Metastatic ependymoma: a multi-institutional retrospective analysis of prognostic factors
Stergios Zacharoulis1, Lingyun Ji, Ian F Pollack
1Department of Pediatrics, The Royal Marsden Hospital, Sutton, Surrey, UK. stergios.zacharoulis@rmh.nhs.uk
Purpose:
Metastatic ependymoma is exceedingly rare at diagnosis with variable prognosis reported in the literature. The purpose of this study was to identify prognostic factors in children with metastatic ependymoma.
Procedure:
Data regarding diagnosis, treatment and follow-up for 40 patients from eight institutional cohorts were collected.
Results:
Twenty-nine (72%) patients were less than 36 months of age at the time of diagnosis, 28% were females, and 90% of the patients had posterior fossa tumors. Gross total resection (GTR) of the primary tumor was achieved in 16 patients (40%). Adjuvant therapy was variable and included craniospinal irradiation (CSRT), chemotherapy, and chemotherapy with focal irradiation. The 5-year event free survival (EFS) and overall survival (OS) from the time of diagnosis were 29% (+/-7%) and 43% (+/-8%), respectively. Age at diagnosis was associated significantly with both EFS and OS (P < 0.001 for EFS, and P = 0.01 for OS). Patients who were 24-35 months of age at diagnosis had a 5-year EFS of 66% and a 5-year OS of 73%; both survival rates were superior to those of patients younger than 24 months of age or older than 36 months. Patients with GTR achieved a 5-year EFS of 35% and OS of 59%, compared to a 5-year EFS of 25% and OS of 32% for patients who did not achieve GTR (P = 0.12 for EFS, P = 0.03 for OS).
Conclusions:
GTR should be attempted in patients with metastatic ependymoma. A subgroup of patients between 24 and 35 months might have a favorable outcome.
