Supratentorial Extraventricular Ependymoma: Retrospective Analysis of 15 Patients at a Single Institution

Background: Supratentorial extraventricular ependymoma (STEE) is an extremely rare central nervous system (CNS) neoplasm, the clinical characteristics and optimal treatment of which remain unclear. We retrospectively analyzed the clinical characteristics and treatment outcomes of 15 patients with STEE.

Methods: Fifteen patients with STEE were identified, and their clinical, radiologic, and surgical records were reviewed.

Results: The 15 patients included 6 males (40%) and 9 females (60%), with a median age of 15 years. Nine patients (60%) underwent gross total resection, 5 patients (33.3%) underwent subtotal resection, and 1 patient (6.7%) underwent biopsy. Eight patients received adjuvant radiotherapy, and 3 received adjuvant chemotherapy. The 5-year overall survival (OS) rates of patients with World Health Organization (WHO) grade II and grade III STEE were 100% and 30%, respectively, and the corresponding 2-year progression-free survival (PFS) rates were 77.8% and 20%. The differences in OS and PFS rates between WHO grades II and III STEE were statistically significant (P < 0.01 and 0.02, respectively). Anaplastic histology (WHO grade III) was a poor prognostic factor for PFS (P = 0.04). The recurrence pattern was local recurrence in all patients; all cases were treated primarily by redo surgery. Three patients with recurrent WHO grade II STEE were alive at more than 64, 52, and 44 months after redo surgery.

Conclusions: STEE is an extremely rare CNS neoplasm. The histological grade of STEE is an important prognostic factor. Microsurgical resection might play a major role in both initial treatment and treatment of recurrent tumors.