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. 2018 Jun;138(2):435-445.
doi: 10.1007/s11060-018-2815-7. Epub 2018 Mar 6.

Patterns of relapse for children with localized intracranial ependymoma

Brian De  1 , Yasmin Khakoo  2   3   4 , Mark M Souweidane  3   5   6 , Ira J Dunkel  2   3 , Suchit H Patel  1 , Stephen W Gilheeney  2 , Kevin C De Braganca  2   4 , Matthias A Karajannis  2 , Suzanne L Wolden  7
Affiliations

Patterns of relapse for children with localized intracranial ependymoma

Brian De et al. J Neurooncol. 2018 Jun.

Abstract

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.

Keywords: CNS tumors; Chemotherapy; Ependymoma; Radiation therapy; Surgery.

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Conflict of interest statement

Conflict of interest

All other authors declare no conflicts.

Figures

Fig. 1
Fig. 1
Summary of treatments used. GTR gross total resection, STR subtotal resection, RT radiotherapy, chemo chemotherapy
Fig. 2
Fig. 2
Kaplan-Meier OS and FFS curves for patients presenting to our institution for upfront management. Median follow-up duration was 4.4 years (range 0.2–32.9 years). OS overall survival, FFS failure-free survival
See this image and copyright information in PMC

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