PMC5640879_01
Brain magnetic resonance imaging 2 months after the onset of symptoms revealed an abnormal signal with hyper-density on T2-weighted images in the left thalamus and the dilated left Sylvian fissure (A). Overall enhancement was present in the cerebral meningeal, the cerebellar tentorium, the pia mater around left the thalamus, and the quadrigeminal cistern, but not in the left thalamus (B,C).
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F1
Initial brain MRI revealed a dilated left Sylvian fissure and an abnormal signal on the medial-dorsal side of the left thalamus (Figure 1A). Diffuse enhancement was noticed in the meninges, the left thalamus adjacent ventricular ependyma, the cerebellar tentorium, and the quadrigeminal cistern, whereas no enhancement was identified in the parenchyma (Figures 1B,C).
Contrasted brain magnetic resonance imaging 9 months after symptoms onset showed multiple enhanced encapsulated cyst-like lesions in the Sylvian fissure, dilated cortex sulci in the bilateral hippocampus and the medialis of bilateral frontal lobes, and a generally enhanced mass in the quadrigeminal cistern (A). The sagittal image clearly shows grape-line cysts along the Sylvian fissure (B).
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F2
Overall contrast enhancement was present in the cerebral meninges, the cerebellar tentorium, the cyst walls, and the quadrigeminal cistern (Figures 2A,B).
Brain magnetic resonance imaging 1 year after symptoms onset. The subarachnoid space was markedly enlarged. More contrasted cyst-like lesions were present in the Sylvian fissure, the dilated cortex, and the left basal ganglia area and a new non-contrasted solid lesion appeared in the left paraventricular white matter. The previous left thalamus lesion was consistently unenhanced (A-C). Magnetic resonance spectroscopy indicated a Cho/Cr wave ratio higher than 2.5 in the left thalamus and basal ganglia area (D), and approximately 1.0 in the contralateral symmetric area (E).
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F3
All the previous changes were found to be more pronounced, including the cyst-like signals in the dilated cortical sulci, the mass in the quadrigeminal cistern, and the enlarged subarachnoid space, and general meningeal enhancement in the brain and upper segment of the cervical spinal cord (Figures 3A-C). Magnetic resonance spectroscopy (MRS) indicated a Cho/Cr wave ratio higher than 2.5 in the left thalamus and basal ganglia area (Figure 3D), and 1.0 in the right symmetric area that appeared normal on MRI (Figure 3E).
Histological examinations of a specimen obtained in left temporal lobe. Tumor tissue was localized along the cerebral meninges (HE stain, 100x) (A). Spindle-shaped tumor cells with pleomorphic hyperchromatic nuclei and an increased mitotic rate spread in the subarachnoid space (B) (200x). The glial fibrillary acidic protein stain was strongly positive in the cytoplasm of tumor cells (C) (400x).
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F4
Brain biopsy of the left temporal lobe was performed and spindle-shaped tumor cells were found to be spreading into the leptomeninges and subarachnoid space, which were strongly positive for glial fibrillary acidic protein (GFAP) and S100, partly positive for OLIG2 and P53, and negative for IDH1(R132H), ARTX, and NeuN (Figure 4).