BCOR Positive Central Nervous System Neuroepithelial Tumor Masquerading as a Meningioma

Indian J Surg Oncol. 2024 Jun;15(2):359-363. doi: 10.1007/s13193-024-01894-1. Epub 2024 Feb 8.


The central nervous system tumor with BCL-6 interacting corepressor internal tandem duplication (BCOR-ITD) is a molecularly defined entity with an internal tandem duplication in exon 15 of the BCOR gene. It is histologically characterized by a solid pattern with spindle-shaped cells and a dense capillary network. Very few cases of this rare entity have been reported till date. We present a case of BCOR-positive CNS tumor in a 3-year-old child who presented with scalp swelling. A differential diagnosis of CNS tumor with BCOR expression should be considered whenever high-grade tumors with histopathological features of glial or ependymal tumors do not express the classical glial markers.

Keywords: BCOR-ITD; Central nervous system tumor; Embryonal; Meningioma; Neuroepithelial.

Publication types

  • Case Reports