Paediatric Oncology Unit, A. Gemelli University Hospital, Catholic University of Sacred Hearth, Largo A. Gemelli, 8, 00168, Rome, Italy.
Pediatric Surgery Unit Gemelli University Hospital, Catholic University of Sacred Heart, Rome, Italy.
World J Surg Oncol. 2018 Jul 13;16(1):140. doi: 10.1186/s12957-018-1434-2.
We report our experience and outcomes about the management of Askin's tumors [AT], which are rare primitive neuroectodermal tumors (PNETs) that develop within the soft tissue of the thoracopulmonary region, typically in children and adolescents.
We retrospectively analyzed the charts of 9 patients affected by AT (aged 6-15 years), treated at the Paediatric Oncology Unit of Gemelli University Hospital in Rome between January 2001 and December 2016.
All nine patients underwent to biopsy followed by neoadjuvant chemotherapy. At the end of the neoadjuvant chemotherapy, they underwent to surgical removal of the residual tumor. Five patients with positive tumor margins and/or necrosis< 90% received local radiotherapy. Two patients with metastasis received an intensified treatment, with the addition of high dose adjuvant chemotherapy followed by peripheral blood stem cells rescue. No statistically significant correlation was found between outcome and gender; the presence of any metastasis and the radiotherapy. The overall survival was 65.14 months (95% confidence interval [95%CI], 45.81-84.48), and the 5 years survival was 60%, at a median follow-up of 53.1 months.
Our study confirms that a multimodal treatment with surgery, chemotherapy, and radiotherapy may increase the survival in AT pediatric patients.
我们报告了我们在 Askin 肿瘤（AT）管理方面的经验和结果，AT 是一种罕见的原始神经外胚层肿瘤（PNET），在胸肺区域的软组织中发展，通常发生在儿童和青少年中。
我们回顾性分析了 2001 年 1 月至 2016 年 12 月期间在罗马杰梅利大学医院儿科肿瘤病房接受治疗的 9 例 AT 患者的病历。
所有 9 例患者均接受了活检，随后进行了新辅助化疗。在新辅助化疗结束时，他们接受了残余肿瘤的手术切除。5 例肿瘤边缘阳性和/或坏死<90%的患者接受了局部放疗。2 例有转移的患者接受了强化治疗，在接受高剂量辅助化疗后，进行外周血干细胞解救。结果与性别、是否有任何转移和放疗之间无显著相关性。总生存率为 65.14 个月（95%置信区间[95%CI]：45.81-84.48），5 年生存率为 60%，中位随访时间为 53.1 个月。
我们的研究证实，手术、化疗和放疗的多模态治疗可能会提高 AT 儿科患者的生存率。
