Preusser Matthias, Hoischen Alexander, Novak Klaus, Czech Thomas, Prayer Daniela, Hainfellner Johannes A, Baumgartner Christoph, Woermann Friedrich G, Tuxhorn Ingrid E, Pannek Heinz W, Bergmann Markus, Radlwimmer Bernhard, Villagrán Rafael, Weber Ruthild G, Hans Volkmar H
Institute of Neurology, Medical University of Vienna, Vienna, Austria.
Am J Surg Pathol. 2007 Nov;31(11):1709-18. doi: 10.1097/PAS.0b013e31804a7ebb.
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.
血管中心性胶质瘤最近被描述为一种具有独特临床病理特征的新型癫痫相关肿瘤。我们报告了另外8例这种罕见肿瘤类型的临床和病理结果，并通过基因组分析扩展了对其特征的认识。几乎所有患者都有长期耐药性癫痫病史。皮质 - 皮质下肿瘤位于颞叶和顶叶。癫痫发作始于3至14岁，手术在6至70岁进行。组织学上，肿瘤的特征为弥漫性生长和显著的血管周围肿瘤细胞排列，具有星形胶质细胞/室管膜分化特征，但缺乏肿瘤性神经元特征。未观察到坏死和血管增生，有丝分裂稀少或无。MIB - 1增殖指数范围为<1%至5%。免疫组化方面，所有病例胶质纤维酸性蛋白、波形蛋白、蛋白S100B均呈阳性染色，足板蛋白染色情况不一，并显示上皮膜抗原阳性的胞质小点。3例中的2例电镜检查显示有室管膜特征。通过染色体比较基因组杂交分析基因组不平衡发现，8例中的1例仅存在6q24至q25染色体带缺失这一改变。3例中的1例通过阵列比较基因组杂交进行高分辨率筛查，确定来自11p11.2染色体带的2个相邻克隆存在拷贝数增加，该染色体带包含蛋白酪氨酸磷酸酶受体J型（PTPRJ）基因。所有患者在随访时间从半年至6.9年期间均无癫痫发作且无肿瘤复发迹象。我们的研究结果支持之前的两份报告，即血管中心性胶质瘤是一种低级别恶性的新型胶质肿瘤实体。
