Monomorphous Angiocentric Glioma: A Distinctive Epileptogenic Neoplasm With Features of Infiltrating Astrocytoma and Ependymoma

Abstract

We present 8 examples of a neoplasm with features of both astrocytoma and ependymoma that may represent a distinct clinicopathologic entity. The cerebral hemispheric tumors occurred in patients that were 3, 4, 12, 14, 15, 26, 30, and 37 years of age. All presented with seizures that, with the exception of 2, began in childhood. Magnetic resonance imaging studies showed ill-defined, T2-hyperintense, generally noncontrast-enhancing lesions that, although centered on the cortex or amygdala, extended into the underlying white matter for a short distance. Histologically, the variably infiltrative tumors were distinctively angiocentric with well-developed perivascular pseudorosettes in some cases. Longitudinal and/or circumferential orientations of perivascular cells were common also. The cells were uniform in their cytologic features from case to case and were bipolar in all but one case. A glial nature was inferred from immunoreactivity for GFAP, and ependymal differentiation was suggested by positivity for EMA in three cases and ultrastructural features in one. Overall, the tumors were biologically indolent except for one that recurred and ultimately proved fatal.

“Unusual brain stone”: heavily calcified primary neoplasm with some features suggestive of angiocentric glioma

“…16 Angiocentric glioma was first described as a separate entity in 2005 and was included in the World Health Organization (WHO) classification of brain tumors in 2007. 8,19 Given the recency of the description, information on long-term follow-up of individual cases and the long-term course of these lesions is limited. Although the tumor is regarded as low grade (WHO Grade I) with no recurrence, a recurrent case with glioblastoma-like features has recently been reported.…”

“…8,10 Angiocentric glioma is a relatively recently recognized low-grade usually supratentorial tumor of children or young adults associated with drug-resistant seizures and with a good prognosis. 8,13,19 Its cell of origin is uncertain, but it may derive from astrocytic, ependymal, radial glial, or neuronal elements. 6 It is not usually associated with dense calcification.…”

“…6 Wang et al proposed the term monomorphous angiocentric glioma for lesions lacking a neuronal component. 19 Isocitrate dehydrogenase mutation (IDH1 R132H), is absent in diffuse angiocentric glioma. 14 Minor calcification has previously been described as a feature of angiocentric glioma, 1 but the presence of such dense calcification resembling a stone in this case may be a consequence of the substantial age of the tumor (at least 16 years).…”

Central Nerve System Malignant Tumors

“…This type, first described as epilepsy in patients usually with the long term. monomorphous angiocentric glioma reveal EVA positive intra cytoplasmic dots, it is differ from ependymomas because of the accumulating of tumor cells in the subpial area, and also they are highly infiltrative aspect [34].…”

Angiocentric Glioma and Surrounding Cortical Dysplasia Manifesting as Intractable Frontal Lobe Epilepsy-Case Report-

“…Angiocentric glioma was first reported in 2005, 4,11) and was recently classified as a new entity in the 2007 World Health Organization Classification of Tumors of the Central Nervous System. Since then, a total of 36 cases have been reported.…”