Pediatric Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
Ann Surg Oncol. 2013 Jan;20(1):73-9. doi: 10.1245/s10434-012-2587-9. Epub 2012 Aug 10.
Treatment of synovial sarcoma (SS) is challenging because of its unpredictable clinical behavior. We reviewed our institutional experience with pediatric SS to identify prognostic indicators and survival outcomes.
We retrospectively reviewed all pediatric/adolescent patients (age<22 years) with confirmed SS treated from 1970 to 2010. Patient and clinical characteristics were evaluated for prognostic significance and survival outcomes.
We identified 111 patients. The median age was 15.4 years. Sixty-seven tumors (60%) were monophasic, 42 (38%) were biphasic, and 2 (2%) were of unknown histology. Median follow-up was 5.3 years (range 0.8-36.8 years), 5-year overall survival (OS) was 73%, and 10-year OS was 65%. Greater tumor size (stratified as ≤5 cm, >5 cm, or ≥10 cm) (P=0.001) and depth (P=0.03) correlated with decreased OS. Primary tumor location in the upper extremity correlated with increased OS when compared with lower-extremity and central lesions (P=0.05). Bone and/or neurovascular invasion negatively impacted survival (P=0.02). Multivariate analysis revealed that tumor size (trichotomized) was the dominant and sole factor in discriminating survival risk. Neither radiotherapy nor chemotherapy correlated with improved 5-year survival.
Tumor size, depth, invasion, and primary location affect survival in pediatric SS. The role of radiotherapy and chemotherapy for SS warrants future study.
滑膜肉瘤（SS）的治疗具有挑战性，因为其临床表现不可预测。我们回顾了我们机构治疗小儿 SS 的经验，以确定预后指标和生存结果。
我们回顾性分析了 1970 年至 2010 年间确诊的小儿/青少年（<22 岁）SS 患者。评估患者和临床特征与预后意义和生存结果的关系。
共确定了 111 例患者。中位年龄为 15.4 岁。67 个肿瘤（60%）为单相，42 个（38%）为双相，2 个（2%）组织学未知。中位随访时间为 5.3 年（范围 0.8-36.8 年），5 年总生存率（OS）为 73%，10 年 OS 为 65%。较大的肿瘤大小（分层为≤5cm、>5cm 或≥10cm）（P=0.001）和深度（P=0.03）与 OS 降低相关。与下肢和中央病变相比，上肢的原发性肿瘤位置与 OS 增加相关（P=0.05）。骨和/或神经血管侵犯对生存有负面影响（P=0.02）。多变量分析显示，肿瘤大小（三分位数）是区分生存风险的主要和唯一因素。放疗和化疗均与 5 年生存率的提高无关。
肿瘤大小、深度、侵犯和原发部位影响小儿 SS 的生存。放疗和化疗在 SS 中的作用值得进一步研究。
