PMC6350233_01
A: Axial FLAIR sequence shows an inhomogeneous space-occupying lesion in the right region displacing the adjacent structures, compressing the right lateral ventricle, and obliterating the foramen of Monroe leading to a consecutive widening of the left lateral ventricle. B: Coronal T2-weighted image emphasizes the hyperintense cystic parts of the lesion. Flow void phenomena, corresponding to abnormal vessels are seen intralesionally on both sequences. C: H & E staining shows a cellular compact tumor with uniform cells displaying round to ovoid nuclei. Widespread intense immunoreactivity for (D) vimentin, (E) EGFR, and (F) Bcl2 was detectable. G: A widespread and strong nuclear expression of BCOR was present. H: Olig2 was expressed in a fraction of cells (Original magnification: C - H, x 200).
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Figure1
HGNET-BCOR in a 5-year-old male patient with a large 7 x 6 x 8-cm-sized frontally located, well demarcated tumor (Figure 1A, B). Histopathologically the tumor tissue was compact and highly cellular with rather uniform cells displaying round to ovoid nuclei with fine chromatin structure and a scant, slightly eosinophilic cytoplasm (Figure 1C). Immunohistochemically, a strong and widespread vimentin (Figure 1D), EGFR (Figure 1E, F), and NCAM expression was detectable. The majority of tumor cells showed a strong nuclear BCOR immunoreactivity (C-10, 1 : 200, Santa Cruz Biotechnology, Dallas, TX, USA) (Figure 1G). Olig2 was expressed in a fraction of cells (Figure 1H) and a weak cytoplasmic CD99 immunoreactivity was found.