Castex Marie-Pierre, Rubie Hervé, Stevens Michael C G, Escribano Carlota Calvo, de Gauzy Jérôme Sales, Gomez-Brouchet Anne, Rey Annie, Delattre Olivier, Oberlin Odile
Hematology Oncology and Orthopedic Surgery Units, Children's Hospital, Toulouse, France.
J Clin Oncol. 2007 Apr 1;25(10):1176-82. doi: 10.1200/JCO.2005.05.0559.
To evaluate the outcome of children with an extraosseous Ewing tumor (EOE) according to treatment.
Children with EOE were treated either with the strategy used for malignant mesenchymal tumors (MMTs) by the International Society of Pediatric Oncology (SIOP) or with the French Society of Pediatric Oncology (SFOP) regimen used for osseous Ewing tumors (OET). The MMT strategy included vincristine/actinomycin for small and resected tumors or ifosfamide/vincristine/actinomycin for unfavorable sites or unresectable tumors. Surgical excision was to be attempted after four courses, followed by local irradiation in case of residue. Osseous Ewing (OE) protocol included three courses of cyclophosphamide/doxorubicin followed either by two similar courses in case of good response or two courses of ifosfamide/etoposide in case of no response. After resection of the primary, treatment included conventional chemotherapy in case of good histologic response and high-dose chemotherapy and radiotherapy for poor response. All diagnosis specimens were reviewed by the panel.
Between 1989 and 1999, 63 patients were registered. Characteristics of patients treated by both protocols were similar. Five-year overall survival (OS) and event-free survival (EFS) of those treated with the OE protocol are 83% and 75%, respectively, which is significantly better than the OS and EFS of those treated with the MMT strategy (59% and 44%, respectively; P = .04 and .008, respectively). The size of the primary and the type of protocol influenced patients' EFS. In multivariate analysis, only the regimen had an impact on OS and EFS.
Our study shows that patients with EOE should be treated with OE regimens, probably because of the use of anthracyclines.
根据治疗方法评估骨外尤文肉瘤（EOE）患儿的治疗结果。
EOE患儿采用国际儿科肿瘤学会（SIOP）用于恶性间叶性肿瘤（MMT）的治疗策略，或采用法国儿科肿瘤学会（SFOP）用于骨尤文肉瘤（OET）的方案进行治疗。MMT策略包括对于小的且可切除肿瘤使用长春新碱/放线菌素，对于预后不良部位或不可切除肿瘤使用异环磷酰胺/长春新碱/放线菌素。四个疗程后尝试手术切除，如有残留则进行局部放疗。骨尤文（OE）方案包括三个疗程的环磷酰胺/阿霉素，若反应良好则后续进行两个类似疗程，若无反应则进行两个疗程的异环磷酰胺/依托泊苷。原发灶切除后，若组织学反应良好则进行常规化疗，若反应不佳则进行高剂量化疗和放疗。所有诊断标本均由专家小组进行复查。
1989年至1999年间，登记了63例患者。两种方案治疗的患者特征相似。采用OE方案治疗的患者5年总生存率（OS）和无事件生存率（EFS）分别为83%和75%，显著优于采用MMT策略治疗的患者的OS和EFS（分别为59%和44%；P值分别为0.04和0.008）。原发灶大小和方案类型影响患者的EFS。多因素分析显示，只有治疗方案对OS和EFS有影响。
我们的研究表明，EOE患者应采用OE方案治疗，可能是因为使用了蒽环类药物。
