Incidence and outcomes of extremity soft-tissue sarcomas in children

Michael C. Cheung, Ying Zhuge, Relin Yang, Michael P. Ogilvie, Leonidas Koniaris, Maria M. Rodríguez, Juan E. Sola

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Background: We studied the outcomes of pediatric extremity tumors on a population scale. Methods: The Surveillance, Epidemiology, and End Results database (1973-2006) was queried for all patients under 20 y of age. Results: Overall, 1175 patients were identified. The median age at diagnosis was 12 y, but most patients were ≥10 y of age (72%, n = 842). Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79%, n = 879). The most common tissue of origin was muscle (43%, n = 474). Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140). Most patients presented with local disease (64%, n = 699), and underwent surgical intervention (88%, n = 1027), but did not have radiotherapy (62%, n = 710). RMS was more likely to present in younger children (P <0.001) and with distant disease (P <0.001). Older patients were more likely to receive radiotherapy than infants (P <0.001). Overall 5-y survival was 79%. RMS had significantly worse 5-y survival (56% versus 85% for NRSTS, P <0.001). Surgical intervention was associated with higher 5-y survival (84% versus 48%, P <0.001). Radiotherapy was associated with worse 5-y survival (74% versus 83%, P = 0.002). Multivariate analysis identified RMS (HR 2.20, P <0.001), nerve and muscle (not synovial sarcoma) tissue of origin (HR 2.26, P = 0.002, and HR 1.59, P = 0.036), regional or distant disease (HR 1.65, P = 0.011, and HR 5.96, P <0.001, respectively), and lack of surgical intervention (HR 2.20, P <0.001) as independent predictors of poor outcome. Conclusions: Extremity sarcomas are most common in older children. RMS is more common in younger children, but is associated with lower survival, and is an independent prognostic indicator of mortality.

Original languageEnglish (US)
Pages (from-to)282-289
Number of pages8
JournalJournal of Surgical Research
Volume163
Issue number2
DOIs
StatePublished - Oct 2010
Externally publishedYes

Fingerprint

Rhabdomyosarcoma
Sarcoma
Extremities
Survival
Incidence
Radiotherapy
Synovial Sarcoma
Muscles
Neoplasms
Epidemiology
Multivariate Analysis
Databases
Pediatrics
Mortality
Population

Keywords

  • extremity tumors
  • outcomes
  • pediatrics
  • sarcoma
  • SEER

ASJC Scopus subject areas

  • Surgery
  • Medicine(all)

Cite this

Cheung, M. C., Zhuge, Y., Yang, R., Ogilvie, M. P., Koniaris, L., Rodríguez, M. M., & Sola, J. E. (2010). Incidence and outcomes of extremity soft-tissue sarcomas in children. Journal of Surgical Research, 163(2), 282-289. https://doi.org/10.1016/j.jss.2010.04.033

Incidence and outcomes of extremity soft-tissue sarcomas in children. / Cheung, Michael C.; Zhuge, Ying; Yang, Relin; Ogilvie, Michael P.; Koniaris, Leonidas; Rodríguez, Maria M.; Sola, Juan E.

In: Journal of Surgical Research, Vol. 163, No. 2, 10.2010, p. 282-289.

Research output: Contribution to journalArticle

Cheung, MC, Zhuge, Y, Yang, R, Ogilvie, MP, Koniaris, L, Rodríguez, MM & Sola, JE 2010, 'Incidence and outcomes of extremity soft-tissue sarcomas in children', Journal of Surgical Research, vol. 163, no. 2, pp. 282-289. https://doi.org/10.1016/j.jss.2010.04.033
Cheung, Michael C. ; Zhuge, Ying ; Yang, Relin ; Ogilvie, Michael P. ; Koniaris, Leonidas ; Rodríguez, Maria M. ; Sola, Juan E. / Incidence and outcomes of extremity soft-tissue sarcomas in children. In: Journal of Surgical Research. 2010 ; Vol. 163, No. 2. pp. 282-289.
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abstract = "Background: We studied the outcomes of pediatric extremity tumors on a population scale. Methods: The Surveillance, Epidemiology, and End Results database (1973-2006) was queried for all patients under 20 y of age. Results: Overall, 1175 patients were identified. The median age at diagnosis was 12 y, but most patients were ≥10 y of age (72{\%}, n = 842). Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79{\%}, n = 879). The most common tissue of origin was muscle (43{\%}, n = 474). Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140). Most patients presented with local disease (64{\%}, n = 699), and underwent surgical intervention (88{\%}, n = 1027), but did not have radiotherapy (62{\%}, n = 710). RMS was more likely to present in younger children (P <0.001) and with distant disease (P <0.001). Older patients were more likely to receive radiotherapy than infants (P <0.001). Overall 5-y survival was 79{\%}. RMS had significantly worse 5-y survival (56{\%} versus 85{\%} for NRSTS, P <0.001). Surgical intervention was associated with higher 5-y survival (84{\%} versus 48{\%}, P <0.001). Radiotherapy was associated with worse 5-y survival (74{\%} versus 83{\%}, P = 0.002). Multivariate analysis identified RMS (HR 2.20, P <0.001), nerve and muscle (not synovial sarcoma) tissue of origin (HR 2.26, P = 0.002, and HR 1.59, P = 0.036), regional or distant disease (HR 1.65, P = 0.011, and HR 5.96, P <0.001, respectively), and lack of surgical intervention (HR 2.20, P <0.001) as independent predictors of poor outcome. Conclusions: Extremity sarcomas are most common in older children. RMS is more common in younger children, but is associated with lower survival, and is an independent prognostic indicator of mortality.",
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AU - Cheung, Michael C.

