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Prognostic factors in intracranial ependymomas in children
D Figarella-Branger1, M Civatte, C Bouvier-Labit
1Service d'Anatomie Pathologique et de Neuropathologie, Hôpital de la Timone, Marseille, France. Dominique.Figarella-Branger@medecine.univ-mrs.fr
Insights
Complete surgical removal is key for treating pediatric intracranial ependymomas. Histological features and Ki-67 labeling index predict outcomes, guiding personalized treatment strategies for better survival rates.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Cancer pathology
Background:
- Intracranial ependymomas in children are rare, with controversial prognostic factors, particularly histological composition.
- Understanding these factors is crucial for improving treatment outcomes.
Purpose of the Study:
- To identify prognostic factors influencing survival in pediatric intracranial ependymomas.
- To evaluate the role of histological features and adjuvant therapies on outcomes.
Main Methods:
- Retrospective analysis of 37 children with intracranial ependymomas over 20 years.
- Univariate and multivariate analyses of patient age, sex, tumor location, extent of resection, histology, and adjuvant therapies.
- Histopathological analysis included endothelial proliferation, necrosis, differentiation, mitotic index, cellularity, and Ki-67 labeling index (LI).
Main Results:
- Five-year overall survival (OS) and progression-free survival (PFS) rates were 45% and 25%, respectively.
- Total surgical resection and median infratentorial location were associated with better outcomes.
- Loss of differentiating structures, necrosis, endothelial proliferation, high mitotic index, and Ki-67 LI > 1 were negative prognostic factors.
- Adjuvant therapies did not improve prognosis in cases with complete resection but enhanced PFS in incomplete resections.
Conclusions:
- Total tumor removal is the primary treatment for pediatric intracranial ependymomas.
- Histological parameters and Ki-67 LI are valuable predictors of outcome.
- Distinguishing median infratentorial ependymomas from lateral types is important for surgical planning.
Object:
The occurrence of intracranial ependymomas in children is relatively infrequent, and their prognostic factors are still controversial, especially regarding histological composition.
Methods:
A retrospective study was conducted of 37 children treated during the last 20 years for intracranial ependymomas at the Hôpital de la Timone. Both univariate and multivariate statistical analyses were performed to assess the prognostic relevance of patient age and sex, extent of tumor removal, location of the tumor (supratentorial compared with infratentorial, median compared with lateral), tumor histological composition, and adjuvant therapies in affecting the 5-year progression-free survival (PFS) rate and overall survival (OS) rate. The following histopathological features, either alone or in combination, were analyzed: endothelial proliferation, necrosis, loss of differentiating structures (present compared with absent), the number of mitotic figures per 10 hpf, and cellularity (number of nuclei/5 hpf). In addition, immunohistochemical detection of Ki-67 antigen was performed and the Ki-67 labeling index (LI) evaluated in all cases. The 5-year OS and PFS rates were 45% and 25%, respectively (median follow up 34 months). Four patients died of disease without remission (median 163 days) and disease in 21 patients relapsed: 18 in situ and three both in situ and distantly. On univariate analysis total surgical resection and median infratentorial location were associated with a better outcome (p < 0.002) for both OS and PFS. Loss of differentiating structures was associated with poor prognosis (p < 0.008) and the combination of necrosis, endothelial proliferation, and mitotic index greater than 5 was also a negative predictive factor for both OS (p < 0.002) and PFS (p = 0.02). The PFS time was shorter in patients younger than 4 years of age and in patients in whom a Ki-67 LI greater than 1 was found (p = 0.03 and 0.006, respectively). Adjuvant radiotherapy and chemotherapy were not relevant to prognosis. Moreover, among the 15 patients in whom total excision was achieved, OS was better in those who did not receive adjuvant therapies. In contrast, adjuvant therapies significantly enhanced PFS time in patients in whom tumor excision was incomplete.
Conclusions:
This study and analysis of the literature further highlight that total tumor removal is the treatment of choice for ependymomas in children. Postoperative measurement of residual tumor is required, especially because a subgroup of patients might be treated by surgery alone. Median infratentorial ependymomas have to be distinguished from the lateral type. Appropriate and reproducible histological parameters and Ki-67 LI are of interest as predictors of outcome.