Diaz-Beveridge Robert, Lorente David, Torres Barbara, Cañete Adela, Rodrigo Esteban, Bruixola Gema, Berlanga Pablo, Reche Encarnacion, Montalar Joaquin, Verdeguer Amparo, Aparicio Jorge
*Medical Oncology Department †Pediatric Oncology Department, University Hospital La Fe, Valencia, Spain.
J Pediatr Hematol Oncol. 2015 Jul;37(5):e278-84. doi: 10.1097/MPH.0000000000000339.
The treatment of Ewing Sarcoma family of tumors is multimodal, both in children and adults. Axial location and metastases are classic prognostic factors. However, the worse prognosis in older patients is more controversial.
Retrospective analysis was performed of pediatric and adult patients treated with the 2001 SEOP protocol: 6 cycles of VIDE chemotherapy (CT). If no progression was observed, local (surgery and/or radiotherapy) and consolidation treatments were performed adjusted to prognosis: 8 cycles of VAC in standard-risk patients or 1 cycle of VAC and high-dose CT and autologous transplant in the case of increased risk.We analyzed induction CT toxicity, type of consolidation treatment, and disease-free (DFS) and overall (OS) survival by the Kaplan-Meier method, with a log-rank analysis of prognostic factors with regard to OS.
Thirty-six patients were analyzed (2003 to 2011). Sixty percent were male, with a median age of 16 years (range, 7 to 57 y). The most frequent location was axial (43%), followed by extremities (34%), extraosseous (18%), and ribs (9%). Fifty-four percent of patients had metastases, of which, 58% were pulmonary.The median follow-up period was 36 months (5 to 101 mo). Median DFS was 25 months (16 to 34 mo) and median OS 29 months (19 to 40 mo), with a 3-year OS of 40%. Median OS from progression was 7 months (0.4 to 15 mo). Age <15 years and normal lactate dehydrogenase levels were associated with prolonged OS.
Induction CT with the VIDE regimen was feasible in most patients, with a low risk for early progression. Hematological toxicity was substantial but manageable. Adult patients had a worse prognosis. Survival after progression was dismal.
尤因肉瘤家族性肿瘤的治疗在儿童和成人中都是多模式的。轴向位置和转移是典型的预后因素。然而，老年患者预后较差这一点更具争议性。
对采用2001年SEOP方案治疗的儿科和成人患者进行回顾性分析：6周期VIDE化疗（CT）。如果未观察到进展，则根据预后进行局部（手术和/或放疗）和巩固治疗：标准风险患者进行8周期VAC治疗，高风险患者进行1周期VAC、高剂量CT和自体移植。我们通过Kaplan-Meier方法分析诱导CT毒性、巩固治疗类型以及无病生存期（DFS）和总生存期（OS），并对OS的预后因素进行对数秩分析。
分析了36例患者（2003年至2011年）。60%为男性，中位年龄16岁（范围7至57岁）。最常见的部位是轴向（43%），其次是四肢（34%）、骨外（18%）和肋骨（9%）。54%的患者有转移，其中58%为肺转移。中位随访期为36个月（5至101个月）。中位DFS为25个月（16至34个月），中位OS为29个月（19至40个月），3年OS率为40%。进展后的中位OS为7个月（0.4至15个月）。年龄<15岁和乳酸脱氢酶水平正常与OS延长相关。
采用VIDE方案的诱导CT在大多数患者中是可行的，早期进展风险低。血液学毒性较大但可控。成年患者预后较差。进展后的生存率很低。
