Ewing's sarcoma of soft tissues in childhood: A report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991
Ewing's sarcoma of soft tissues in childhood: A report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991
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DOI：
10.1200/jco.1997.15.2.574
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发表时间：
1997-02-01
影响因子：
45.3
通讯作者：
Maurer, HM
中科院分区：
文献类型：
--
作者：
Raney, RB;Asmar, L;Maurer, HM
目的：1972年至1991年，在三项组间横纹肌肉瘤研究临床试验（IRS-I、IRS-II和IRS-III）中登记的2，792例患者中，有130例（5%）患有骨外尤文肉瘤（EOE）。我们在这里报告的结果，这种tumor.Patients和方法的综合治疗：130例患者年龄小于21岁，70（54%）是微尘。原发肿瘤部位位于躯干41例，四肢34例，头颈部23例，腹膜后/骨盆21例，其他部位11例。114例患者在诊断时无转移。在21例患者中，肿瘤被完全切除;在30例患者中，局部或区域肿瘤被大体切除，在63例患者中，大体可见的肉瘤被留下。16例患者（12%）在诊断时有远处转移。所有患者均给予多药化疗，大多数接受放疗（XRT），没有接受骨髓transplantation.Results：107例（82%）患者取得了完全缓解。在10年时，分别有62%、61%和77%的患者在IRS-I、IRS-II或IRS-III治疗方案治疗后存活，与所有IRS患者的数据相似。在最后一次随访评估时，42例患者死于进展性肿瘤，1例死于感染。肿瘤发生在头颈部、四肢和躯干的患者，以及在开始化疗前接受肿瘤完全切除的患者，10年生存率最高。对于局部、大体残留肿瘤的患者，在长春新碱、更生霉素、环磷酰胺（VAC）和XRT的联合治疗中加入多柔比星（DOX），39例患者的生存率没有显著提高（10年时存活率为62%），与24例接受VAC和XRT（未使用DOX）治疗的患者相比结论：该系列研究表明，儿童EOE对多模式治疗的反应与横纹肌肉瘤（RMS）相似。在VAC化疗基础上加用DOX治疗总残余EOE的患者无明显获益。(C)1997年，美国临床肿瘤学会。
Purpose: One hundred thirty of 2,792 patients (5%) registered on three Intergroup Rhabdomyosarcoma Study clinical trials (IRS-I, -II, and -III) from 1972 to 1991 had an extraosseous Ewing's sarcoma (EOE). We report here the results of multimodality therapy for this tumor.Patients and Methods: The 130 patients were less than 21 years of age; 70 (54%) were motes. Primary tumor sites were on the trunk in 41 patients, an extremity in 34, the head/neck in 23, the retroperitoneum/pelvis in 21, and other sites in 11. One hundred fourteen patients had no metastases at diagnosis. In 21 patients, the tumor was completely resected; in 30, the localized or regional tumor was grossly resected, and in 63 patients, grossly visible sarcoma was left behind. Sixteen patients (12%) had distant metastases at diagnosis. All patients were given multiagent chemotherapy and most received irradiation (XRT); none were treated with bone marrow transplantation.Results: One hundred seven patients (82%) achieved a complete response. At 10 years, 62%, 61%, and 77% of the patients were alive after treatment an IRS-I, IRS-II, or IRS-III therapeutic protocols, respectively, similar to figures obtained in all IRS patients. At last follow-up evaluation, 42 patients had died of progressive tumor and one of infection. Survival at 10 years was most likely for patients with tumor that arose in the head and neck, extremities, and trunk, and for those who underwent grossly complete tumor removal before initiation of chemotherapy. For patients with localized, gross residual tumor, adding doxorubicin (DOX) to the combination of vincristine, dactinomycin, cyclophosphamide (VAC), and XRT did not significantly improve survival in 39 patients (62% alive at 10 years) compared with that of 24 patients treated with VAC and XRT without DOX (65% alive at 10 years, P = .93).Conclusion: This series indicated that EOE in children is similar to rhabdomyosarcoma (RMS) in its response to multimodal treatment. No benefit was apparent from the addition of DOX to VAC chemotherapy in patients with gross residual EOE. (C) 1997 by American Society of Clinical Oncology.