Abstract
BACKGROUND: Ependymal tumors (ET) are neuroepithelial malignancies of the CNS that occur in both children and in 1.8% of adults. Anatomically arise along the entire neuroaxis comprising the hemispheres, the hindbrain and the spinal cord. More than 20% of primary spinal cord tumors are of ET lineage and all have a variable clinical behavior. In 40% of patients (pts) are incurable because of the scarceness of effective treatment options. The 5-year overall survival (OS) is about 70% to 89% in adult pts. The surgical resection extent has been the only clinical prognostic known associated with survival. The current standard of care includes maximal safe surgical resection, followed by focal radiotherapy (RT). Our goal is to review pts outcome with these rare tumors in adults presented in multidisciplinary board to post-operatory RT. MATERIAL AND METHODS: Review of 12 pts with ET diagnosis who underwent RT between 2008–2018. Data collected at Glintt HS ®; Mosaiq ® from our institution database. Local recurrence free survival (LRFS), distance progression-free survival (PFS) and overall survival (OS) were calculated with IBM SPSS Statistics 21.0. Survival curves were generated using Kaplan-Meier method. Surgery resection, histopathological grade WHO classification and RT dose in local recurrence were analyzed. The impact of clinical and therapeutic variables on survival was evaluated by log-rank test. RESULTS: With a median age at diagnosis 44 (23–78) years old. Pts were 2 females and 10 males. Anatomical sites were 1 pt supratentorial; 6 pts infratentorial and 4 pts spinal cord. Staging included in 75% pts lumbar puncture and cranial/neuroaxis MRI. GTR was achieved in 77% of pts. WHO grade was III in 3 pts; grade II in 7 pts and 2 pts grade I. Median RT dose was 54Gy (50.4–59.4) with 2 pts with boost to posterior fossa and one pt 36 Gy for neuroaxis. The 5 and 10-year OS were 78% and 23% respectively, calculated from the surgery data to the last follow-up/death. 5-years free-local recurrence survival (FLRS) 73% calculated from the surgery date to the local recurrence date; with a 5-years distant progression-free survival (PFS) 100%. In this analysis 5-year LRFS was 67% for STR and 80% for GTR (p=0.715); 5-year LRFS was 100% pts WHO I; 0% pts WHO II; 75% pts WHO III (p=0.712) and 5-year LRFS RT doses were 100% > 54 Gy and 75% ≤54Gy (p=0.035). CONCLUSION: Current analysis includes only 12 pts with different anatomical sites ET, with heterogeneous behaviors, has no power to reveal optimum RT dose nor identify pts WHO grade that deferral post-operatory RT. Our 5 year OS likewise publications for adult. Genetic classification can accrue ET, resulting in more precise diagnostics, sharpen prognostic and improved therapy, beeing now surgery and RT therapeutic mainstays.