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Current management in the treatment of intramedullary ependymomas in children
Christian Joachim Entenmann1, Adéla Mišove2, Miloslav Holub3
1Department of Neurosurgery, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic. christian.entenmann@gmail.com.
Insights
Gross total resection (GTR) and tumor grade are key for pediatric intramedullary ependymoma survival. This study highlights management inconsistencies and prognostic factors for this rare spinal cord tumor.
Area of Science:
- Pediatric neurosurgery
- Pediatric oncology
- Spinal cord tumors
Background:
- Pediatric intramedullary ependymoma management lacks large-scale studies, often relying on adult data.
- This study addresses the scarcity of pediatric-specific data for this rare tumor.
Purpose of the Study:
- To share institutional experience with pediatric intramedullary ependymoma.
- To compare findings with existing literature.
- To review current management and identify inconsistencies.
Main Methods:
- Retrospective analysis of 5 pediatric patients with intramedullary ependymoma (excluding myxopapillary type) treated between 2004 and 2021.
- Evaluation of tumor grade, extent of surgical resection, adjuvant therapies, and outcomes.
Main Results:
- 4 cases of grade II and 1 case of grade III ependymoma were identified (mean age 11.2 years).
- Gross total resection (GTR) was achieved in 40% of patients, with no recurrences observed post-GTR.
- Subtotal resection (STR) was associated with recurrence in all cases; 80% of patients showed neurological improvement or stability.
- Two patients died of disease during a mean follow-up of 73 months.
Conclusions:
- Gross total resection (GTR) and tumor grade are critical prognostic factors for long-term survival.
- Uncertainties remain regarding optimal adjuvant therapy and prognostic indicators in pediatric intramedullary ependymoma.
Purpose:
Current management of pediatric intramedullary ependymoma is extrapolated from adult series since large studies in children are unavailable. This has led us to share our experience with this rare tumor and compare it to the literature and to review and highlight important aspects of current management and point out inconsistencies.
Methods:
This is a retrospective analysis of patients with intramedullary ependymoma managed at our institution between 2004 and 2021.
Results:
During the study period, 5 patients were treated for intramedullary ependymoma. Cases of myxopapillary ependymoma were excluded. The mean age of our cohort was 11.2 years. We identified 4 cases of grade II ependymoma and 1 case of grade III ependymoma. Gross tumor removal (GTR) was achieved in two patients (40%) of patients. One patient was treated with radiotherapy for recurrence and two patients received chemotherapy. There were no cases of recurrence among patients treated with GTR, but in all patients treated with STR. Eighty percent of patients either improved or stayed stable neurologically. During follow-up (mean 73 months), 2 patients died of disease.
Conclusion:
GTR and tumor grade remain the key prognostic factor of long-term tumor-free survival. Many questions prevail regarding outcomes, correct use of adjuvant therapy, and prognostic factors.
