Supratentorial cortical ependymoma: A systematic literature review and case illustration

Abstract

Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21.2 years. Males and females comprised 58.8% (90/153) and 41.2% (63/153) of cases, respectively. The most common presenting symptom was seizure activity occurring in 44.4% of the cohort (68/153). The recently recognized C11orf95-RELA fusion was identified in 13.7% of the cohort (21/153) and 95.5% of cases (21/22) reporting molecular characterization. World Health Organization grades 2 and 3 were reported in 52.3% (79/151) and 47.7% (72/151) of cases, respectively. The frontal lobe was involved in the majority of cases (54.9%, 84/153). Gross total resection was achieved in 80.4% of cases (123/153). Tumor recurrence was identified in 27.7% of cases (39/141). Mean clinical follow-up was 41.3 months. Mean overall survival of patients who expired was 27.4 months whereas mean progression-free survival was 15.0 months. Comparatively, cortical ependymomas with C11orf95- RELA fusions and supratentorial ependymomas with C11orf95 RELA fusions exhibited differing clinical outcomes. Further studies with larger sample sizes are necessary to investigate the significance of RELA fusions on survival in cortical ependymomas and to determine whether cortical ependymomas with C11orf95- RELA fusions should be classified as a distinct entity.

Astroblastoma With a Novel YAP1::BEND2 Fusion: A Case Report

“…However, our case exhibited a novel YAP1::BEND2 fusion alteration rather than the previously described MN1::BEND2 fusion. To our knowledge, YAP1 fusions have not previously been identified in astroblastoma; rather, YAP1 rearrangements are known to define one subgroup of supratentorial ependymomas 10,20 . Microarray analysis demonstrated chromothripsis of chromosomes X and 11, with results supporting the YAP1 rearrangement.…”

“…To our knowledge, YAP1 fusions have not previously been identified in astroblastoma; rather, YAP1 rearrangements are known to define one subgroup of supratentorial ependymomas. 10,20 Microarray analysis demonstrated chromothripsis of chromosomes X and 11, with results supporting the YAP1 rearrangement. Interestingly, chromothripsis 11 has been reported in several brain tumors, most commonly involving YAP1 in supratentorial ependymomas.…”

Cortical Ependymoma Presenting as Subclinical Seizures in a Very Young Child

“…In the pediatric population, the majority of cases are infratentorial and may be complicated by incomplete resection whereas supratentorial lesions are more amenable to complete resection. The role of chemotherapy in children is still under debate but may be beneficial in very young children at risk of extensive radiation damage [ 7 ].…”

“…A recently published study by Cuoco et al based on a systematic review of published cases of supratentorial ependymomas showed the mean age of presentation as 21.2 years in a reported number of 153 cases. Out of the total, 47 cases were of pediatric age groups, and only nine cases involved very young children less than three years [ 7 ]. Two other cases of supratentorial cortical ependymoma in very young children were reported by Mehdi et al and Khilji et al in 2016 and 2014, respectively [ 2 , 4 ].…”