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Intracranial ependymomas in children
J W Goldwein1, J M Leahy, R J Packer
1Radiation Oncology Center, Hospital of the University of Pennsylvania, Philadelphia 19104.
Insights
Local control of intracranial ependymal tumors is crucial for treatment success. Higher radiation doses and Caucasian race are favorable prognostic factors for survival in pediatric patients.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Radiation Oncology
Background:
- Intracranial ependymal tumors are a significant challenge in pediatric neuro-oncology.
- Treatment strategies have evolved, but local control remains a critical issue.
Purpose of the Study:
- To evaluate treatment outcomes and identify prognostic factors for children with intracranial ependymal tumors.
- To analyze the impact of various therapeutic modalities on survival and progression-free survival.
Main Methods:
- Retrospective analysis of 51 children treated between 1970 and 1988.
- Evaluation of factors including tumor resection extent, radiation dose, patient age, race, and chemotherapy use.
- Calculation of 5-year actuarial survival and progression-free survival (PFS) rates.
Main Results:
- 5-year survival and PFS rates were 46% and 30%, respectively.
- Local disease control was significantly better with tumor doses exceeding 4500 cGy and in Caucasian patients.
- Survival was improved for patients over 4 years old, receiving local radiation doses above 4500 cGy, and for Caucasian patients.
Conclusions:
- Inability to achieve local disease control is the primary cause of treatment failure.
- Older age at diagnosis, higher local radiation dose, and Caucasian race are favorable prognostic indicators.
- Extent of resection and chemotherapy did not significantly impact survival in this cohort.
Abstract:
Between 1970 and 1988, 51 children with intracranial ependymal tumors (33-infratentorial, 18-supratentorial received initial treatment at the University of Pennsylvania. Therapy consisted of total or near total tumor resection in 15 patients and partial resection or biopsy in 36. Postoperative irradiation alone was given to 18, chemotherapy to 4, and a combination of these two modalities to 26. Patients have been followed for a median period of 7.75 years. The 5-year actuarial survival and progression-free survival (PFS) rates are 46% and 30%, respectively. Of the 30 patients who have progressed, 29 did so locally and one died before the site of failure could be determined. Six patients also had disease outside the primary site at relapse; three of them had received craniospinal irradiation. Local control was significantly better for patients whose tumor dose exceeded 4500 cGy (32% vs. 0%, p = .01) and for Caucasian patients (34% vs. 15%, p =.05). Survival was better for patients who were over 4 years of age at diagnosis (55% vs. 30%, p = .04), for patients who received local radiation doses above 4500 cGy (51% vs. 18%, p = .01), and for Caucasian patients (43% vs. 14%, p = .01). Extent of resection, histology, location, the use of cranial or craniospinal irradiation, and the use of chemotherapy did not significantly impact on survival. We conclude that the inability to control local disease remains the single most important factor leading to treatment failure. Older age, higher local radiation dose, and Caucasian race appear to be the only favorable prognostic factors.