Pediatric Oncology Unit, Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
SIREDO Oncology Center Care, (Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France.
Pediatr Blood Cancer. 2024 Jul;71(7):e31038. doi: 10.1002/pbc.31038. Epub 2024 Apr 28.
Though the prognosis for pediatric patients with localised synovial sarcoma (SS) is generally good, the chances of being cured after relapse are limited. This study describes a retrospective multi-institutional series of relapsing SS patients treated at six selected European referral centers for pediatric sarcoma.
The study included 41 patients <21 years with relapsing SS, treated between 2002 and 2022. The analysis included patient's characteristics at first diagnosis, first-line treatments, clinical findings at relapse, and second-line treatment modalities.
The first relapse occurred within 3-132 months (median 18 months) after first diagnosis and was local in 34%, metastatic in 54%, and both in 12%. Treatment at first relapse included surgery in 56% of cases, radiotherapy in 34%, and systemic therapy in 88%. In all, 36 patients received second-line medical treatment, that was chemotherapy in 32 cases (with 10 different regimens) and targeted therapy in four. No patient was included in an early-phase clinical trial as second-line therapy-line therapy. Overall response rate was 42%. Median event-free survival (EFS) was 12 months, postrelapse 5-year EFS was 15.8%. Median overall survival (OS) was 30 months, postrelapse 5-year OS was 22.2%. At the Cox's multivariable regression analysis, OS was significantly associated with time and type of relapse.
Pediatric patients with relapsed SS have a poor prognosis and generally receive an individualized approach, due to the lack of a uniform standardized approach. New comprehensive strategies are needed to improve the knowledge on the biologic landscape of SS and develop tailored prospective clinical trials.
尽管局部性滑膜肉瘤（SS）患儿的预后通常较好，但复发后的治愈机会有限。本研究描述了在六个选定的欧洲儿科肉瘤转诊中心治疗的复发 SS 患儿的回顾性多机构系列。
该研究纳入了 41 名<21 岁的复发性 SS 患者，治疗时间为 2002 年至 2022 年。分析包括患者首次诊断时的特征、一线治疗、复发时的临床发现和二线治疗方式。
首次复发发生在首次诊断后 3-132 个月（中位数 18 个月），局部复发占 34%，远处转移占 54%，两者均占 12%。首次复发的治疗包括手术（56%）、放疗（34%）和全身治疗（88%）。共有 36 名患者接受了二线药物治疗，其中 32 例（采用 10 种不同方案）接受了化疗，4 例接受了靶向治疗。没有患者作为二线治疗方案纳入早期临床试验。总体缓解率为 42%。无事件生存（EFS）的中位数为 12 个月，复发后 5 年 EFS 为 15.8%。总生存（OS）的中位数为 30 个月，复发后 5 年 OS 为 22.2%。在 Cox 多变量回归分析中，OS 与复发的时间和类型显著相关。
复发性 SS 患儿的预后较差，由于缺乏统一的标准化方法，通常采用个体化方法治疗。需要新的综合策略来提高对 SS 生物学特征的认识，并制定有针对性的前瞻性临床试验。
