Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Oncology. 2011;80(1-2):21-8. doi: 10.1159/000327222. Epub 2011 May 23.
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.
We retrospectively reviewed pediatric-type sarcoma patients older than 15 years at a single institution.
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).
We identified the prognostic variables which may facilitate risk-adapted therapies for this rare adult sarcoma group, which should be further investigated.
小儿型肉瘤，如尤文肉瘤（EWS）/原始神经外胚层肿瘤家族和横纹肌肉瘤，在成年患者中相对少见。对于这一人群的最佳治疗策略以及与儿科患者相比的成年患者预后仍存在争议。
我们回顾性分析了一家机构中年龄大于 15 岁的小儿型肉瘤患者。
在韩国首尔三星医疗中心，共确定了 1995 年至 2009 年间的 84 例连续患者。中位年龄为 30 岁，范围为 15-74 岁。47 例（56.0%）患者被诊断为尤文肉瘤/原始神经外胚层肿瘤家族，34 例（40.5%）为横纹肌肉瘤，3 例（3.6%）为促结缔组织增生性小圆细胞肿瘤。中位随访时间为 5.9 年。所有患者的中位总生存期为 33.1 个月（95%CI 13.5-52.7），所有患者的中位无事件生存期为 14.4 个月（95%CI 5.9-22.9 个月）。多因素分析显示，局限性疾病是总生存期延长的显著独立预后因素（风险比 0.30，95%CI 0.14-0.66，p=0.003），而有利的原发肿瘤部位与无事件生存期延长相关（风险比 0.33，95%CI 0.11-0.98，p=0.045）。
我们确定了可能有助于为这一罕见的成年肉瘤患者群体制定风险适应治疗的预后变量，这些变量应进一步研究。
