Pediatric Adolescent and Young Adult Department, Institut Curie, Paris, France.
Pediatr Blood Cancer. 2014 Aug;61(8):1387-93. doi: 10.1002/pbc.25001. Epub 2014 Mar 24.
Twenty-five to 32% of patients with synovial sarcoma (SS) relapse after appropriate treatment, and experience a poor outcome. Patients who can be salvaged by second-line therapy need to be more clearly identified.
Data of patients treated in SFCE (Société Française des Cancers de l'Enfant) centers with an initial diagnosis of localized SS before the age of 18 years and treated from 1/1988 to 12/2008, and who experienced at least one relapse, were retrieved. After descriptive analysis, statistical analysis was performed to determine prognostic factors.
Thirty-seven patients were identified. First relapse occurred after a median interval of 24 months and was localized in 73.0% of cases and metastatic in 24.3% of cases. Treatment of relapse consisted of new surgery in 75.7% of cases, second-line chemotherapy in 73.0% of cases and radiotherapy in 48.6% of cases. Response rate to ifosfamide-based regimens was 36.4%. Overall, 70.3% patients achieved a second complete remission. Median 5-year-event-free survival was 32.8% and 5-year overall survival was 42.1%. Factors significantly correlated with better survival were primary tumor involving the limbs, age less than 12 years at diagnosis, absence of chemotherapy or radiotherapy as initial treatment and local relapse.
Despite its poor overall outcome, relapse of synovial sarcoma sometimes remains curable. Aggressive surgery, when possible, in combination with chemotherapy and radiotherapy is the recommended treatment. Ifosfamide-based regimens may remain effective in patients with relapsed SS. However, alternative therapies should be proposed in patients with poor prognostic factors.
25%至 32%的滑膜肉瘤（SS）患者在适当治疗后复发，且预后较差。需要更明确地识别出可以通过二线治疗挽救的患者。
检索了 1988 年 1 月至 2008 年 12 月期间在法国儿童癌症协会（SFCE）中心接受初始诊断为 18 岁以下局限性 SS 治疗且至少经历过一次复发的患者数据。在描述性分析后，进行了统计分析以确定预后因素。
共确定了 37 名患者。首次复发发生在中位间隔 24 个月后，73.0%的病例为局限性复发，24.3%的病例为转移性复发。复发治疗包括 75.7%的病例进行新手术、73.0%的病例进行二线化疗和 48.6%的病例进行放疗。伊立替康为基础的方案的缓解率为 36.4%。总体而言，70.3%的患者达到第二次完全缓解。中位 5 年无事件生存率为 32.8%，5 年总生存率为 42.1%。与生存较好相关的因素是原发性肿瘤累及四肢、诊断时年龄小于 12 岁、初始治疗未接受化疗或放疗以及局部复发。
尽管滑膜肉瘤总体预后较差，但复发有时仍可治愈。尽可能进行积极的手术，结合化疗和放疗是推荐的治疗方法。对于复发的 SS 患者，伊立替康为基础的方案可能仍然有效。然而，对于预后不良的患者，应提出替代治疗方案。
