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.

Keywords: Anaplastic ependymomas; extraventricular; supratentorial; surgery; treatment.

Figure 1

(a-d) Case 1: (a) Contrast-enhanced computed tomographic scan demonstrating a cortical lesion of the left frontal lobe, with surrounding edema with mass effect, homogeneously enhanced. (b) Coronal T2-weighted magnetic resonance imaging demonstrating a hypointense with the gray matter cortical lesion. (c) Coronal T1-weighted magnetic resonance imaging with gadolinium enhancement demonstrating left frontal not-well defined strongly enhancing mass with mass effect and leptomeningeal spread through Sylvian fissure. (d) Postoperative contrast enhanced computed tomographic scan demonstrating gross total excision

Figure 2

Case 1: Histopathological findings. Hematoxylin and eosin-stained section showing perivascular pseudorosettes (a nuclear zones formed by radially arranged tumor cell processes surrounding central blood vessels)

Figure 3

(a-d) Case 2: (a) Axial contrast-enhanced T1-weighted magnetic resonance imaging demonstrating a heterogeneous cystic enhancing mass located intra-axially and in the extraventricular space in the left temporo-insular region. (b) Axial T2-weigheted magnetic resonance imaging demonstrating previous intratumoral hemorrhage. (c and d) Postoperative axial computed tomographic scan (c) and (d) contrast-enhanced computed tomographic scan demonstrating gross total resection

Figure 4

(a-d) Case 2: Histopathological findings. (a) Hematoxylin and eosin-stained section showing increased cellularity with perivascular pseudorosettes. (b-d) Immunohistochemical stainings for glial fibrillary acidic protein, epithelial membrane antigen, and Ki-67 labeling index revealing approximately 10%