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Intracranial ependymoma in children: analysis of prognostic factors
1Department of Clinical Radiotherapy, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Insights
Long-term cure rates for pediatric intracranial ependymoma remain suboptimal. Local recurrence is the primary failure pattern, suggesting a need for improved treatment strategies for this childhood brain tumor.
Area of Science:
- Pediatric Oncology
- Neuro-Oncology
- Radiation Oncology
Background:
- Intracranial ependymoma is a significant pediatric brain tumor.
- Treatment outcomes for children with ependymoma have historically been challenging.
Purpose of the Study:
- To evaluate the long-term outcomes and treatment patterns for pediatric intracranial ependymoma.
- To identify prognostic factors influencing survival and recurrence in this patient cohort.
Main Methods:
- Retrospective analysis of 25 children treated between 1955 and 1986.
- Review of treatment modalities including surgery, craniospinal irradiation, local field irradiation, and chemotherapy.
- Analysis of tumor location (supratentorial vs. infratentorial), grade, and patient demographics.
Main Results:
- The overall long-term cure rate was suboptimal, with local recurrence being the main pattern of failure.
- Histologic grade appeared to be a prognostic factor for supratentorial tumors.
- Prognosis was worse for female patients and infants under 3 years of age.
- Survival data showed 8 patients alive and disease-free for up to 12.5 years.
Conclusions:
- Current treatment strategies yield suboptimal long-term cure rates for pediatric intracranial ependymoma.
- Histologic grade, patient sex, and age are potential prognostic indicators.
- Routine spinal irradiation may be safely omitted in well-staged patients, and the role of adjuvant chemotherapy requires further investigation.
Abstract:
Between 1955 and 1986, 25 children (aged 2 weeks to 15 years) were treated for intracranial ependymoma at M.D. Anderson Cancer Center. Nine patients had supratentorial primaries (5 high-grade, 4 low-grade), and 16 had infratentorial primaries (9 high-grade, 7 low-grade). Five patients had gross complete resection and 20 had incomplete resection. Seven patients received craniospinal irradiation (25-36 Gy to the neuro-axis, 45-55 Gy to tumor bed), 12 received local field irradiation (29-60 Gy, median 50 Gy). Five infants had adjuvant chemotherapy without radiotherapy, and 6 children had post-radiotherapy adjuvant chemotherapy, and 12 patients had salvage chemotherapy with various agents and number of courses. Eight patients are alive, disease-free and without relapse from 1 year to 12 1/2 years from diagnosis (median 42 months). The primary failure pattern was local recurrence. The data suggest that 1) the long-term cure rate of children with ependymoma is suboptimal; 2) histologic grade may be of prognostic importance for supratentorial tumors; 3) prognosis appears worse for girls and infants under 3 years of age; 4) in well-staged patients routine spinal irradiation could be omitted; 5) the role of adjuvant chemotherapy is unclear.
