s Oncology Group study ARST0332
- Creators
- Sara Regina Kreimer - Stanford University School of Medicine, Stanford, CADixin Shen - University of Southern California Keck School of Medicine, Los Angeles, CASheri L Spunt - Stanford University School of Medicine, Stanford, CASimon C Kao - University of Iowa College of Medicine, Iowa City, IADavid Parham - University of Southern California Keck School of Medicine, Los Angeles, CASarah S Donaldson - Stanford University School of Medicine, Stanford, CAMatthew Ladra - Johns Hopkins University School of Medicine, Baltimore, MDMichael J Monument - University of Calgary, Calgary, AB, CanadaDonald A Barkauskas - University of Southern California Keck School of Medicine, Los Angeles, CADouglas S Hawkins - Seattle Children’s Hospital, University of Washington, Fred Hutchinson Cancer Research Center, Seattle, WAR. Lor Randall - Huntsman Cancer Institute at the University of Utah, Salt Lake City, UT
- Resource Type
- Journal article
- Publication Details
- Journal of clinical oncology, Vol.36(15_suppl), pp.10546-10546
- DOI
- 10.1200/JCO.2018.36.15_suppl.10546
- ISSN
- 0732-183X
- eISSN
- 1527-7755
- Language
- English
- Date published
- 05/20/2018
- Academic Unit
- Radiology; Stead Family Department of Pediatrics
- Record Identifier
- 9984051975902771
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