Raciborska Anna, Bilska Katarzyna, Rychlowska-Pruszynska Magdalena, Drabko Katarzyna, Chaber Radosław, Pogorzała Monika, Połczyńska Katarzyna, Godziński Jan, Rodriguez-Galindo Carlos, Wożniak Wojciech
Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Warsaw, Poland.
Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Warsaw, Poland.
J Pediatr Surg. 2014 Oct;49(10):1500-4. doi: 10.1016/j.jpedsurg.2014.04.013. Epub 2014 Jul 4.
Pelvic Ewing sarcoma (ES) is commonly associated with a worse prognosis. Large size and location limit local control options to radiation therapy, and local recurrences are common. We evaluated the impact of hemipelvectomy and radiation on outcomes, including function.
Thirty-nine patients (median age 13.5years) with ES of the pelvis and sacral bones were treated during the period 2000-2012. Fifteen were treated with definitive radiotherapy (RT), 9 patients underwent hemipelvectomy alone, and 15 were treated with combined hemipelvectomy and RT.
Twenty patients (51.2%) are alive with a median follow-up 3.2years from diagnosis. Median time from diagnosis to relapse was 1.3years. Three-year estimates of EFS and OS were 47% and 61%, respectively. Patients treated with surgery or surgery with RT had better outcome than patients treated with RT only (3-year OS 78% or 81% vs. 36%, respectively, p=0.00083). The outcome of patients with pelvic ES treated with hemipelvectomy was not significantly different from the outcome of all patients with Ewing sarcoma treated on the national Polish protocol.
Internal hemipelvectomy offers good chances of cure for patients with pelvic ES, with a reasonable rate of complications and good function.
盆腔尤文肉瘤（ES）通常预后较差。肿瘤体积大及位置特殊限制了局部控制手段，只能采用放射治疗，且局部复发很常见。我们评估了半骨盆切除术和放疗对包括功能在内的治疗结果的影响。
2000年至2012年期间，对39例（中位年龄13.5岁）盆腔和骶骨尤文肉瘤患者进行了治疗。15例接受了根治性放疗（RT），9例仅接受了半骨盆切除术，15例接受了半骨盆切除术联合放疗。
20例（51.2%）患者存活，从诊断开始的中位随访时间为3.2年。从诊断到复发的中位时间为1.3年。无事件生存期（EFS）和总生存期（OS）的三年估计值分别为47%和61%。接受手术或手术联合放疗的患者比仅接受放疗的患者预后更好（三年总生存期分别为78%或81%对36%，p=0.00083）。接受半骨盆切除术治疗的盆腔尤文肉瘤患者的治疗结果与按照波兰全国方案治疗的所有尤文肉瘤患者的治疗结果无显著差异。
半骨盆切除术为盆腔尤文肉瘤患者提供了良好的治愈机会，并发症发生率合理，功能良好。
