• World Neurosurg · Sep 2019

    Clinicopathological and genetic features of supratentorial cortical ependymomas.

    • Yuji Matsumoto, Tomotsugu Ichikawa, Kazuhiko Kurozumi, Yoshihiro Otani, and Isao Date.
    • Department of Neurological Surgery, Okayama University Graduate School of Medicine, Okayama, Japan.
    • World Neurosurg. 2019 Sep 1; 129: e417-e428.

    ObjectiveSupratentorial 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.MethodsEight 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.ResultsThe 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 <3 years. Although 4 patients developed recurrence, all were alive throughout the follow-up period. Compared with previously reported CEs, our patients were younger and had larger tumors; however, the clinical outcomes did not differ significantly.ConclusionsAlthough most CEs in our group were immunopositive for L1CAM and showed C11orf95-RELA fusion, which have been associated with a poor prognosis in supratentorial ependymomas, all our patients had good outcomes. Gross total resection and adjuvant radiotherapy contributed to the relatively favorable prognosis of CEs compared with other supratentorial ependymomas.Copyright © 2019 Elsevier Inc. All rights reserved.

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