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 except...

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Veröffentlicht in:Journal of neuropathology and experimental neurology 2005-10, Vol.64 (10), p.875-881
Hauptverfasser: Wang, Min, Tihan, Tarik, Rojiani, Amyn M, Bodhireddy, Surender R, Prayson, Richard A, Iacuone, John J, Alles, Ajit J, Donahue, David J, Hessler, Richard B, Kim, Jung H, Haas, Mark, Rosenblum, Marc K, Burger, Peter C
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container_title Journal of neuropathology and experimental neurology
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creator Wang, Min
Tihan, Tarik
Rojiani, Amyn M
Bodhireddy, Surender R
Prayson, Richard A
Iacuone, John J
Alles, Ajit J
Donahue, David J
Hessler, Richard B
Kim, Jung H
Haas, Mark
Rosenblum, Marc K
Burger, Peter C
description 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.
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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. 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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.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Astrocytoma - pathology</subject><subject>Biological and medical sciences</subject><subject>Brain Neoplasms - complications</subject><subject>Brain Neoplasms - diagnosis</subject><subject>Brain Neoplasms - metabolism</subject><subject>Brain Neoplasms - pathology</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Ependymoma - pathology</subject><subject>Epilepsy - etiology</subject><subject>Female</subject><subject>Female genital diseases</subject><subject>Glial Fibrillary Acidic Protein - metabolism</subject><subject>Glioma - complications</subject><subject>Glioma - diagnosis</subject><subject>Glioma - metabolism</subject><subject>Glioma - pathology</subject><subject>Gynecology. Andrology. 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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.</abstract><cop>Hagerstown, MD</cop><pub>American Association of Neuropathologists, Inc</pub><pmid>16215459</pmid><doi>10.1097/01.jnen.0000182981.02355.10</doi><tpages>7</tpages></addata></record>
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subjects Adolescent
Adult
Astrocytoma - pathology
Biological and medical sciences
Brain Neoplasms - complications
Brain Neoplasms - diagnosis
Brain Neoplasms - metabolism
Brain Neoplasms - pathology
Child
Child, Preschool
Ependymoma - pathology
Epilepsy - etiology
Female
Female genital diseases
Glial Fibrillary Acidic Protein - metabolism
Glioma - complications
Glioma - diagnosis
Glioma - metabolism
Glioma - pathology
Gynecology. Andrology. Obstetrics
Humans
Immunohistochemistry
Magnetic Resonance Imaging
Male
Medical sciences
Microscopy, Electron
Mucin-1 - metabolism
Neurology
Tumors
Tumors of the nervous system. Phacomatoses
title Monomorphous Angiocentric Glioma: A Distinctive Epileptogenic Neoplasm With Features of Infiltrating Astrocytoma and Ependymoma
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