University Department of Oral and Maxillofacial Surgery at 'A. & P. Kyriakou' Children's Hospital of Athens (Head: Professor Ioannis Iatrou), Dental School, University of Athens, Thivon and Levadias Str, 11527, Ampelokipi, Athens, Greece.
University Department of Oral and Maxillofacial Surgery at 'A. & P. Kyriakou' Children's Hospital of Athens (Head: Professor Ioannis Iatrou), Dental School, University of Athens, Thivon and Levadias Str, 11527, Ampelokipi, Athens, Greece.
J Craniomaxillofac Surg. 2018 Feb;46(2):213-221. doi: 10.1016/j.jcms.2017.12.009. Epub 2017 Dec 16.
The rarity of Ewing's sarcoma (ES) in the maxillofacial region of children, coupled with the technical challenge of resection and associated functional and cosmetic impairment has resulted in deficient data regarding the optimal local control of the disease.
To describe our experience in the management of primary maxillofacial ES in children, focusing on the therapeutic modalities for local control of the disease.
Single institution observational study.
This is a single institution review of patients, treated between 2007 and 2016.
Six primary maxillofacial ES were treated according to the EURO-EWING 99 protocol, consisting of a uniform chemotherapy regimen, combined selectively with surgery and radiotherapy as local treatment. Patients' mean age was 9.42 years (range 6-12.5 years). One patient initially suffered from metastasis and succumbed to the disease; another refused further treatment following chemotherapy and was lost to follow-up. Four patients underwent surgery and adjuvant radiotherapy successfully. At a mean follow-up of 3.78 years relapse-free and overall survival rates were 60% and 80% respectively. The aesthetic and functional outcome was satisfactory in all treated patients.
In eligible cases the combination of chemotherapy with surgery and adjuvant radiotherapy results in optimal oncological and functional outcome for children with ES of the maxillofacial region. Metastasis and poor response to chemotherapy are the most important adverse prognostic factors.
儿童颌面部尤文肉瘤（ES）较为罕见，且由于切除手术难度大，术后会导致功能和美容受损，因此关于该疾病的最佳局部控制方法的数据有限。
描述我们在儿童原发性颌面部 ES 治疗方面的经验，重点介绍疾病局部控制的治疗方法。
单机构观察性研究。
这是对 2007 年至 2016 年间在我们机构接受治疗的患者进行的单机构回顾性研究。
根据 EURO-EWING 99 方案，6 例原发性颌面部 ES 患者接受了治疗，方案包括标准化疗方案，选择性联合手术和放疗作为局部治疗。患者的平均年龄为 9.42 岁（范围 6-12.5 岁）。1 例患者最初发生转移，最终死亡；另 1 例患者在化疗后拒绝进一步治疗并失访。4 例患者成功接受了手术和辅助放疗。在平均 3.78 年的随访中，无复发生存率和总生存率分别为 60%和 80%。所有接受治疗的患者的美学和功能结果均令人满意。
在符合条件的情况下，化疗联合手术和辅助放疗可为儿童颌面部 ES 患者带来最佳的肿瘤学和功能结果。转移和化疗反应不良是最重要的不良预后因素。
