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Long-term life expectancy for children with ependymoma and medulloblastoma
Jonathan E Frandsen1, Aaron Wagner1, Robert J Bollo2
1Department of Radiation Oncology, Huntsman Cancer Hospital, University of Utah School of Medicine, Salt Lake City, Utah.
Insights
Children surviving 5 years after diagnosis of medulloblastoma (MB) or intracranial ependymoma (IE) have good long-term survival potential. MB patients show better survival than IE patients, though deaths can occur decades later.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Survival Analysis
Background:
- Long-term follow-up data for pediatric intracranial ependymoma (IE) and medulloblastoma (MB) survivors is limited.
- Understanding long-term survival potential is crucial for assessing quality of life and potential for a normal lifespan in these young patients.
Purpose of the Study:
- To determine the long-term survival potential in children diagnosed with MB or IE who have survived at least 5 years post-diagnosis.
- To analyze survival trends decades after initial diagnosis for pediatric brain tumor survivors.
Main Methods:
- Retrospective analysis of the Surveillance, Epidemiology, and End Results (SEER) Program database.
- Identification of pediatric patients (0-19 years) diagnosed with MB or IE between 1973 and 2011.
- Creation of a cohort of 5-year survivors and application of Cox proportional hazards models and Kaplan-Meier estimates for long-term survival analysis.
Main Results:
- The study identified 876 MB and 474 IE patients alive 5 years post-diagnosis.
- Thirty-year overall survival (OS) and cancer-specific survival (CSS) for MB survivors were 70.2% and 80.1%, respectively.
- Thirty-year OS and CSS for IE survivors were 57.3% and 68.8%, respectively, with MB showing improved CSS (P=0.04).
Conclusions:
- A notable proportion of deaths related to disease occur decades after treatment for both IE and MB.
- Long-term survival is achievable for a significant number of 5-year survivors of both pediatric brain tumor types.
- Patients with medulloblastoma demonstrate a comparatively lower risk of disease-related death than those with intracranial ependymoma when considering 5-year survivors.
Objectives:
There is a paucity of long-term follow-up data for children with intracranial ependymoma (IE) and medulloblastoma (MB). What happens to these children 20, 30, or 40 years after diagnosis? Do they have potential for a normal lifespan? The purpose of this study was to ascertain the long-term survival potential in children with MB or IE who have survived 5 years from diagnosis.
Methods:
A retrospective analysis was conducted using the SEER Program. Children (ages 0-19 years) from 1973 to 2011 with a diagnosis of MB or IE were identified. A cohort was created of potentially cured patients who survived 5 years from diagnosis. Cox proportional hazards models and Kaplan-Meier estimates were utilized to analyze long-term survival.
Results:
We identified 876 patients with MB and 474 patients with IE who were alive 5 years from diagnosis. Patients with MB had a 30-year overall survival (OS) and cancer-specific survival (CSS) of 70.2% and 80.1%, respectively. Patients with IE had a 30-year OS and CSS of 57.3% and 68.8%, respectively. When comparing MB with IE, MB had improved CSS (P = 0.04) and trended toward increased OS (P = 0.10).
Conclusions:
A significant number of deaths due to disease occur for several decades after treatment for both IE and MB. Despite this, the potential for long-term survival exists in 5-year survivors of both histologies. If alive at 5 years from diagnosis, patients with MB tend to have a lower risk of death from disease compared to those with IE.
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