Surgical Experience of a Rare Ventricular Tumor: A Case Report

In this case report, we present a rare case of a suspicious central nervous system (CNS) high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR). A 29-year-old woman ini-tially presented with an incidental ventricular tumor detected on computed tomography. The brain magnetic resonance imaging revealed a 6 × 5 cm heterogeneously enhancing solid cystic mass with coarse calcifications in the right lateral ventricle. Preoperative radiological diagnosis suggested a cen-tral neurocytoma, and subsequent surgery was done in gross total resection with satisfactory recov-ery. However, after three years, tumor recurrence was observed, leading to revision surgery. Based on the histopathological evaluation according to the 2021 World Health Organization classification, the tumor was suggested as CNS HGNET-BCOR. Adjuvant radiotherapy was planned as part of the treatment strategy. This case report contributes to the limited knowledge about intraventricular CNS HGNET-BCOR tumors, offering insights into diagnosis and treatment approaches for this rare en-tity.