title: 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.
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at Houston, Medical School, Houston, Texas, USA.
authors.forename: Min
authors.initials: M
authors.lastname: Wang
authors.forename: Tarik
authors.initials: T
authors.lastname: Tihan
authors.forename: Amyn M
authors.initials: AM
authors.lastname: Rojiani
authors.forename: Surender R
authors.initials: SR
authors.lastname: Bodhireddy
authors.forename: Richard A
authors.initials: RA
authors.lastname: Prayson
authors.forename: John J
authors.initials: JJ
authors.lastname: Iacuone
authors.forename: Ajit J
authors.initials: AJ
authors.lastname: Alles
authors.forename: David J
authors.initials: DJ
authors.lastname: Donahue
authors.forename: Richard B
authors.initials: RB
authors.lastname: Hessler
authors.forename: Jung H
authors.initials: JH
authors.lastname: Kim
authors.forename: Mark
authors.initials: M
authors.lastname: Haas
authors.forename: Marc K
authors.initials: MK
authors.lastname: Rosenblum
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
mesh_terms: D000293:Adolescent; D000328:Adult; D001254:Astrocytoma / Q000473:pathology*; D001932:Brain Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004806:Ependymoma / Q000473:pathology*; D004827:Epilepsy / Q000209:etiology*; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D005910:Glioma / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D008854:Microscopy, Electron; D018396:Mucin-1 / Q000378:metabolism
chemical_list: D005904:Glial Fibrillary Acidic Protein; D018396:Mucin-1