AU - Zhuge, Ying

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AU - Ogilvie, Michael P.

AU - Koniaris, Leonidas

AU - Rodríguez, Maria M.

AU - Sola, Juan E.

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N2 - Background: We studied the outcomes of pediatric extremity tumors on a population scale. Methods: The Surveillance, Epidemiology, and End Results database (1973-2006) was queried for all patients under 20 y of age. Results: Overall, 1175 patients were identified. The median age at diagnosis was 12 y, but most patients were ≥10 y of age (72%, n = 842). Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79%, n = 879). The most common tissue of origin was muscle (43%, n = 474). Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140). Most patients presented with local disease (64%, n = 699), and underwent surgical intervention (88%, n = 1027), but did not have radiotherapy (62%, n = 710). RMS was more likely to present in younger children (P <0.001) and with distant disease (P <0.001). Older patients were more likely to receive radiotherapy than infants (P <0.001). Overall 5-y survival was 79%. RMS had significantly worse 5-y survival (56% versus 85% for NRSTS, P <0.001). Surgical intervention was associated with higher 5-y survival (84% versus 48%, P <0.001). Radiotherapy was associated with worse 5-y survival (74% versus 83%, P = 0.002). Multivariate analysis identified RMS (HR 2.20, P <0.001), nerve and muscle (not synovial sarcoma) tissue of origin (HR 2.26, P = 0.002, and HR 1.59, P = 0.036), regional or distant disease (HR 1.65, P = 0.011, and HR 5.96, P <0.001, respectively), and lack of surgical intervention (HR 2.20, P <0.001) as independent predictors of poor outcome. Conclusions: Extremity sarcomas are most common in older children. RMS is more common in younger children, but is associated with lower survival, and is an independent prognostic indicator of mortality.

AB - Background: We studied the outcomes of pediatric extremity tumors on a population scale. Methods: The Surveillance, Epidemiology, and End Results database (1973-2006) was queried for all patients under 20 y of age. Results: Overall, 1175 patients were identified. The median age at diagnosis was 12 y, but most patients were ≥10 y of age (72%, n = 842). Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79%, n = 879). The most common tissue of origin was muscle (43%, n = 474). Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140). Most patients presented with local disease (64%, n = 699), and underwent surgical intervention (88%, n = 1027), but did not have radiotherapy (62%, n = 710). RMS was more likely to present in younger children (P <0.001) and with distant disease (P <0.001). Older patients were more likely to receive radiotherapy than infants (P <0.001). Overall 5-y survival was 79%. RMS had significantly worse 5-y survival (56% versus 85% for NRSTS, P <0.001). Surgical intervention was associated with higher 5-y survival (84% versus 48%, P <0.001). Radiotherapy was associated with worse 5-y survival (74% versus 83%, P = 0.002). Multivariate analysis identified RMS (HR 2.20, P <0.001), nerve and muscle (not synovial sarcoma) tissue of origin (HR 2.26, P = 0.002, and HR 1.59, P = 0.036), regional or distant disease (HR 1.65, P = 0.011, and HR 5.96, P <0.001, respectively), and lack of surgical intervention (HR 2.20, P <0.001) as independent predictors of poor outcome. Conclusions: Extremity sarcomas are most common in older children. RMS is more common in younger children, but is associated with lower survival, and is an independent prognostic indicator of mortality.

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