Case 4 -

Angiocentric Glioma, WHO Grade I Arie Perry Washington University School of Medicine St. Louis, MO

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Clinical History The patient is a 24-year woman who presented with a history of partial seizures since the age of 13. EEG mapped her seizure focus to the left medial temporo-occipital lobe and MR images showed a vaguely enhancing, T2/FLAIR hyperintense, gyriform lesion in the same region. The lesion was slightly enlarged compared to studies performed 8 years and 1 year previously. Therefore, a surgical resection was performed. There is no evidence of recurrence after one year of clinical followup. Case 4 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 4 - Figure 1 T2-weighted MRI showing a bright gyriform lesion involving the cortex of the left medial temporo-occipital lobe. Case 4 - Figure 2 Moderately cellular intracortical tumor with nuclear palisading in subpial and perivascular regions. Case 4 - Figure 3 The tumor cells have oval to elongate nuclei with delicate fibrillary processes, reminiscent of astrocytoma. Case 4 - Figure 4 Perivascular nuclear free zone resembling the pseudorosettes of ependymoma. Case 4 - Figure 5 Subpial palisades with nuclei arranged both perpendicular and parallel to the pial surface. Case 4 - Figure 6 Perivascular and subpial aggregates of tumor cells. Case 4 - Figure 7 GFAP stain highlighting thin tumoral processes, some of which radiate towards blood vessels. Case 4 - Figure 8 The immunostain for neurofilament protein highlighted numerous entrapped axons, consistent with an infiltrative growth pattern. The angiocentric tumor pattern is also highlighted in relief. Case 4 - Figure 9 A subset of tumor cells displayed dot-like cytoplasm immunoreactivity for epithelial membrane antigen (EMA). Case 4 - Figure 10 Electron microscopy revealed spindled tumor cells with electron dense cytoplasm rich in intermediate filaments and numerous microvilli. Differential Diagnosis Angiocentric glioma, WHO grade I Diffuse astrocytoma, WHO grade II Tanycytic ependymoma, WHO grade II Final Diagnosis Angiocentric glioma, WHO grade I Key words: brain tumor, angiocentric glioma, ependymoma, WHO References Burger PC, Jouvet A, Preusser M et al. Angiocentric glioma. In: Louis DN, Ohgaki H, Wiestler OD and Cavenee WK eds. WHO classification of tumours of the central nervous system. Lyon: IARC, 2007: 92-93. Lellouch-Tubiana A, Boddaert N, Bourgeois M et al. Angiocentric neuroepithelial tumor (ANET): a new epilepsy-related clinicopathological entity with distinctive MRI. Brain Pathol 2005; 15: 4: 281-6. Wang M, Tihan T, Rojiani AM et al. Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. J Neuropathol Exp Neurol 2005; 64: 10: 875-81.