Tabrizi P, Letts M
Division of Pediatric Orthopedics, Children's Hospital of Eastern Ontario, University of Ottawa, Canada.
Am J Orthop (Belle Mead NJ). 1999 Aug;28(8):440-6.
Since 1979, 15 children with rhabdomyosarcoma have been treated at our institution. Included in this group are six children who presented with rhabdomyosarcoma of an extremity or trunk, requiring the use of combined multimodality therapy. The patients were clinically grouped and treated in accordance with the Intergroup Rhabdomyosarcoma Study protocol. All patients received combination chemotherapy based on their respective stage of disease at diagnosis. When feasible, the primary tumor was resected en bloc before chemotherapy was begun. After surgery, patients with unclear postoperative surgical margins and an initial good response to chemotherapy received radiotherapy to the primary site and at the regional lymphatics. Three of six patients developed or maintained a complete tumor response to induction chemotherapy. Radiotherapy maintained control of local disease in both groups. Overall, four patients, including one with disseminated disease at diagnosis, are alive, with a median survival time from diagnosis of 39 months. In children, treatment must be individualized, but complete local excision of the tumor with a tumor-free margin should be the goal. Major ablative amputation surgery was not performed.
自1979年以来，我院共收治了15例横纹肌肉瘤患儿。其中6例为四肢或躯干横纹肌肉瘤患儿，需采用多模式联合治疗。这些患者按照横纹肌肉瘤协作组研究方案进行临床分组和治疗。所有患者均根据诊断时疾病的各自阶段接受联合化疗。可行时，在开始化疗前整块切除原发肿瘤。术后手术切缘不明确且对化疗初期反应良好的患者，接受原发部位及区域淋巴结放疗。6例患者中有3例对诱导化疗产生或维持了完全肿瘤反应。放疗在两组中均维持了局部疾病的控制。总体而言，4例患者存活，其中1例诊断时即有播散性疾病，自诊断起的中位生存时间为39个月。对于儿童患者，治疗必须个体化，但应以完整切除肿瘤且切缘无肿瘤为目标。未进行主要的截肢手术。
