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Challenges in Treating Childhood Infratentorial Ependymoma: A Low- and Middle-Income Country Experience
Mithra Ghalibafian1, Sajad Mirzaei2, Theodore Girinsky3
1Department of Radiation Oncology, MAHAK Pediatric Cancer Treatment and Research Center, Tehran, Iran.
Insights
Timely radiation therapy is crucial for treating pediatric posterior fossa ependymoma in low- and middle-income countries. Delayed treatment significantly worsens survival rates, highlighting the need for improved access and adherence to follow-up care.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Neuro-oncology
Background:
- Patients in low- and middle-income countries (LMICs) face significant challenges in accessing specialized pediatric cancer care.
- Limited expertise and suboptimal diagnostic/treatment modalities hinder effective management of childhood cancers.
Purpose of the Study:
- To report the experience of treating pediatric posterior fossa ependymoma (PFE) at MAHAK, a dedicated childhood cancer charity in Iran.
- To analyze the outcomes of PFE treatment in a resource-limited setting.
Main Methods:
- Retrospective review of pediatric PFE patients treated at MAHAK between November 2008 and January 2016.
- Collection and analysis of data on pre-referral management, in-hospital treatment, and patient outcomes.
Main Results:
- Of 54 PFE patients, 43 received adjuvant radiation therapy and 11 were initially irradiated post-recurrence.
- Patients irradiated initially after recurrence had poor outcomes (5-year OS: 27%).
- Timely radiation therapy (within 77 days of surgery) significantly improved 5-year OS (74% vs 32%; P=.05).
- Poor compliance with follow-up was observed, with cognitive decline noted in 50% of assessed patients.
- Growth hormone deficiency was detected but untreated in all cases.
Conclusions:
- Access to pediatric neurosurgery, anesthesia, and timely radiation therapy are critical challenges in LMICs.
- Chemotherapy is not a suitable substitute for delaying radiation therapy in young children with PFE.
- Emphasizing the importance of long-term follow-up for pediatric cancer patients is essential for parents and medical teams.
Purpose:
Patients and physicians in low- and middle-income countries (LMICs) face challenges owing to limited expertise and suboptimal access to appropriate diagnostic and treatment modalities. We report our experience in treating posterior fossa ependymoma (PFE) at MAHAK, a charity organization in Iran whose radiation oncology department is the only one exclusively dedicated to childhood cancer in the whole country.
Methods And Materials:
Pediatric patients with PFE referred to MAHAK between November 2008 and January 2016 were identified. Details on investigations and management done before referral were collected. Management at MAHAK and patient outcomes were analyzed.
Results:
Of 80 patients diagnosed as having ependymoma, 54 with PFE were identified. Forty-three patients received adjuvant radiation therapy, and 11 were irradiated initially after recurrence. At a median follow-up of 5.1 years (range, 0.3-9.7 years), the latter group had the worst outcome, with a 5-year overall survival (OS) rate of 27% (95% CI, 7%-54%). Patients who started radiation therapy within 77 days after initial surgery had a better outcome compared with those who started later (5-year OS: 74% vs 32%; P = .05). Compliance with follow-up recommendations was poor. Only 22% of the patients had at least 2 IQ test assessments, and 50% showed some decline over time. Three cases of growth hormone deficiency were detected, but none of the patients received replacement therapy.
Conclusions:
Access to pediatric neurosurgery, anesthesia, and timely radiation therapy are among the most challenging obstacles to be overcome in LMICs. Our series confirmed that chemotherapy is not an appropriate option for delaying radiation therapy, especially in young children. The importance of long-term follow-up should be acknowledged by the parents and medical team.
