Clinicopathological and Genetic Features of Supratentorial Cortical Ependymomas

Supratentorial cortical ependymomas (CEs) are rare. These lesions, selectively occurring in the superficial cortex, have not been fully characterized. We analyzed the clinicopathological and genetic features of CEs. Eight patients with CEs from our institution and 84 other reported CE cases were inc...

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Bibliographic Details
Published in:World neurosurgery 2019-09, Vol.129, p.e417-e428
Main Authors: Matsumoto, Yuji, Ichikawa, Tomotsugu, Kurozumi, Kazuhiko, Otani, Yoshihiro, Date, Isao
Format: Article
Language:English
Subjects:
Adolescent
C11orf95-RELA fusion
Child
Child, Preschool
Cortical
Ependymoma
Ependymoma - genetics
Ependymoma - pathology
Ependymoma - surgery
Humans
Infant
L1CAM
Neural Cell Adhesion Molecule L1 - metabolism
Oncogene Fusion
Radiotherapy, Adjuvant
Retrospective Studies
Supratentorial Neoplasms - genetics
Supratentorial Neoplasms - pathology
Supratentorial Neoplasms - surgery
Treatment Outcome
Tumor Burden
Young Adult
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Summary:Supratentorial cortical ependymomas (CEs) are rare. These lesions, selectively occurring in the superficial cortex, have not been fully characterized. We analyzed the clinicopathological and genetic features of CEs. Eight patients with CEs from our institution and 84 other reported CE cases were included in the present study. We retrospectively reviewed their clinical characteristics, imaging findings, treatment methods, pathological features, molecular status, and clinical outcomes. The median age at diagnosis of our 8 patients was 7.5 years. The mean tumor diameter was 70 mm. All the tumors had a cystic appearance, and calcification was observed in 6. Gross total resection was achieved in 6 patients and subtotal resection in 2 patients. Of the 8 tumors, 7 were World Health Organization grade III and 1 was World Health Organization grade II. Six tumors were immunopositive for L1 cell adhesion molecule (L1CAM). We investigated the presence of C11orf95–RELA fusion in 5 patients, all of whom exhibited it. Postoperative radiotherapy was performed for all patients with grade III tumors, except for children aged
ISSN:1878-8750
1878-8769
DOI:10.1016/j.wneu.2019.05.166