Treatment challenges and outcomes for pediatric intracranial ependymoma at a single institution in Iran

ABSTRACT

Background: 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.

Procedure: 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.

Results: 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.

Conclusion: 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.

KEYWORDS:

• Disease management
• ependymoma
• Iran
• pediatric oncology

Conflict of interest

The authors have no conflicts of interest to declare.

Acknowledgment

We thank colleagues in the Medical Records department at MPCTRC who provided the medical documents for this review, Mr. Tim Hammond for assistance with graphic design of the figures, and Dr. Angela McArthur for editing the manuscript. Also, we thank late Dr. Elham Olad for help during the initial steps of this project.