Cancer Res Treat.  2015 Jan;47(1):9-17. 10.4143/crt.2013.157.

Incidence and Survival of Pediatric Soft Tissue Sarcomas: Comparison between Adults and Children

  • 1Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Korea.
  • 2Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
  • 3Department of Orthopedic Surgery, Yonsei University College of Medicine, Seoul, Korea.
  • 4Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.


Pediatric-type sarcomas such as rhabdomyosarcoma (RMS), Ewing sarcoma (EWS), primitive neuroectodermal tumor (PNET), and desmoplastic small round-cell tumor (DSRCT) are rare in adults, with limited studies on their prognosis and optimal treatment strategies. We aimed to examine the outcome of children and adult patients with RMS, EWS, PNET, and DSRCT and relevant prognostic factors.
We retrospectively reviewed 220 pediatric-type sarcoma patients at a single institution between 1985 and 2011. Comparisons were made in order to examine differences in demographics, disease characteristics, and survival. Survival analyses were performed using the Kaplan-Meier method with log-rank tests and Cox proportional hazards models.
A total of 220 consecutive patients were identified at our institute. Median age was 15.6 years (range, 0 to 81 years) and there were 108 children (49%) and 112 adult patients (51%). According to histological classification, 106 patients (48.2%) had RMS, 60 (27.3%) had EWS, 50 (22.7%) had PNET, and 4 (1.8%) had DSRCT. With a median follow-up period of 6.6 years, the estimated median overall survival (OS) of all patients was 75 months (95% confidence interval [CI], 27.2 to 122.8 months) and median event-free survival (EFS) for all patients was 11 months (95% CI, 8.8 to 13.2 months). No significant difference in OS and EFS was observed between adults and children. In multivariate analysis, distant metastasis (hazard ratio [HR], 1.617; 95% CI, 1.022 to 2.557; p=0.040) and no debulking surgery (HR, 1.443; 95% CI, 1.104 to 1.812; p=0.012) showed independent association with worse OS.
Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.


Rhabdomyosarcoma; Ewing sarcoma; Primitive neuroectodermal tumors; Desmoplastic small round-cell tumor

MeSH Terms

Desmoplastic Small Round Cell Tumor
Disease-Free Survival
Follow-Up Studies
Multivariate Analysis
Neoplasm Metastasis
Neuroectodermal Tumors, Primitive
Proportional Hazards Models
Retrospective Studies
Sarcoma, Ewing


  • Fig. 1. (A) Comparison of overall survival (OS) in adults and children. (B) Comparison of event-free survival (EFS) in adults and children. (C) Comparison of OS according to histology in all patients. (D) Comparison of EFS according to histology in children. RMS, rhabdomyosarcoma; EWS, Ewing sarcoma; PNET, primitive neuroectodermal tumor; DSRCT, desmoplastic small round-cell tumor.

  • Fig. 2. Overall survival (OS) according to number of poor prognostic factors.

  • Appendix. 1. Comparison of event-free survival (EFS) (A) and overall survival (OS) (B) of adolescents and young adults (AYA) population with the rest of the population.



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