Analysis of prognostic factors of pediatric-type sarcomas in adult patients

Hee Kyung Ahn, Ji Eun Uhm, Jeeyun Lee, Do Hoon Lim, Sung Wook Seo, Ki Sun Sung, Su Jin Lee, Duk Joo Lee, Kyung Kee Baek, Won Seog Kim, Joon Oh Park

Research output: Contribution to journalReview articlepeer-review

8 Scopus citations

Abstract

Objective: Pediatric-type sarcomas such as Ewing's sarcoma (EWS)/primitive neuroectodermal tumor family and rhabdomyosarcoma are relatively uncommon in adult patients. Optimal treatment strategies for this population and prognosis in adult patients compared with pediatric patients remain controversial. Methods: We retrospectively reviewed pediatric-type sarcoma patients older than 15 years at a single institution. Results: A total of 84 consecutive patients between 1995 and 2009 were identified at the Samsung Medical Center, Seoul, Korea. Median age was 30 years with a range of 15-74 years. Forty-seven patients (56.0%) were diagnosed with Ewing's sarcoma/primitive neuroectodermal tumor family, 34 (40.5%) with rhabdomyosarcoma and 3 (3.6%) with desmoplastic round-cell tumor. Median follow-up duration was 5.9 years. Median overall survival for all patients was 33.1 months (95% CI 13.5-52.7) and median event-free survival for all patients was 14.4 months (95% CI 5.9-22.9 months). Multivariate analysis revealed that localized disease was a significant independent prognostic factor for longer overall survival (hazard ratio 0.30, 95% CI 0.14-0.66, p = 0.003), and favorable primary tumor sites were associated with longer event-free survival (hazard ratio 0.33, 95% CI 0.11-0.98, p = 0.045). Conclusion: We identified the prognostic variables which may facilitate risk-adapted therapies for this rare adult sarcoma group, which should be further investigated.

Original languageEnglish
Pages (from-to)21-28
Number of pages8
JournalOncology
Volume80
Issue number1-2
DOIs
StatePublished - Jun 2011

Keywords

  • Desmoplastic small round-cell tumor
  • Ewing's sarcoma
  • Pediatric-type sarcoma
  • Primitive neuroectodermal tumor family
  • Prognosis
  • Rhabdomyosarcoma

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