Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan - The Japanese musculoskeletal Oncology Group cooperative study
Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan - The Japanese musculoskeletal Oncology Group cooperative study
复制标题
DOI：
10.1002/cncr.22481
复制
发表时间：
2007-02-15
期刊：
影响因子：
6.2
通讯作者：
Yabe, Hiroo
中科院分区：
文献类型：
--
作者：
Obata, Hideto;Ueda, Takafumi;Yabe, Hiroo
背景资料。骨尤文肉瘤家族(ESFT)在日本极为罕见。本研究的目的是评估日本骨ESFT患者的临床结果和预后因素，并比较欧美和日本人群之间的差异。方法：作者对1981-2003年间在日本接受骨ESFT治疗的243名患者进行了回顾性分析。局部治疗为手术治疗者占35%，手术加放疗者占40%，单纯放疗者占22%，不进行局部治疗者占3%。除3例患者外，其余患者均接受不同方案的多药化疗。结果患者的中位年龄为16岁。53%的患者以躯干为原发部位，47%的患者为四肢。41例患者在发病时有转移。中位随访时间为66个月。单因素生存分析显示，出现转移、原发灶位于躯干、年龄<16岁、肿瘤大小<10 cm、对诱导化疗反应差、单纯局部放疗的患者无事件生存率(EFS)显著降低。多因素分析进一步证实前3个因素是显著的不良预后因素。在201名局限性疾病患者中，接受当前化疗方案(包括异环磷酰胺和依托泊苷)的45名患者的5年EFS率(67.6%)明显好于其他患者。结论：在日本，使用当前化疗方案(包括异环磷酰胺和依托泊苷)后，日本局限性骨骼ESFT患者的临床结果显著改善，与其他主要欧美患者的结果相当。影响预后的因素也几乎相同。
BACKGROUND. Ewing sarcoma family of tumors (ESFT) of bone is extremely rare in Japan. The objectives of the Current study were to assess the clinical outcome and prognostic factors of patients with ESFT of bone in Japan and to compare them between Euro-American and Japanese populations.METHODS. The authors conducted a retrospective analysis of 243 patients who were treated for ESFT of bone in Japan between 1981 and 2003. Local therapy was surgery in 35% of patients, surgery combined with radiotherapy in 40% of patients, radiotherapy alone in 22%, of patients, and no local treatment in 3% of patients. All but 3 patients received various regimens of multidrug chemotherapy.RESULTS. The median patient age was 16 years. The primary disease sites were the trunk in 53% of patients and the extremities in 47% of patients. Forty-one patients had metastases at presentation. The median follow-up was 66 months. A univariate survival analysis demonstrated that patients who had metastases at presentation, primary site in the trunk, age >= 16 years, tumor size >= 10 cm, tumor that responded poorly to induction chemotherapy, and local treatment with radiotherapy alone had a significantly worse event-free survival (EFS). A multivariate analysis further verified that the former 3 factors were significant adverse prognostic factors. Of 201 patients with localized disease, 45 patients who received current chemotherapy regimens that included ifosfamide and etoposide had a significantly better 5-year EFS rate (67.6%) compared with other patients.CONCLUSIONS. The clinical outcome of patients with localized ESFT of bone in Japan has improved markedly with the use of current chemotherapy regimens that include ifosfamide and etoposide and has become comparable to the Outcomes observed in other major series of Euro-American patients. The prognostic factors are also almost identical.