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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 MethodsWe retrospectively reviewed 220 pediatric-type sarcoma patients at a single institutionbetween 1985 and 2011. Comparisons were made in order to examine differences indemographics, disease characteristics, and survival. Survival analyses were performed usingthe Kaplan-Meier method with log-rank tests and Cox proportional hazards models. ResultsA total of 220 consecutive patients were identified at our institute. Median age was 15.6years (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 periodof 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 allpatients was 11 months (95% CI, 8.8 to 13.2 months). No significant difference in OS andEFS 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. ConclusionMetastatic disease and no surgical treatment are poor prognostic factors for OS amongpediatric-type sarcomas for both adults and children.
