儿童滑膜肉瘤多中心33例临床分析

摘要

目的 分析儿童滑膜肉瘤的临床特点、治疗方法及预后.方法 回顾性分析2008年1月至2014年11月重庆医科大学附属儿童医院、上海交通大学附属上海儿童医学中心、中山大学附属肿瘤医院、首都医科大学附属北京儿童医院4家医院收治的33例滑膜肉瘤患儿的临床资料和治疗情况,并进行预后分析.结果 33例患儿中位诊断年龄为8.0岁(4个月至16岁).初诊时27例为局部肿块,6例出现肺、胸膜、骨或远处淋巴结转移.24例直接手术,9例行肿瘤活检术,其中3例在新辅助化疗3～4疗程后行二期手术;22例术后进行化疗,中位疗程数为7个(1～12个);10例术后进行局部放疗,总剂量为25～60 Gy,次数为5～25次.8例患儿在活检明确诊断或直接手术后化疗0～2疗程,放弃进一步治疗;其余25例按计划完成治疗,治疗结束时21例达到完全缓解(CR),3例疾病稳定(SD),1例疾病进展(PD).11例患儿在缓解后中位随访时间14.5个月(3～48个月)因肿瘤原位复发或转移出现PD.除去8例放弃治疗的患儿,25例患儿中位随访时间为36个月(3～92个月).随访至2015年11月,25例完成治疗的患儿,预计3年无病生存率(event-free survival,EFS)为(61.9±10.1)％.将患儿的年龄(≤10岁,＞10岁)、性别,肿瘤原发部位(四肢,轴线部位)、体积大小(≤5 cm,＞5 cm)、IRS分期、诊断时是否远处转移以及首次术后有无残留各临床特征进行单因素预后分析,发现首次术后无残留患儿的无事件生存率更高(P=0.017),未发现其余各因素对预后的影响有统计学意义.结论 儿童滑膜肉瘤最多见于四肢,治疗方法以综合治疗为主,但缓解后易晚期复发或转移,首次术后无残留是影响预后的重要因素,有待寻找更有效的治疗方法提高长期疗效.%Objective To explore the clinical features,outcomes and correlative prognostic factors of pediatric synovial sarcoma.Methods From January 2008 to November 2014,33 newly diagnosed children with synovial sarcoma were enrolled from Children's Hospital of Chongqing Medical University,Shanghai Children's Medical Center,Sun Yat-sen University Cancer Center and Beijing Children's Hospital.Their clinical features,surgical approaches,chemoradiotherapeutic protocols and prognostic factors were retrospectively analyzed.Results The median age at diagnosis was 8.0 (1/3 to 16) years.Twenty-seven cases had local tumors and 6 cases had lung,pleural mesothelioma,bone or distant lymph node metastasis.Tumor was resected initially in 24 patients.The remaining 9 patients had an initial biopsy and three of them underwent tumor resection after 3-4 cycles of neoadjuvant chemotherapy.Twenty-two patients received postoperative chemotherapy with a median of 7 (1-12) cycles.Five to 25 sessions of postoperative radiotherapy were offered to 10 patients with a total dose of 25-60 Gy.Eight patients gave up treatment after biopsy or after direct surgery with 0-2 cycles chemotherapy applied postoperatively.All other 25 received planned comprehensive treatments.At the end of treatment,the outcomes had complete response (n =21),stable disease (n =3) and progressive disease (n =1).Tumor metastasis or local relapse occurred in 11 patients after a median time of 14.5 (3-48) months.Except for 8 cases giving up treatment,the median follow-up period was 36 (3-92) months.The estimated 3 year event-free survival was (61.9 ± 10.1)％.Patients with completely excised tumors after initial surgery had significantly better event-free survival while significant prognostic factors were not found on univariate analysis,including age (≤ 10,＞ 10),gender,primary site (extremities/axial),size (≤5 crn,＞5 cm),IRS staging or distant metastasis.Conclusions Pediatric synovial sarcoma arises frequently in extremities.Comprehensive treatment is recommended for pediatric synovial sarcoma.However,late local recurrence and metastasis are frequent after remissior.Completely excised tumor after initial surgery is an important prognostic factor.Effective treatment is required for improving its long-term outcome.