Lopez Jose Luis, Pérez Concepcion, Marquez Catalina, Cabrera Patricia, Perez Jose Maria, Ramirez Gema Lucia, Ordoñez Rafael, Praena-Fernandez Juan Manuel, Ortiz Maria Jose
Virgen del Rocío University Hospital, Department of Radiation Oncology, Sevilla, Spain.
Virgen del Rocío University Hospital, Department of Hematology, Sevilla, Spain.
Rep Pract Oncol Radiother. 2011 May 20;16(5):163-9. doi: 10.1016/j.rpor.2011.04.002.
Attempts to improve survival outcomes of patients with high risk Ewing's sarcoma (ES) have focused on chemotherapy dose intensification strategies.
The objective of this study is to retrospectively evaluate clinical characteristics and outcome of pediatric patients with high risk ES treated at a single institution.
From 1995 to 2008, seventeen patients (male:female, 14:3) were treated with dose-intensive therapy in our institution. Median age at diagnosis was 10 years (range: 2-15). Seven patients had metastases at diagnosis (lung in 6 cases and bone in one case). Eleven patients presented with unresectable disease. Fifteen (88.2%) received the Spanish Society of Pediatric Oncology protocol which includes six cycles of vincristine, doxorubicin, ifosfamide and etoposide. Two out of the six cases that were resectable received postoperative radiation. In addition, eleven patients received definitive radiation therapy. Finally, twelve (70.5%) out of 17 patients received myeloablative therapy with melphalan/etoposide. The rest of patients (N = 5) received busulfan/melphalan.
Median follow-up was 78 months (range: 15-155 months). Initial responses were complete in all patients, but 9 of them developed progression disease. Seven patients became long-term event-free survivors. No patient died of toxicity after transplantation. The 2- and 5-year overall survival rates for all patients were 93% and 73%, respectively. Event-free survival rates were 74% and 54% at 2 and 5 years, respectively.
This single-institution experience suggests that myeloablative therapy against high risk ES is effective and safe.
提高高危尤因肉瘤（ES）患者生存结局的尝试主要集中在化疗剂量强化策略上。
本研究的目的是回顾性评估在单一机构接受治疗的高危ES儿科患者的临床特征和结局。
1995年至2008年，17例患者（男：女，14：3）在本机构接受了剂量强化治疗。诊断时的中位年龄为10岁（范围：2至15岁）。7例患者在诊断时有转移（6例肺部转移，1例骨转移）。11例患者表现为不可切除的疾病。15例（88.2%）接受了西班牙儿科肿瘤学会方案，该方案包括六个周期的长春新碱、多柔比星、异环磷酰胺和依托泊苷。6例可切除病例中有2例接受了术后放疗。此外，11例患者接受了根治性放疗。最后，17例患者中有12例（70.5%）接受了美法仑/依托泊苷清髓治疗。其余患者（N = 5）接受了白消安/美法仑治疗。
中位随访时间为78个月（范围：15至155个月）。所有患者最初均有完全缓解，但其中9例出现疾病进展。7例患者成为长期无事件生存者。移植后无患者死于毒性反应。所有患者的2年和5年总生存率分别为93%和73%。无事件生存率在2年和5年时分别为74%和54%。
这一单一机构的经验表明，针对高危ES的清髓治疗是有效且安全的。
