Tashvighi Maryam, Mehrvar Azim, Hedayati Asl Amir Abbas, Mehrvar Narjes, Ghorbani Raheb, Naderi Ali, Alebouyeh Mardawig, Sattary Erfan, Niktoreh Mofrad Naghmeh, Qaddoumi Ibrahim, Faranoush Mohammad
a MAHAK Pediatric Cancer Treatment and Research Center , Tehran , Iran.
b AJA University of Medical Sciences , Tehran , Iran.
Pediatr Hematol Oncol. 2018 Feb;35(1):60-75. doi: 10.1080/08880018.2018.1435758. Epub 2018 Mar 22.
The scarcity of information on pediatric ependymoma in Iran motivated this study. Our main objectives were to determine outcomes, identify clinical management challenges at a nongovernment hospital in Iran, and devise guidelines for improving care.
A retrospective chart review was performed for pediatric patients with ependymoma who were younger than 15 years and treated at MPCTRC between 2007 and 2015. Records included patient demographics, treatment regimens used, duration of follow-up, and outcomes. Clinical outcomes [ie, 3-year overall survival (OS) and progression-free survival (PFS)] were determined based on the age at diagnosis (younger or older than 3 years) by using the Kaplan-Meier method.
In total, 73 eligible patients were enrolled; 20 patients were in the younger group, and 53 were in the older group. The majority (91.8%, n = 67) of patients underwent initial gross-total or partial surgical resection, and 6 (8.2%) had a biopsy. Twenty-one patients experienced ependymoma recurrence. The median time to relapse was 1 year. The median duration of follow-up and PFS were 25 and 17 months, respectively. The 3-year OS and PFS were 61% and 59.5%, respectively. At the time of this project, 27 patients had died, and 35 were alive with no evidence of disease.
Our study demonstrated inferior outcomes of Iranian children with ependymoma. To improve our care for these children, a paradigm shift must occur that includes radiation therapy as standard of care, second-look surgery, a multidisciplinary team approach, and potentially twinning initiatives.
伊朗小儿室管膜瘤的信息匮乏促使开展本研究。我们的主要目标是确定治疗结果，识别伊朗一家非政府医院在临床管理方面的挑战，并制定改善护理的指南。
对2007年至2015年期间在MPCTRC接受治疗的15岁以下小儿室管膜瘤患者进行回顾性病历审查。记录包括患者人口统计学资料、使用的治疗方案、随访时间和治疗结果。采用Kaplan-Meier方法根据诊断时的年龄（3岁以下或3岁以上）确定临床结果[即3年总生存率（OS）和无进展生存率（PFS）]。
总共纳入了73例符合条件的患者；20例患者属于较年轻组，53例属于较年长组。大多数患者（91.8%，n = 67）接受了初次大体全切或部分手术切除，6例（8.2%）进行了活检。21例患者出现室管膜瘤复发。复发的中位时间为1年。随访的中位时间和PFS分别为25个月和17个月。3年OS和PFS分别为61%和59.5%。在本项目开展时，27例患者已死亡，35例存活且无疾病证据。
我们的研究表明伊朗患室管膜瘤儿童的治疗结果较差。为改善对这些儿童的护理，必须发生范式转变，包括将放射治疗作为标准治疗方法、二次手术、多学科团队方法以及可能的结对倡议。
