Gupta Shruti, Rangari Kamlesh Vasant, Mehrotra Anant, Pal Lily, Jaisawal Awadhesh Kumar, Kumar Raj
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India.
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India.
Childs Nerv Syst. 2020 Mar;36(3):641-646. doi: 10.1007/s00381-019-04445-0. Epub 2020 Jan 2.
Angiocentric glioma (AG) is a relatively uncommon clinico-pathological entity that presents in childhood. Angiocentric glioma displays various histopathological features which resemble cortical ependymoma, astroblastoma, and pilomyxoid astrocytoma and schwannoma. The astrocytes in angiocentric glioma appear peculiarly elongated, bipolar in shape, and characteristically arranged around blood vessels. They resemble radial glia and tanycytes morphologically. Unlike ependymomas, AG is a diffusely infiltrating lesion and perivascular processes are often much thicker than those in classic ependymomas.
AG usually present clinically as seizures, often as medically intractable epilepsy. In the indexed case, apart from unusual presentation with features of raised intra-cranial tension, an unusual histological picture of a more cellular oligodendroglioma like component was also seen.
The appropriate diagnosis is critical as AG is usually slowly growing and treatable by surgical excision alone.
血管中心性胶质瘤（AG）是一种相对不常见的临床病理实体，多发生于儿童期。血管中心性胶质瘤呈现出多种组织病理学特征，类似于皮质室管膜瘤、成星形细胞瘤、毛黏液样星形细胞瘤和神经鞘瘤。血管中心性胶质瘤中的星形胶质细胞显得特别细长，呈双极形，且典型地围绕血管排列。它们在形态上类似于放射状胶质细胞和伸展细胞。与室管膜瘤不同，AG是一种弥漫性浸润性病变，血管周围的突起通常比经典室管膜瘤中的要厚得多。
AG临床上通常表现为癫痫发作，常为药物难治性癫痫。在该索引病例中，除了出现颅内压升高的异常表现外，还可见到一种不寻常的组织学图像，即有一个细胞较多的少突胶质细胞瘤样成分。
正确的诊断至关重要，因为AG通常生长缓慢，仅通过手术切除即可治愈。
