Abstract：
To evaluate the treatment results, prognostic parameters, and treatment-related toxicity in patients with Ewing sarcoma (ES)/primitive neuroectodermal tumor (PNET) of the chest wall who underwent surgery, chemotherapy, and radiotherapy (RT) in a tertiary referral center.
The data of 24 patients under 18 years of age with a histologic diagnosis of ES/PNET in the chest wall that received RT in our department between February 2003 and July 2020 were retrospectively evaluated. RT was applied to the primary site±whole involved chest wall and to the whole lung in patients with lung metastasis.
The median age was 8.5 years (range: 1.5 to 17 y), 15 (63%) patients were female and 9 were male (37%). The tumor localization was extrathoracic in 18 (75%) and intrathoracic in 6 (25%) patients. Mediastinal lymph node and distant metastasis (DM) was present in 5 (21%) and 4 (16%) cases at diagnosis, respectively. The median follow-up after RT was 47 months (range: 11 to 162 mo). The 2-year and 5-year overall survival, event-free survival, local recurrence-free survival, and pleural recurrence-free survival were 83% and 48%, 48% and 42%, 74% and 48%, and 61% and 52%, respectively. The overall local control rate was 83% and the pleural control rate was 67%. RT was well tolerated, with 1 case of grade 3 acute dermatitis and 1 case of grade 3 subacute radiation pneumonitis. Late toxicity was observed in 3 (13%) cases.
Long-term survival can be achieved with extended-field RT even in patients with ES/PNET of the chest wall with DM. The low toxicity rates allow us to draw the conclusion that RT with modern techniques is an effective and safe treatment modality for these patients.
The data of 24 patients under 18 years of age with a histologic diagnosis of ES/PNET in the chest wall that received RT in our department between February 2003 and July 2020 were retrospectively evaluated. RT was applied to the primary site±whole involved chest wall and to the whole lung in patients with lung metastasis.
The median age was 8.5 years (range: 1.5 to 17 y), 15 (63%) patients were female and 9 were male (37%). The tumor localization was extrathoracic in 18 (75%) and intrathoracic in 6 (25%) patients. Mediastinal lymph node and distant metastasis (DM) was present in 5 (21%) and 4 (16%) cases at diagnosis, respectively. The median follow-up after RT was 47 months (range: 11 to 162 mo). The 2-year and 5-year overall survival, event-free survival, local recurrence-free survival, and pleural recurrence-free survival were 83% and 48%, 48% and 42%, 74% and 48%, and 61% and 52%, respectively. The overall local control rate was 83% and the pleural control rate was 67%. RT was well tolerated, with 1 case of grade 3 acute dermatitis and 1 case of grade 3 subacute radiation pneumonitis. Late toxicity was observed in 3 (13%) cases.
Long-term survival can be achieved with extended-field RT even in patients with ES/PNET of the chest wall with DM. The low toxicity rates allow us to draw the conclusion that RT with modern techniques is an effective and safe treatment modality for these patients.
摘要：
目的：为了评估治疗效果，预后参数，和治疗相关的毒性与尤文肉瘤（ES）/原始神经外胚层肿瘤（PNET）的患者的胸壁接受手术，化疗,和放疗（RT）在三级转诊中心。
方法：对2003年2月至2020年7月在我科接受放疗的24例18岁以下胸壁组织诊断为ES/PNET患者的资料进行回顾性评估。在有肺转移的患者中，将RT应用于原发部位±整个累及的胸壁和整个肺。
结果：中位年龄为8.5岁（范围：1.5至17岁），15例（63%）患者为女性，9例为男性（37%）。18例（75％）患者为胸腔外肿瘤，6例（25％）为胸腔内肿瘤。诊断时纵隔淋巴结和远处转移（DM）分别有5例（21％）和4例（16％）。分别。RT后的中位随访时间为47个月（范围：11至162个月）。2年和5年总生存率，无事件生存，局部无复发生存，胸膜无复发生存率分别为83%和48%,48%和42%,74%和48%，61%和52%，分别。总体局部控制率为83％，胸膜控制率为67％。RT耐受性良好，3级急性皮炎1例,3级亚急性放射性肺炎1例。在3例（13％）中观察到晚期毒性。
结论：即使在患有DM的胸壁ES/PNET的患者中，扩展视野RT也可以实现长期生存。低毒性率使我们得出结论，采用现代技术的RT是这些患者的有效且安全的治疗方式。
方法：对2003年2月至2020年7月在我科接受放疗的24例18岁以下胸壁组织诊断为ES/PNET患者的资料进行回顾性评估。在有肺转移的患者中，将RT应用于原发部位±整个累及的胸壁和整个肺。
结果：中位年龄为8.5岁（范围：1.5至17岁），15例（63%）患者为女性，9例为男性（37%）。18例（75％）患者为胸腔外肿瘤，6例（25％）为胸腔内肿瘤。诊断时纵隔淋巴结和远处转移（DM）分别有5例（21％）和4例（16％）。分别。RT后的中位随访时间为47个月（范围：11至162个月）。2年和5年总生存率，无事件生存，局部无复发生存，胸膜无复发生存率分别为83%和48%,48%和42%,74%和48%，61%和52%，分别。总体局部控制率为83％，胸膜控制率为67％。RT耐受性良好，3级急性皮炎1例,3级亚急性放射性肺炎1例。在3例（13％）中观察到晚期毒性。
结论：即使在患有DM的胸壁ES/PNET的患者中，扩展视野RT也可以实现长期生存。低毒性率使我们得出结论，采用现代技术的RT是这些患者的有效且安全的治疗方式。
