Angiocentric glioma: report of clinico-pathologic and genetic findings in 8 cases.
Angiocentric glioma has recently been described as a novel epilepsy associated tumor with distinct clinico-pathologic features. We report the clinical and pathologic findings in 8 additional cases of this rare tumor type and extend its characterization by genomic profiling. Almost all patients had a history of long-standing drug-resistant epilepsy. Cortico-subcortical tumors were located in the temporal and parietal lobes. Seizures began at 3 to 14 years of age and surgery was performed at 6 to 70 years. Histologically, the tumors were characterized by diffuse growth and prominent perivascular tumor cell arrangements with features of astrocytic/ependymal differentiation, but lacking neoplastic neuronal features. Necrosis and vascular proliferation were not observed and mitoses were sparse or absent. MIB-1 proliferation indices ranged from <1% to 5%. Immunohistochemically, all cases stained positively for glial fibrillary acidic protein, vimentin, protein S100B, variably for podoplanin, and showed epithelial membrane antigen-positive cytoplasmic dots. Electron microscopy showed ependymal characteristics in 2 of 3 cases investigated. An analysis of genomic imbalances by chromosomal comparative genomic hybridization revealed loss of chromosomal bands 6q24 to q25 as the only alteration in 1 of 8 cases. In 1 of 3 cases, a high-resolution screen by array-comparative genomic hybridization identified a copy number gain of 2 adjacent clones from chromosomal band 11p11.2 containing the protein-tyrosine phosphatase receptor type J (PTPRJ) gene. All patients are seizure free and without evidence of tumor recurrence at follow-up times ranging from 1/2 to 6.9 years. Our findings support 2 previous reports proposing that angiocentric glioma is a novel glial tumor entity of low-grade malignancy.
Institute of Neurology, Medical University of Vienna, Vienna, Austria.
Matthias
M
Preusser
Alexander
A
Hoischen
Klaus
K
Novak
Thomas
T
Czech
Daniela
D
Prayer
Johannes A
JA
Hainfellner
Christoph
C
Baumgartner
Friedrich G
FG
Woermann
Ingrid E
IE
Tuxhorn
Heinz W
HW
Pannek
Markus
M
Bergmann
Bernhard
B
Radlwimmer
Rafael
R
Villagrán
Ruthild G
RG
Weber
Volkmar H
VH
Hans
D000293:Adolescent; D000328:Adult; D000368:Aged; D001253:Astrocytes / Q000473:pathology; D001932:Brain Neoplasms / Q000737:chemistry / Q000150:complications / Q000235:genetics* / Q000601:surgery / Q000648:ultrastructure*; D002454:Cell Differentiation; D049109:Cell Proliferation; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002880:Chromosomes, Human, Pair 11; D002896:Chromosomes, Human, Pair 6; D004805:Ependyma / Q000473:pathology; D004827:Epilepsy / Q000235:genetics* / Q000473:pathology / Q000517:prevention & control; D005060:Europe; D005260:Female; D005500:Follow-Up Studies; D018628:Gene Dosage; D020869:Gene Expression Profiling / Q000379:methods; D015972:Gene Expression Regulation, Neoplastic; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D005910:Glioma / Q000737:chemistry / Q000150:complications / Q000235:genetics* / Q000601:surgery / Q000648:ultrastructure*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D008875:Middle Aged; D018396:Mucin-1 / Q000032:analysis; D009414:Nerve Growth Factors / Q000032:analysis; D009693:Nucleic Acid Hybridization; D020411:Oligonucleotide Array Sequence Analysis; D054631:Receptor-Like Protein Tyrosine Phosphatases, Class 3 / Q000235:genetics; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins / Q000032:analysis; D013997:Time Factors; D016896:Treatment Outcome; D014746:Vimentin / Q000032:analysis; D000098853:Podoplanin
D005904:Glial Fibrillary Acidic Protein; D008562:Membrane Glycoproteins; D018396:Mucin-1; D009414:Nerve Growth Factors; D054631:Receptor-Like Protein Tyrosine Phosphatases, Class 3; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins; D014746:Vimentin; C117468:PDPN protein, human; D000098853:Podoplanin; C579160:S100B protein, human; C486288:PTPRJ protein, human