Article Figures & Data
Figures
- FIG 1.
Common MR imaging features of t-PCNSV in 4 different patients. A and E: 68-year-old man (case 5), B and F: 16-year-old male adolescent (case 6), C and G: 42-year-old man (case 8); and D and H: 55-year-old woman (case 7). Axial postcontrast T1-weighted images (A–D) demonstrate lobar masses. The masses have patchy heterogeneous enhancement (mottled appearance) on A–C (open arrow) and nodular and linear (perivascular enhancement) on D (open arrowhead). Leptomeningeal enhancement is noted on A, B, and D (black arrows). Subependymal enhancement is observed on C (white arrow). Axial susceptibility-weighted imaging (E, F, and H) and axial T2*-weighted gradient-echo imaging (G) demonstrate microhemorrhages. Punctate microhemorrhages are shown in E–H (white arrowheads). Linear microhemorrhages are demonstrated on G and H (black arrowheads).
- FIG 2.
MR imaging findings with histopathologic correlation of t-PCNSV. A 42-year-old man (case 1) presented with first episode seizure. Axial fluid-attenuated inversion recovery (A) shows a hyperintense lesion involving the cortex and subcortical white matter (white arrow). Axial post gadolinium T1-weighted imaging (B) demonstrates that the lesion has small nodular enhancement (black arrow). The initial imaging diagnosis was glioma. This patient underwent a gross total resection of the mass. Hematoxylin and eosin-stained (C: 2 mm and D: 200 µm magnifications) demonstrated vasculocentric transmural lymphocytic infiltration involving the leptomeningeal artery (C, white arrowhead) and parenchymal cortical arteries (C and D, black arrowheads). Immunohistochemically, the most infiltrating lymphocytic cell is positive for CD20 marker for B-cell lymphocytes (E) and CD3 marker for T-cell lymphocytes (F). The minority of inflammatory cells are macrophages that are positive for CD163 (G).
- FIG 3.
Recurrent granulomatous t-PCNSV. A 30-year-old man presented with headaches (case 4). A and B: Initial brain MR imaging shows conglomerate ring-enhancing mass centered in the right basal ganglia (arrow) with subependymal enhancement (arrowhead) on axial post gadolinium T1-weighted images (Gd-T1WI) (A). This mass does not show diffusion restriction on DWI (B). He underwent stereotactic-guided biopsy and histopathology showed granulomatous t-PCNSV. He was treated with corticosteroid and immunosuppressive agents. C: Follow-up MR imaging at 12 months shows the resolution of the disease. D and E: Follow-up MR imaging at 48 months demonstrates recurrent disease in the same region on Gd-T1WI (D). This mass does not show an increase in relative cerebral blood volume on dynamic susceptibility contrast MR perfusion (E). He was again treated with corticosteroids and an immunosuppressive agent. F: Follow-up Gd-T1WI at 77 months confirms the remission of the disease.
Tables
Patient Demographics Age, median; range (years) 42 (16–68) Sex, male (%) 7 (70%) Clinical presentations, n (%) Headache 4 (40%) Seizure 3 (30%) Hemiparesis 3 (30%) Confusion 2 (20%) Visual disturbance 2 (20%) Cognitive impairment 1 (10%) Word-finding difficulty 1 (10%) Location of involvement, n (%) Cortex/subcortical white matter 7 (70%) Deep white matter 6 (60%) Basal ganglia 4 (40%) Brain stem 1 (10%) Enhancement patterns, n (%) Patchy enhancement 5 (50%) Small nodular enhancement 5 (50%) Ring enhancement 2 (20%) Linear/perivascular enhancement 1 (10%) Leptomeningeal enhancement 4 (40%) Subependymal enhancement 3 (30%) Microhemorrhages, n (%) 8 (80%) Patients with available CSF analysis, n (%) 5 (50%) Abnormal CSF protein (>0.5 g/L) 3 (60%) CSF leukocytosis (>5 cells/mm3) 4 (80%) Patients with available serum inflammatory marker, n (%) 8 (80%) High ESR (>20 mL/h) 2 (25%) High CRP (>5 mg/L) 2 (25%) Surgical procedure Stereotactic-guided biopsy 5 (50%) Tumor resection 4 (40%) Open wedge biopsy 1 (10%) Histopathology Lymphocytic vasculitis 7 (70%) Lymphocytic plus necrotizing vasculitis 2 (20%) Granulomatous vasculitis 1 (10%) Treatment Steroid alone 5 (50%) Steroid plus immunosuppressive agents 5 (50%) Total duration follow-upa, median (range), months 18 (4–77)a ↵a Three patients did not have available clinical follow-up detail.
