PMC10088409_01
Pre-operative MRI demonstrates a large right parietotemporal heterogeneously enhancing lesion with a cystic component and hypervascularity around the periphery. (a)-(c) T1-weighted images with contrast. (d, e) T2-weighted images. (f) Perfusion image.
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Magnetic resonance imaging (MRI) of the brain demonstrated a well-circumscribed large heterogeneously enhancing lesion of the right parietotemporal lobe with a cystic component without significant surrounding vasogenic edema (Figure 1).
Histopathology consistent with high-grade glioma with variable cellular morphology, a high proliferative index, numerous mitotic figures, perivascular pseudorosettes, true rosettes, complex microvascular changes, extensive geographic and palisading necrosis, interspersed entrapped neurons as well as some malignant multinucleated cells. (a, b) Perivascular rosettes (10x). (c, d) Perivascular rosettes (20x). (e) Embryonal area (5x). (f) Embryonal area (10x). (g) Embryonal area with rosettes (20x). (h) Embryonal immature area (40x). (i) Mix of morphologies with ganglion cells (20x). (j) Embryonal immature area with fascicular architecture and high-grade nuclei (20x). (k) Giant cell area with areas of necrosis (10x). (l) Giant cell area with areas of necrosis (20x).
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Overall, these findings were histologically consistent with high-grade glioma (Figure 2).
Immunohistochemistry consistent with diffuse hemispheric glioma, H3 G34-mutant. (a)-(c) H3 G34R/V stain with variable positivity with much of the tumor exhibiting weak nuclear staining in approximately 10% but some areas with dense 3 + nuclear staining in clusters (10x). (d) H3 K27M negative stain (10x). (e) ATRX with loss of nuclear staining (10x). (f) IDH1 R132H negative stain (10x). (g) OLIG2 negative stain (10x). (h) p53 mutated, strong positivity in viable tumor (10x). (i) Ki-67 significantly elevated with approximately 60% overall with some areas approaching 80%.
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H3 G34R/V exhibited variable positivity with much of the tumor demonstrating weak nuclear staining in approximately 10% but some areas with dense nuclear staining in clusters (Figure 3).
Florescence in situ hybridization. (a) 19q deletion not detected. (b) 1p deletion not detected. (c) BRAF with abnormal signal pattern of >2F (44%) consistent with gains or extra copies of the BRAF locus. (d) MET non-amplified. (e) MYCN non-amplified. (f) PDGFRA non-amplified.
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However, gains of the BRAF locus (>2F, 44%) were observed indicative of gains or extra copies of the BRAF gene region on chromosome seven (Figure 4).