Premium
Metastatic ependymoma: A multi‐institutional retrospective analysis of prognostic factors
Author(s) -
Zacharoulis Stergios,
Ji Lingyun,
Pollack Ian F.,
Duffner Patricia,
Geyer Russel,
Grill Jacques,
Schild Steven,
Jaing TangHer,
Massimino Maura,
Finlay Jonathan,
Sposto Richard
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21276
Subject(s) - medicine , ependymoma , chemotherapy , radiation therapy , overall survival , prognostic variable , retrospective cohort study , pediatrics , surgery , oncology
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. Pediatr Blood Cancer 2008;50:231–235. © 2007 Wiley‐Liss, Inc.