case_images: PMC10698862_01
case_images: Preoperative imaging for index case of PLNTY with high-grade features. (A-C): T1-weighted axial, coronal and sagittal sequences with contrast demonstrating multifocal enhancement involving the right frontal lobe and cingulate gyrus. (D): T2/FLAIR-weighted axial sequence showing the bulk of the lesion to be T2/FLAIR hyperintense with clear mass effect and some surrounding vasogenic edema. (E): Susceptibility-weighted imaging showing two areas susceptibility likely corresponding to intralesional calcification given the matching hyperdensities seen on (F): axial computed-tomography imaging.
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case_images: CT demonstrated multiple masses associated with calcification in the right frontal lobe with vasogenic edema ( Figure 1F ). T1 hyperintensity. There was infiltrative appearing T2 signal hyperintensity extending across the midline along the corpus callosum with mild associated mass effect ( Figures 1A-E ).
case_images: Pathological examination of surgical specimen including DNA methylation analysis. (A): Histologic examination with geographic regions of necrosis with (B): adjacent regions of sinusoidal neovascularization. The majority of tumor tissue demonstrated a cytologically bland neoplasm, (C): comprised of regions with an infiltrative and (D): occasionally more demarcated growth pattern, often associated with microcalcification. (E): High power images of the tumor demonstrate some features suggestive of neurocytic differentiation, including open chromatin and prominent nucleoli. (F): The greatest degree of nuclear atypia was seen in regions adjacent to the necrosis, which demonstrated variable immunoreactivity for (G): GFAP and (H): synaptophysin, with (I): strong, diffuse staining for CD34. (J): Of note, the Ki-67 proliferative index was low, <1% in atypical cells. (K): Copy number analysis obtained from methylation array profiling data demonstrates whole chromosome gains of chromosomes 7, 8, 19, and 20, and loss of chromosome 10, and copy number gain of the FGFR3-TACC3 locus, consistent with the underlying fusion identified.
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case_images: Permanent histologic sections, however, showed a moderately cellular neuroepithelial neoplasm with abundant microcalcification, with perivascular, perineuronal, and subpial growth ( Figures 2C, D ). Regions of necrosis and neovascularization were thought to represent intra-tumoral infarction rather than true tumoral necrosis or microvascular proliferation ( Figures 2A, B ). The remainder of the tumor appeared cytologically bland with cytoplasmic clearing and branched capillaries resembling oligodendroglioma ( Figure 2E ). Immunohistochemistry demonstrated strong staining for GFAP in some portions of the tumor and synaptophysin reactivity in other portions ( Figures 2F-H ). Notably, the tumor was diffusely, strongly positive for CD34 ( Figure 2I ). The Ki-67 proliferation index was <1% ( Figure 2J ). No match was obtained, and copy number analysis revealed polysomy of chromosomes 7 and 8 with monosomy of chromosome 10 ( Figure 2K ).
case_images: PMC10698862_02
case_images: Progression-Free Survival (PFS) of glioblastomas from case series with FGFR3-TACC3 fusion. Median progression-free survival was 6 months (n = 8, 6 events). Hashmark indicates censored.
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case_images: Median progression-free survival in this cohort was 6 months ( Figure 3 ), while median overall survival was 15 months ( Figure 4 ).
case_images: Overall Survival (OS) of glioblastomas from case series with FGFR3-TACC3 fusion. Median overall survival of 15 months (n=8, 3 events). Hashmark indicates censored.
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case_images: Median progression-free survival in this cohort was 6 months ( Figure 3 ), while median overall survival was 15 months ( Figure 4 ).