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Title: Non-Rhabdomyosarcoma Soft Tissue Sarcomas in Children: A Surveillance, Epidemiology, and End Results Analysis Validating COG Risk Stratifications

Purpose: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group of sarcomas that encompass over 35 histologies. With an incidence of ∼500 cases per year in the United States in those <20 years of age, NRSTS are rare and therefore difficult to study in pediatric populations. We used the large Surveillance, Epidemiology, and End Results (SEER) database to validate the prognostic ability of the Children's Oncology Group (COG) risk classification system and to define patient, tumor, and treatment characteristics. Methods and Materials: From SEER data from 1988 to 2007, we identified patients ≤18 years of age with NRSTS. Data for age, sex, year of diagnosis, race, registry, histology, grade, primary size, primary site, stage, radiation therapy, and survival outcomes were analyzed. Patients with nonmetastatic grossly resected low-grade tumors of any size or high-grade tumors ≤5 cm were considered low risk. Cases of nonmetastatic tumors that were high grade, >5 cm, or unresectable were considered intermediate risk. Patients with nodal or distant metastases were considered high risk. Results: A total of 941 patients met the review criteria. On univariate analysis, black race, malignant peripheral nerve sheath (MPNST) histology, tumors >5 cm, nonextremity primary, lymph node involvement, radiation therapy, and higher risk group were associated with significantly worsemore » overall survival (OS) and cancer-specific survival (CSS). On multivariate analysis, MPNST histology, chemotherapy-resistant histology, and higher risk group were significantly poor prognostic factors for OS and CSS. Compared to low-risk patients, intermediate patients showed poorer OS (hazard ratio [HR]: 6.08, 95% confidence interval [CI]: 3.53-10.47, P<.001) and CSS (HR: 6.27; 95% CI: 3.44-11.43, P<.001), and high-risk patients had the worst OS (HR: 13.35, 95% CI: 8.18-21.76, P<.001) and CSS (HR: 14.65, 95% CI: 8.49-25.28, P<.001). Conclusions: The current COG risk group stratification for children with NRSTS has been validated with a large number of children in the SEER database.« less
Authors:
 [1] ;  [1] ;  [2] ;  [3] ;  [4] ;  [5] ; ;  [3] ;  [5] ;  [3] ;  [1]
  1. Department of Radiation Oncology, University of Colorado School of Medicine, Aurora, Colorado (United States)
  2. Department of Radiation Oncology, Billings Clinic, Billings, Montana (United States)
  3. Division of Hematology and Oncology, Department of Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States)
  4. Division of Urology, Department of Surgery, University of Colorado Denver School of Medicine, Aurora, Colorado (United States)
  5. Department of Orthopedics, University of Colorado Denver School of Medicine, Aurora, Colorado (United States)
Publication Date:
OSTI Identifier:
22458727
Resource Type:
Journal Article
Resource Relation:
Journal Name: International Journal of Radiation Oncology, Biology and Physics; Journal Volume: 92; Journal Issue: 2; Other Information: Copyright (c) 2015 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved.; Country of input: International Atomic Energy Agency (IAEA)
Country of Publication:
United States
Language:
English
Subject:
62 RADIOLOGY AND NUCLEAR MEDICINE; ANIMAL TISSUES; CHEMOTHERAPY; CHILDREN; COMPARATIVE EVALUATIONS; DIAGNOSIS; EPIDEMIOLOGY; HAZARDS; HISTOLOGY; LYMPH NODES; METASTASES; MONITORING; PATIENTS; PEDIATRICS; POPULATIONS; RADIOTHERAPY; REVIEWS; SARCOMAS; USA