Patterns of relapse for children with localized intracranial ependymoma.

TitlePatterns of relapse for children with localized intracranial ependymoma.
Publication TypeJournal Article
Year of Publication2018
AuthorsDe B, Khakoo Y, Souweidane MM, Dunkel IJ, Patel SH, Gilheeney SW, De Braganca KC, Karajannis MA, Wolden SL
JournalJ Neurooncol
Date Published2018 Jun
KeywordsAdolescent, Brain Neoplasms, Child, Child, Preschool, Disease Management, Ependymoma, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Recurrence, Retrospective Studies, Survival Analysis, Treatment Outcome, Young Adult

We examined patterns of relapse and prognostic factors in children with intracranial ependymoma. Records of 82 children diagnosed with localized intracranial ependymoma were reviewed. 52% first presented to our institution after relapse. Median age at initial diagnosis was 4 years (range 0-18 years). Gender was 55% male. Initial tumor location was infratentorial in 71% and supratentorial in 29%. Histology was WHO Grade II in 32% and Grade III in 68%. As part of definitive management, 99% had surgery, 70% received RT (26% 2D/3D-conformal RT[CRT], 22% intensity-modulated RT [IMRT], 22% proton), and 37% received chemotherapy. Median follow-up was 4.6 years (range 0.2-32.9). Overall, 74% of patients relapsed (50% local, 17% distant, 7% local + distant) at a median 1.5 (range 0.1-17.5) years. Five-year OS and FFS for patients presenting prior to relapse are 70% (95% confidence interval [CI], 50-83%) and 48% (95% CI 30-64%), respectively. On log-rank, superior overall survival (OS) was demonstrated for gross total resection (p = 0.03). Superior failure-free survival (FFS) was demonstrated for age < 5 years (p = 0.04). No difference in OS or FFS was found between 2D/3D-CRT versus IMRT/proton (p > 0.05). On multivariate analysis, age ≤ 5 was independently associated with a lower risk of death and failure versus older patients (p < 0.05). Contrary to previous reports, young age may not be a poor prognostic factor in patients who can tolerate intensive treatment. Future studies examining patients stratified by clinical and molecular attributes are warranted.

Alternate JournalJ Neurooncol
PubMed ID29511977
PubMed Central IDPMC6756472
Grant ListP30 CA008748 / CA / NCI NIH HHS / United States