Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma.
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.
Wang
Min
M
Department of Pathology and Laboratory Medicine, University of Texas Health Science Center at Houston, Medical School, Houston, Texas, USA.
Tihan
Tarik
T
Rojiani
Amyn M
AM
Bodhireddy
Surender R
SR
Prayson
Richard A
RA
Iacuone
John J
JJ
Alles
Ajit J
AJ
Donahue
David J
DJ
Hessler
Richard B
RB
Kim
Jung H
JH
Haas
Mark
M
Rosenblum
Marc K
MK
Burger
Peter C
PC
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
D005904:Glial Fibrillary Acidic Protein; D018396:Mucin-1