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

Neuropathology - Tập 32 Số 5 - Trang 479-491 - 2012
Hajime Miyata1, Masae Ryufuku1, Yuichi Kubota2, Taku Ochiai2, Kaku Niimura3, Tomokatsu Hori3,2
1Department of Neuropathology, Research Institute for Brain and Blood Vessels – Akita, Akita
2Department of Neurosurgery, Tokyo Women's Medical University
3Department of Neurosurgery, Moriyama Memorial Hospital, Tokyo, Japan

Tóm tắt

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.

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Neuropathology

Tập 32 Số 5

479-491

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