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Intracranial ependymomas in childhood. Survival and functional results of 47 cases
Insights
This study on pediatric intracranial ependymomas found that infratentorial tumors frequently metastasize to the spine. Researchers recommend craniospinal irradiation for infratentorial ependymomas to improve outcomes.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Radiation oncology
Background:
- Ependymomas are primary tumors of the central nervous system.
- Intracranial ependymomas present unique challenges in pediatric populations.
- Understanding metastatic patterns is crucial for treatment planning.
Purpose of the Study:
- To analyze the clinical characteristics and outcomes of pediatric intracranial ependymomas.
- To investigate the patterns of recurrence and metastasis in these tumors.
- To propose optimized radiotherapy strategies based on tumor location and histology.
Main Methods:
- Retrospective analysis of 47 pediatric intracranial ependymomas operated between 1969-1979.
- Review of treatment modalities including surgery and radiotherapy.
- Evaluation of recurrence rates, metastasis patterns, and survival data.
Main Results:
- Malignant ependymomas constituted 69% of the series.
- Operative mortality was 17%, with a 5-year survival of 39% (51% excluding operative deaths).
- Infratentorial ependymomas showed a high rate of intraspinal metastasis (33%), especially malignant types (50%).
Conclusions:
- Metastases from supratentorial ependymomas are rare and typically remain supratentorial.
- Infratentorial ependymomas have a significant propensity for intraspinal seeding, regardless of malignancy.
- Craniospinal irradiation is recommended for infratentorial ependymomas; localized irradiation for supratentorial ependymomas based on histology.
Abstract:
A pediatric series of 47 intracranial ependymomas (15 supra- and 32 infratentorial) is presented. All children were operated upon between 1969 and 1979. Among these children, 35 completed the treatment with radiotherapy. The irradiation was usually limited to the intracranial content but in 7 patients, it was extended to the spinal axis as well. Malignant ependymomas represented 69% of the whole series (86% of the supratentorial and 53% of the infratentorial ependymomas). The operative mortality rate was 17%. The 5-year survival rate was 39% in the whole series and 51% when excluding postoperative mortality. Recurrences developed in 41% of cases and metastases in 20%. The study of this series and of the literature points out that: (1) metastases from supratentorial ependymomas are not frequent, almost always supratentorial and secondary to a malignant ependymoma, and (2) metastases from infratentorial ependymomas are almost always intraspinal and occur in one third of these tumors. The rate of seeding is especially high in the case of malignant infratentorial ependymomas (50%) but is also 15% in the case of benign tumors. These data lead the authors to propose a craniospinal irradiation in the case of infratentorial ependymomas whether benign or malignant, an irradiation of only the brain in the case of malignant supratentorial ependymoma, an irradiation of only the tumoral bed when ependymomas are benign and supratentorial.