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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 6  |  Page : 677-679

Angiocentric Glioma: A treatable cause of epilepsy: Report of a rare case

1 Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
2 Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
3 Department of Neuroradiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India

Date of Submission04-Nov-2014
Date of Decision09-Nov-2014
Date of Acceptance20-Dec-2014
Date of Web Publication16-Jan-2015

Correspondence Address:
Mehar Chand Sharma
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.149405

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How to cite this article:
Kakkar A, Sharma MC, Suri V, Kaushal S, Chandra SP, Garg A, Sarkar C. Angiocentric Glioma: A treatable cause of epilepsy: Report of a rare case. Neurol India 2014;62:677-9

How to cite this URL:
Kakkar A, Sharma MC, Suri V, Kaushal S, Chandra SP, Garg A, Sarkar C. Angiocentric Glioma: A treatable cause of epilepsy: Report of a rare case. Neurol India [serial online] 2014 [cited 2022 Sep 27];62:677-9. Available from: https://www.neurologyindia.com/text.asp?2014/62/6/677/149405


Angiocentric glioma (AG) is a rare tumor of the central nervous system (CNS) and a little more than 50 cases have been reported. [1],[2],[3] Based on its indolent behavior, lack of mitotic activity, and low proliferation indices, it was classified as World Health Organization (WHO) grade I. [4] However, few cases showing atypical features have been described. The exact biology of this tumor remains cryptic.

An 18-year-old male presented with complex partial seizures since the age of 4 years. Seizure frequency was 5-6/day and failed for multiple antiepileptic drugs. Electroencephalography (EEG) and interictal single-photon emission computed tomography (SPECT) localized the lesion to the left frontal region. Magnetic resonance imaging showed thickening of left inferior frontal gyrus, isointense on T1WI, and hyperintense on T2WI [Figure 1]. The patient had right frontal craniotomy and electrocorticography (ECOG)-guided resection of the lesion. ECOG showed grade IV ECOG changes over the lesion and up to 3 cm in front of it. Lesional area and abnormal area on ECOG were resected. Microscopically, the tumor comprised plump to bipolar cells with eosinophilic cytoplasm, at places showing clearing [Figure 2]a-c. Nuclei were monomorphic, rounded to oval, with fine chromatin and indistinct nucleoli. Mitotic figures and nuclear pleomorphism were absent. At places, tumor cells were arranged radially around blood vessels [Figure 2]a. In other areas, they were present perpendicularly along vessel walls, giving a streaming appearance [Figure 2]c. Where the tumor was infiltrating the cortex, cells were arranged in a palisading fashion parallel to the pial surface [Figure 2]d. Occasional small nests of tumor cells were seen infiltrating between blood vessels and there was subpial spread. Ependymal canals and Rosenthal fibers were absent. On immunohistochemistry [Figure 3], tumor cells showed perinuclear dot-like positivity for epithelial membrane antigen (EMA), variable immunopositivity for glial fibrillary acidic protein (GFAP), vimentin, and S-100 protein, while isocitrate dehydrogenase type 1 (IDH1), synaptophysin, neuron specific enolase (NSE), cytokeratin, and p53 were negative. MIB-1 labelling index (MIB-1 LI) was low (<1%). Post-surgery, patient was seizure-free and taking one drug for 1 year and it was stopped thereafter.
Figure 1: Radiological features: Axial T1 - (a) and T2 - weighted images (b) show a well - defined cortical lesion in right frontal lobe with scalloping of overlying inner table of the skull. The lesion is hypointense on T1 - WI (a) and hyperintense in T2 - WI (b) with a small Cerebrospinal fluid (CSF) - isointense cystic portion in anterior aspect of the lesion. Pre - gadolinium sagittal T1 - weighted image (c) shows a cortical lesion in right frontal lobe (arrows). The lesion does not show any enhancement following gadolinium administration (d)

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Figure 2: Histological features: Photomicrographs showing perivascular arrangement of oval to spindle cells with eosinophilic cytoplasm (a), at places showing clearing of the cytoplasm (b), sweeping arrangement of the spindled cells (c), and parallel arrangement of the cells in the subpial region (d) (HE, ×400)

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Figure 3: Immunohistochemical profile: Immunohistochemistry showing the tumor cells to be immunopositive for glial fibrillary acidic protein (GFAP) (a) and vimentin (b), with dot like positivity for epithelial membrane antigen (EMA) (c). MIB - 1 LI is very low (d) (×400)

