Incidence and survival of pediatric soft tissue sarcomas

Comparison between adults and children

Sun Min Lim, Cheol Joo Yoo, Jung Woo Han, Yong Jin Cho, Soo Hee Kim, Joong Bae Ahn, SunYoung Rha, Sang Joon Shin, Hyuncheol Chung, Woo Ick Yang, Kyoo Ho Shin, Jae Kyung Rho, Hyo Song Kim

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Purpose 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. Materials and Methods 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. Results 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. Conclusion Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.

Original languageEnglish
Pages (from-to)9-17
Number of pages9
JournalCancer Research and Treatment
Volume47
Issue number1
DOIs
Publication statusPublished - 2015 Jan 1

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Sarcoma
Pediatrics
Desmoplastic Small Round Cell Tumor
Survival
Primitive Neuroectodermal Tumors
Incidence
Ewing's Sarcoma
Rhabdomyosarcoma
Confidence Intervals
Disease-Free Survival
Survival Analysis
Proportional Hazards Models
Multivariate Analysis
Demography
Neoplasm Metastasis
Therapeutics

All Science Journal Classification (ASJC) codes

  • Oncology
  • Cancer Research

Cite this

Lim, S. M., Yoo, C. J., Han, J. W., Cho, Y. J., Kim, S. H., Ahn, J. B., ... Kim, H. S. (2015). Incidence and survival of pediatric soft tissue sarcomas: Comparison between adults and children. Cancer Research and Treatment, 47(1), 9-17. https://doi.org/10.4143/crt.2013.157
Lim, Sun Min ; Yoo, Cheol Joo ; Han, Jung Woo ; Cho, Yong Jin ; Kim, Soo Hee ; Ahn, Joong Bae ; Rha, SunYoung ; Shin, Sang Joon ; Chung, Hyuncheol ; Yang, Woo Ick ; Shin, Kyoo Ho ; Rho, Jae Kyung ; Kim, Hyo Song. / Incidence and survival of pediatric soft tissue sarcomas : Comparison between adults and children. In: Cancer Research and Treatment. 2015 ; Vol. 47, No. 1. pp. 9-17.
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title = "Incidence and survival of pediatric soft tissue sarcomas: Comparison between adults and children",
abstract = "Purpose 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. Materials and Methods 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. Results 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. Conclusion Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.",
author = "Lim, {Sun Min} and Yoo, {Cheol Joo} and Han, {Jung Woo} and Cho, {Yong Jin} and Kim, {Soo Hee} and Ahn, {Joong Bae} and SunYoung Rha and Shin, {Sang Joon} and Hyuncheol Chung and Yang, {Woo Ick} and Shin, {Kyoo Ho} and Rho, {Jae Kyung} and Kim, {Hyo Song}",
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Lim, SM, Yoo, CJ, Han, JW, Cho, YJ, Kim, SH, Ahn, JB, Rha, S, Shin, SJ, Chung, H, Yang, WI, Shin, KH, Rho, JK & Kim, HS 2015, 'Incidence and survival of pediatric soft tissue sarcomas: Comparison between adults and children', Cancer Research and Treatment, vol. 47, no. 1, pp. 9-17. https://doi.org/10.4143/crt.2013.157

Incidence and survival of pediatric soft tissue sarcomas : Comparison between adults and children. / Lim, Sun Min; Yoo, Cheol Joo; Han, Jung Woo; Cho, Yong Jin; Kim, Soo Hee; Ahn, Joong Bae; Rha, SunYoung; Shin, Sang Joon; Chung, Hyuncheol; Yang, Woo Ick; Shin, Kyoo Ho; Rho, Jae Kyung; Kim, Hyo Song.

In: Cancer Research and Treatment, Vol. 47, No. 1, 01.01.2015, p. 9-17.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Incidence and survival of pediatric soft tissue sarcomas

T2 - Comparison between adults and children

AU - Lim, Sun Min

AU - Yoo, Cheol Joo

AU - Han, Jung Woo

AU - Cho, Yong Jin

AU - Kim, Soo Hee

AU - Ahn, Joong Bae

AU - Rha, SunYoung

AU - Shin, Sang Joon

AU - Chung, Hyuncheol

AU - Yang, Woo Ick

AU - Shin, Kyoo Ho

AU - Rho, Jae Kyung

AU - Kim, Hyo Song

PY - 2015/1/1

Y1 - 2015/1/1

N2 - Purpose 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. Materials and Methods 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. Results 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. Conclusion Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.

AB - Purpose 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. Materials and Methods 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. Results 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. Conclusion Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.

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