Original ArticlesAngiocentric Glioma Report of Clinico-Pathologic and Genetic Findings in 8 CasesPreusser, Matthias MD* †; Hoischen, Alexander MSc‡; Novak, Klaus MD§; Czech, Thomas MD§; Prayer, Daniela MD∥; Hainfellner, Johannes A. MD*; Baumgartner, Christoph MD, Dipl-Ing¶; Woermann, Friedrich G. MD♯; Tuxhorn, Ingrid E. MD♯; Pannek, Heinz W. MD♯; Bergmann, Markus MD**; Radlwimmer, Bernhard PhD††; Villagrán, Rafael MD‡‡; Weber, Ruthild G. MD‡; Hans, Volkmar H. MD‡‡Author Information *Institute of Neurology †Departments of †Internal Medicine I §Neurosurgery ∥Neuroradiology ¶Neurology, Medical University of Vienna, Vienna, Austria ‡Department of Human Genetics, Rheinische Friedrich-Wilhelms University, Bonn ♯Epilepsy Center Bethel ‡‡Institute for Neuropathology, Evangelisches Krankenhaus Bielefeld, Bielefeld **Institute of Clinical Neuropathology, Klinikum Bremen-Mitte, Bremen ††Division of Molecular Genetics, German Cancer Research Center, Heidelberg, Germany Supported by the Deutsche Krebshilfe (70-3163-Wi3), the German Ministry for Education and Research (National Network for Genome Research, NGFN-2) and the BONFOR program of the Medical Faculty, Rheinische Friedrich-Wilhelms University, Bonn (O-149.0058). Mathias Preusser and Alexander Hoischen contributed equally. Reprints: Volkmar H. Hans, MD, Institut für Neuropathologie, Evangelisches Krankenhaus Bielefeld gGmbH, Remterweg 2, D-33617 Bielefeld, Germany (e-mail: [email protected]). The American Journal of Surgical Pathology: November 2007 - Volume 31 - Issue 11 - p 1709-1718 doi: 10.1097/PAS.0b013e31804a7ebb Buy Metrics Abstract 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. © 2007 Lippincott Williams & Wilkins, Inc.