Adult-onset angiocentric glioma of epithelioid cell-predominant type of the mesial temporal lobe suggestive of a rare but distinct clinicopathological subset within a spectrum of angiocentric cortical ependymal tumors

Abstract

Angiocentric glioma (AG) is defined as an epilepsy-associated stable or slowly growing cerebral tumor primarily affecting children and young adults, histologically consisting mainly of monomorphic, bipolar spindle-shaped cells and occasional round to monopolar columnar epithelioid cells, showing angiocentric growth pattern and features of ependymal differentiation. We describe two clinicopathologically unusual cases of AG. Case 1 is a 54-year-old woman with a 10-year history of complex partial seizures. MRI revealed non-enhancing T1-low, T2/fluid-attenuated inversion recovery (FLAIR)-high intensity signal change in the left hippocampus and amygdala. After selective amygdalohippocampectomy, she had rare non-disabling seizures on medication for over 50 months (Engel's class I). Case 2 is a 37-year-old man with a 3-year history of complex partial seizures. MRI revealed non-enhancing T1-low, T2/FLAIR-high intensity signal change in the left uncus and amygdala. After combined amygdalohippocampectomy and anterior temporal lobectomy, he has been seizure-free for over 11 months. Histologically the tumors in both cases consisted mainly of infiltrating epithelioid cells (GFAP- ∼±, S-100-) with perinuclear epithelial membrane antigen (EMA)-positive dots and rings, showing conspicuous single- and multi-layered angiocentric arrangements. Occasional tumor cells showed spindle-shaped morphology (GFAP+, S-100+) with rare EMA-positive dots aligned radially and longitudinally along parenchymal blood vessels. Focal solid areas showed a Schwannoma-like fascicular arrangement with rare EMA-positive dots and/or sheets of epithelioid cells with abundant EMA dots. Electron microscopic investigation demonstrated features of ependymal differentiation. These cases, together with a few similar cases previously reported, appear to represent a rare but distinct clinicopathological subset of AG characterized by adult-onset, mesial temporal lobe localization and epithelioid cell-predominant histology.