World Health Organization Grade III Supratentorial Extraventricular Ependymomas in Adults: Case Series and Review of Treatment Modalities

Abstract

Context: Supratentorial ependymomas and their anaplastic variants are relatively uncommon central nervous system neoplasms that afflict both adults and children. Aims: Discuss the clinical and pathological features of patients with anaplastic ependymomas involving an extraventricular supratentorial location and review modalities and options of treatment for those rare tumors. Settings and Design: Whereas the treatment algorithm in the pediatric population is well established, however, treatment in the adult population is less defined. Treatment options are exposed through the author s cases and review of the literature. Subjects and Methods: In our case series of two adult patients with supratentorial ependymomas World Health Organization (WHO) Grade III (anaplastic variant), patients presented in both cases in the emergency room after having a generalized tonic–clonic seizure at home the first case, and mild hemiparesis the second case. Results: Patients underwent surgical treatment, and a gross total resection was achieved in both cases. The histopathological examination revealed a diagnosis of anaplastic ependymoma (WHO Grade III). Both patients had additional radiotherapy, and in the first case, adjuvant platinum-based chemotherapy was administered due to leptomeningeal gliomatosis. Conclusion: In our experience, gross total resection was achieved in all patients with supratentorial extraventricular ependymomas WHO Grade III with additional radiotherapy and platinum-based chemotherapy. Patients require initial close serial imaging follow-up. The role of chemotherapy is still uncertain but may be necessary in younger patients and in tumors that behave more like the pediatric ependymomas.