Final Diagnosis -- Angiocentric Glioma, WHO Grade I


Angiocentric glioma, WHO grade I


Angiocentric glioma (AG) was described by two different groups and with two different names as a distinctive epileptogenic neoplasm. Due to the features of an infiltrating astrocytoma and ependymoma at histopathological, immunohistochemical and ultrastructural level, Wan et al proposed the term monomorphous angiocentric glioma (1). Lellouch-Tubian et al reported the presence of a neuronal cell component in all cases of their series and thus classified this tumor type as a mixed glioneuronal neoplasm and used the term angiocentric neuroepithelial tumor (2). Accordingly, this tumor was recognized as a distinct entity in the 2007 WHO classification and named as angiocentric glioma within the category of 'Other Neuroepithelial Tumours' (3). Due to its benign clinical behavior and the possibility of curative surgery, the neoplasm was assigned to WHO grade I (3). Among the cases of angiocentric glioma, seizure was the most common symptom at presentation (1-6). Evidence of adjacent malformation of cortical development/focal cortical dysplasia has been reported in a subset of angiocentric gliomas, suggesting a developmental basis to their origin (5,7). Magnetic resonance imaging of AGs demonstrated supratentorial, non-enhancing, T1-hypointense, T2-hyperintense lesions (1-6). Gross-total resection of this lesion yields excellent results (1-6).

The molecular genetic properties of angiocentric gliomas have not been fully studied. One tumor showed loss at 6q24-q25 by classic CGH, while another showed a gain at 11p11.2 by high resolution array CGH (6). In a recent study of three cases, all tumors showed wild genotype for the IDH1, IDH2 and BRAF V600E genes (8).

Several types of neuroepithelial tumors are known as the cause of intractable epilepsy (7,9). They are generally slow growing, low-grade, cortically based tumors, with indolent course and in many cases they exhibit neuronal in addition to glial differentiation. Gangliogliomas and dysembryoplastic neuroepithelial tumors (DNT) predominate in this group, followed by pleomorphic xanthoastrocytoma, papillary glioneuronal tumor and angiocentric glioma (9).

Histopathological differential diagnosis of angiocentric glioma constantly displaying angiocentric orientation of spindle-shaped cells includes ependymoma, astroblastoma, infiltrating astrocytoma, and dysembryoplastic neuroepithelial tumor.

Though the incidence of angiocentric glioma is not known, most of the reported cases have presented with chronic epilepsy; and despite reports of rare examples with increased mitotic activity (10) or anaplastic features (11), excision alone has most often proved curative. As always, accurate pathological diagnosis is important to prevent overtreatment.


  1. Wang M, Tihan T, Rojiani AM, Bodhireddy SR, Prayson RA, Iacuone JJ, Alles AJ, Donahue DJ, Hessler RB, Kim JH, Haas M, Rosenblum MK, Burger PC (2005) Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma J Neuropathol Exp Neurol 64:875-81.
  2. Lellouch-Tubiana A, Boddaert N, Bourgeois M, Fohlen M, Jouvet A, Delalande O, Seidenwurm D, Brunelle F, Sainte-Rose C (2005) Angiocentric neuroepithelial tumor (ANET): a new epilepsy-related clinicopathological entity with distinctive MRI Brain Pathol 15: 281-286.
  3. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds):WHO Classification of Tumours of the Central Nervous System. IARC:Lyon 2007,
  4. Shakur SF, McGirt MJ, Johnson MW, Burger PC, Ahn E, Carson BS, and Jallo GI (2009) Angiocentric glioma: a case series J Neurosurg Pediatr 3:197-202.
  5. Marburger T, Prayson R (2011) Angiocentric glioma: a clinicopathologic review of 5 tumors with identification of associated cortical dysplasia Arch Pathol Lab Med 135:1037-41.
  6. Preusser M, Hoischen A, Novak K, Czech T, Prayer D, Hainfellner JA, Baumgartner C, Woermann FG, Tuxhorn IE, Pannek HW, Bergmann M, Radlwimmer B, Villagrán R, Weber RG, Hans VH (2007) Angiocentric glioma: report of clinico-pathologic and genetic findings in 8 cases Am J Surg Pathol 31:1709-18.
  7. Prayson RA, Fong J, Najm I (2010) Coexistent pathology in chronic epilepsy patients with neoplasms Modern Pathology 23: 1097-1103.
  8. Buccoliero AM, Castiglione F, Degl'innocenti DR, Moncini D, Spacca B, Giordano F, Genitori L, Taddei GL (2013) Angiocentric glioma: clinical, morphological, immunohistochemical and molecular features in three pediatric cases Clin Neuropathol 32:107-113.
  9. Thom M, Blümcke I, Aronica E (2012) Long-term epilepsy-associated tumors Brain Pathol 22:350-79.
  10. Pokharel S, Parker JR, Parker JC Jr, Coventry S, Stevenson CB, Moeller KK (2011) Angiocentric glioma with high proliferative index: case report and review of the literature Ann Clin Lab Sci 41:257-61.
  11. Aguilar HN, Hung RW, Mehta V, Kotylak T (2012) Imaging characteristics of an unusual, high-grade angiocentric glioma: A case report and review of the literature J Radiol Case Rep 6: 1-10.

Contributed by Figen Soylemezoglu, Cigdem Himmetoglu, Kader K Oguz, Serap Saygi, Nejat Akalan

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