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AG is a novel clinicopathological entity, originally described in association with medically intractable seizures. [1],[2] It is a tumor of children and young adults, accounting for 2.3% of tumors associated with chronic pediatric epilepsy. [5],[6] More than 90% of patients present with medically intractable seizures, which resolve after excision of the tumor. [6] AGs are most frequently located in the superficial frontal and temporal lobes cortex. [5] Radiological features include a solid, well-demarcated cerebrocortical tumor with subcortical extension, hyperintense on T2WI, and lacking contrast enhancement. [4] A cortical rim-like hyperintensity on T1WI and a stalk extending to the ventricular surface on T2WI are characteristic. [1],[2] However, neither of these was seen in our case, precluding preoperative diagnosis. Apart from typical microscopic features as seen in our case, other patterns like fascicular arrangement resembling Antoni-A areas of schwannoma, solid nests of epithelioid-appearing cells, and oligodendroglial-like cells have been described. [2] Cytologic atypia is minimal. Mitoses are infrequent; necrosis and endothelial proliferation are not seen. Recent reports have described occurrence of AG in association with cortical dysplasia (CD). [2],[3],[5],[7] Liu et al., proposed that excision of adjacent dysplastic cortex along with the tumor may be necessary to achieve seizure-free outcome after surgery. [3] Intraoperative ECOG may thus be of value in determining the extent of the epileptogenic area that needs to be resected. Only occasional genetic alterations have been described in AG. IDH1 R132H mutation does not occur in AG; thus, absence of mutant IDH1 protein expression in AG is useful in distinguishing it from diffuse gliomas in limited biopsy samples. [8]

Histogenesis of AG, like that of many newly described neoplasms, remains controversial. Angiocentric growth and dot-like EMA immunopositivity in intracytoplasmic microluminal pattern suggest ependymal differentiation. [2],[4] Ultrastructurally also, AGs have features similar to ependymomas, with intercellular junctions and microlumens filled with microvilli. [2] However, cortical location of AG is contrary to this hypothesis. An origin from neoplastic transformation of radial glial cells during neuronal migration has now been proposed, based on elongated bipolar morphology of tumor cells. [1]

Outcome of AG patients is excellent, with most achieving freedom from seizures following gross total resection, but subtotal resection does not have such good results, with reported seizure-free outcomes of 93% vs 36%. [3],[5] Although benign in nature, one fatality has been attributed to AG, due to progressive disease. [2] The primary tumor showed typical histological features; however, the recurrent tumor displayed increased mitoses and elevated MIB-1 LI (10%), indicating aggressive behavior.

  References Top

Lellouch-Tubiana A, Boddaert N, Bourgeois M, Fohlen M, Jouvet A, Delalande O, et al. Angiocentric neuroepithelial tumor (ANET): A new epilepsy-related clinicopathological entity with distinctive MRI. Brain Pathol 2005;15:281-6.  Back to cited text no. 1
Wang M, Tihan T, Rojiani AM, Bodhireddy SR, Prayson RA, Iacuone JJ, et al. Monomorphous angiocentric glioma: A distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol 2005;64:875-81.  Back to cited text no. 2
Liu CQ, Zhou J, Qi X, Luan GM. Refractory temporal lobe epilepsy caused by angiocentric glioma complicated with focal cortical dysplasia: A surgical case series. J Neurooncol 2012;110:375-80.  Back to cited text no. 3
Burger PC, Jouvet A, Preusser M, Hans VH, Rosenblum MK, Lellouch-Tubiana A. Angiocentric glioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization Classification of Tumours of the Central Nervous System. 4 th ed. Lyon: IARC; 2007. p. 92-3.  Back to cited text no. 4
Takada S, Iwasaki M, Suzuki H, Nakasato N, Kumabe T, Tominaga T. Angiocentric glioma and surrounding cortical dysplasia manifesting as intractable frontal lobe epilepsy--Case report. Neurol Med Chir (Tokyo) 2011;51:522-6.  Back to cited text no. 5
Prayson RA. Tumours arising in the setting of paediatric chronic epilepsy. Pathology 2010;42:426-31.  Back to cited text no. 6
Marburger T, Prayson R. Angiocentric glioma: A clinicopathologic review of 5 tumors with identification of associated cortical dysplasia. Arch Pathol Lab Med 2011;135:1037-41.  Back to cited text no. 7
Raghunathan A, Olar A, Vogel H, Parker JR, Coventry SC, Debski R, et al. Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. Ann Diagn Pathol 2012;16:255-9.  Back to cited text no. 8


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