Rethinking childhood ependymoma: a retrospective, multi-center analysis reveals poor long-term overall survival

• DOI: 10.1007/s11060-017-2568-8
• Publication Date: 2017-07-21T06:09:30Z
• Authors: Amanda E. Marinoff; Clement Ma; Dongjing Guo; Matija Snuderl; Karen D. Wright; Peter E. Manley; Hasan Al-Sayegh; Claire E. Sinai; Nicole J. Ullrich; Karen Marcus; Daphne Haas-Kogan; Liliana Goumnerova; Wendy B. London; Mark W. Kieran; Susan N. Chi; Jason Fangusaro; Pratiti Bandopadh...
• Keywords: Ependymoma

Ependymoma is the third most common brain tumor in children, but there a paucity of large studies with more than 10 years follow-up examining long-term survival and recurrence patterns this disease. We conducted retrospective chart review 103 pediatric patients WHO Grades II/III intracranial ependymoma, who were treated at Dana-Farber/Boston Children's Cancer Blood Disorders Center Chicago's Ann & Robert H. Lurie Hospital between 1985 2008, an additional 360 ependymoma identified from Surveillance Epidemiology End Results (SEER) database. For institutional cohort, we evaluated clinical histopathological prognostic factors overall (OS) progression-free (PFS) using log-rank test, univariate multivariate Cox proportional-hazards models. Overall rates compared to those SEER cohort. Median time was 11 years. Ten-year OS PFS 50 ± 5% 29 5%, respectively. Findings validated independent 10-year 52 3%. GTR grade II pathology associated significantly improved OS. However, not curative for all children. 61 7% 36 6%. Pathological examination confirmed recurrent tumors be 74% occurred primary site. Current treatment paradigms are sufficient provide cure children ependymoma. Our findings highlight urgent need develop novel approaches devastating