case_images: PMC10890803_01
case_images: Magnetic resonance images (MRIs) of a 56-year-old adult male with DLGNT WHO grade 3 (case 34). a, b Cranial MRI sagittal image in T2 FLAIR (a) and T1 with contrast (b) sequences, demonstrating a contrast-enhancing right cerebellar lesion with cystic changes (arrowheads, a, b), as well as diffuse leptomeningeal enhancement sugarcoating the entire surface of the brainstem and cervical medulla (arrows, a, b). c Cranial MRI axial image in T1 sequence with contrast showing bright contrast enhancement of the same cystic, right cerebellar lesion (black arrow). Biopsy of this lesion revealed DLGNT (WHO grade 3) with KIAA1549::BRAF fusion and 1p deletion. d Spinal MRI axial image in T1 sequence with contrast at the disk level between the third and fourth lumbar vertebrae, illustrating the sugarcoating effect (arrowheads) of the leptomeningeal dissemination (LMD) along the caudal fibers (black arrow). e, f Spinal MRI sagittal image of the lumbar spine (e) below the conus medullaris and of the thoracic spine (f) in T1 sequence with contrast, displaying the entire spinal cord sugarcoated by DLGNT dissemination with nodular leptomeningeal thickening. LMD was treated with local irradiation of the neuraxis, which was halted due to the clinical deterioration of the patient.
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case_images: F1
case_images: Magnetic resonance imaging of the neuraxis (shown in Fig. 1) revealed a small cystic lesion of the right cerebellar hemisphere, diffuse leptomeningeal enhancement of the cerebellum, brainstem, and cervical cord, especially ubiquitous nodular thickening of the spinal cord, including caudal fibers, and multiple punctuate subcortical lesions of cerebellar hemispheres.
case_images: a, b Hematoxylin and eosin (H&E) showing monomorphic, oligodendroglioma-like cells in the myxoid matrix without vascular proliferates or necrosis in low (a) and high (b) magnification. c H&E showing up to 4 mitotic figures/10 HPF (2 examples indicated by red arrowheads), conferring our patient (case 34) a WHO grade 3. d-f Immunochemistry staining positive for synaptophysin (d), Olig2 (e), and MIB1/Ki67 (f).
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case_images: F2
case_images: Microscopy revealed tumor cells strongly positive for synaptophysin (Fig. 2d) and negative for chromogranin A and beta-catenin. Biopsy of the cerebellar lesion demonstrated monomorphic, oligodendroglioma-like cells in the myxoid matrix without vascular proliferates or necrosis within the subarachnoid space, partially infiltrating parenchymal tissue (Fig. 2a, b). Neoplastic cells were strongly immunoreactive for CD56, MAP2, Olig2 (Fig. 2e), and alpha thalassemia/mental retardation syndrome X-linked (ATRX) protein. Melan A, and CD138. MIB1/Ki67 labeling index was ca. 15-20% (Fig. 2f).