新辅助化疗结合带血管蒂腓骨移植保肢治疗儿童股骨干尤文肉瘤

Limb Salvage Treatment of Pediatric Ewing's Sarcoma in the Femur Shaft via Neoadjuvant Chemotherapy Combined with Vascularized Fibular Graft

目的:探讨新辅助化疗结合带血管蒂腓骨移植保肢治疗儿童股骨干尤文肉瘤的疗效。方法:采用新辅助化疗结合带血管蒂腓骨移植保肢治疗儿童股骨干尤文肉瘤20例,按Enneking分期分为ⅡA期5例,ⅡB期15例。均进行术前化疗、保肢手术和术后化疗。所有切除标本均行病理切片的坏死率评估以调整术后化疗方案。结果:全组患儿均获随访,随访时间6～45个月,平均30个月。随访至2011年8月30日,17例无局部复发或远处转移。1例术后1年内出现骨盆部转移并肺栓塞死亡,2例术后2年内出现肺部转移,并因肺转移合并呼吸循环衰竭死亡。3年无瘤生存率85%,最终保肢率100%。移植腓骨与股骨连接处术后8～12周内骨性愈合。术后按Enneking评分标准评定疗效优20例。结论:新辅助化疗结合带血管蒂腓骨移植保肢治疗是儿童股骨干尤文肉瘤一种有效的治疗方法。

Objective： To explore the clinical effect of limb salvage treatment on pediatric Ewing＇s sarcoma in the femur shaft via neoadjuvant chemotherapy and vascularized fibular graft. Methods： Twenty patients with pediatric Ewing＇s sarcoma in the femur shaft were classified into Stage A or B＇according to Enneking classification. The patients were treated via preoperative neoadjuvant chemotherapy, limb salvage surgery, and postoperative neoadjuvant chemotherapy. The postoperative chemotherapy was adjusted according to the tumor necrosis rate after surgery. Results： All patients were followed up for 6 months to 45 months （ mean of 30 months ）. Seventeen out of the 20 patients survived without recurrence or distant metastasis until August 30, 2011. One patient developed pelvis metastasis and died of pulmonary embolism one year after surgery, and two patients died of pulmonary metastasis two years after surgery. The 3-year disease-free-survival rate was 85%. All patients were treated via limb salvage. The union between the fibula and the femur shaft was achieved within 8 weeks to 12 weeks after surgery. The results were evaluated using the Enneking evaluation system, which detected effective treatment in 20 patients. Conclusion： Neoadjuvant chemotherapy combined with vascularized fibular graft is one of the effective methods for the treatment of pediatric Ewing＇s sarcoma in the femur shaft.