Rosen G, Caparros B, Mosende C, McCormick B, Huvos A G, Marcove R C
Cancer. 1978 Mar;41(3):888-99. doi: 10.1002/1097-0142(197803)41:3<888::aid-cncr2820410316>3.0.co;2-t.
Twenty previously untreated children with primary Ewing's sarcoma and 8 children with primary tumor and metastatic disease were treated with surgery or radiation therapy (6,000-7,000 rads) for their primary tumor and T-2 chemotherapy. Of the 20 children with primary Ewing's sarcoma treated with T-2 "adjuvant" chemotherapy, 15 had no evidence of recurrent disease for from 31+-82+ months (median 46+ months) from the start of treatment. The actuarial 5-year disease-free survival rate for this group of patients was 75%. Eight patients presenting with metastatic disease had complete responses to T-2 chemotherapy, but 7/8 with metastatic disease eventually had tumor recurrence. Examination of the treatment failures, both those patients relapsing after adjuvant chemotherapy for primary Ewing's sarcoma (5), and those relapsing after having a complete response of metastatic disease (7) to T-2 chemotherapy, revealed that all relapses occurred at the end of the second year of T-2 chemotherapy or after chemotherapy was stopped. In addition, of 23 patients receiving "curative" radiation therapy to their primary tumor, 5 had local recurrence (22%) and 6 (26%) had severe functional debility secondary to combined radiation therapy and T-2 chemotherapy. The conclusions drawn from this experience have led us to consider a new approach to the treatment of Ewing's sarcoma, namely: 1) more aggressive initial or "induction" chemotherapy with subsequent T-2 "maintenance" chemotherapy to eradicate more completely all metastatic microfoci of disease presumed to be present in patients with primary tumor at the time of diagnosis, and ostensively present in patients with metastatic disease; 2) the use of surgery alone or in combination with moderate doses of radiation therapy in those patients in whom we can predict a high frequency of local recurrence (pelvic lesions) or a high percentage of "functional failures" (young children with lower extremity lesions). Preliminary results with this latter approach are encouraging with 11/13 patients with primary Ewing's sarcoma free of disease at 12+-26+ months. A longer follow-up of this more aggressive treatment is needed to determine the superiority of this approach for both increased survival and improved late physical rehabilitation.
20名先前未经治疗的原发性尤因肉瘤患儿和8名患有原发性肿瘤及转移性疾病的患儿接受了针对原发性肿瘤的手术或放射治疗（6000 - 7000拉德）以及T - 2化疗。在接受T - 2“辅助”化疗的20名原发性尤因肉瘤患儿中，15名从治疗开始起31±82个月（中位时间46个月）内无疾病复发迹象。该组患者的精算5年无病生存率为75%。8名出现转移性疾病的患者对T - 2化疗有完全反应，但8名转移性疾病患者中有7名最终出现肿瘤复发。对治疗失败病例的检查发现，无论是那些在原发性尤因肉瘤辅助化疗后复发的患者（5例），还是那些在转移性疾病对T - 2化疗有完全反应后复发的患者（7例），所有复发均发生在T - 2化疗的第二年结束时或化疗停止后。此外，在23名接受原发性肿瘤“根治性”放射治疗的患者中，5名出现局部复发（22%），6名（26%）因放射治疗和T - 2化疗联合导致严重功能障碍。从这一经验得出的结论促使我们考虑一种新的尤因肉瘤治疗方法，即：1）采用更积极的初始或“诱导”化疗，随后进行T - 2“维持”化疗，以更彻底地根除据推测在诊断时原发性肿瘤患者中存在的所有转移性微小病灶，以及明显存在于转移性疾病患者中的病灶；2）对于那些我们可以预测局部复发频率高（盆腔病变）或“功能失败”百分比高（下肢病变的幼儿）的患者，单独使用手术或与中等剂量放射治疗联合使用。后一种方法的初步结果令人鼓舞，13名原发性尤因肉瘤患者中有11名在12±26个月时无疾病。需要对这种更积极的治疗进行更长时间的随访，以确定这种方法在提高生存率和改善后期身体康复方面的优越性。
