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Treatment challenges and outcomes for pediatric intracranial ependymoma at a single institution in Iran
Maryam Tashvighi1, Azim Mehrvar1,2, Amir Abbas Hedayati Asl1
1a MAHAK Pediatric Cancer Treatment and Research Center , Tehran , Iran.
Insights
Pediatric ependymoma outcomes in Iran are poor, with 3-year overall survival at 61%. Improved care requires standard radiation therapy, multidisciplinary teams, and second-look surgery for better pediatric brain tumor survival.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Clinical Outcomes Research
Background:
- Limited data on pediatric ependymoma in Iran.
- Study motivated by the scarcity of information on pediatric ependymoma in Iran.
- Need to identify clinical management challenges and improve care guidelines.
Purpose of the Study:
- Determine outcomes for pediatric ependymoma patients in Iran.
- Identify clinical management challenges at a non-governmental hospital.
- Devise guidelines for improving pediatric ependymoma care.
Main Methods:
- Retrospective chart review of pediatric ependymoma patients (<15 years) from 2007-2015.
- Analysis of patient demographics, treatment regimens, follow-up duration, and outcomes.
- Kaplan-Meier method used to determine 3-year overall survival (OS) and progression-free survival (PFS) based on age at diagnosis.
Main Results:
- 73 eligible patients (20 younger than 3 years, 53 older).
- Majority (91.8%) had surgical resection; 8.2% had biopsy.
- 3-year OS was 61%, 3-year PFS was 59.5%. Median follow-up: 25 months; median PFS: 17 months.
- 21 patients experienced recurrence; median time to relapse: 1 year.
Conclusions:
- Iranian children with ependymoma exhibit inferior outcomes compared to international standards.
- A paradigm shift is necessary for improved pediatric ependymoma care.
- Recommendations include standardizing radiation therapy, implementing second-look surgery, and adopting a multidisciplinary team approach.
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.
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