Table 1.
Summary of PACNS with a tumor-like lesion in adult patients from January 1, 2000, to December 31, 2018.
| References | Age at diagnosis (years) and gender | Clinical presentation | Laboratory findings | CSF analysis | Type of vasculitis | MRI brain results | Location | Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Molloy et al. (2) | 43F | Reduced coordination and visuospatial disorientation with right inferior quadrantanopia | Not mentioned | Not mentioned | Lymphocytic | A large contrast enhancing mass with edema and local mass effect | The left parietal lobe | Surgical removal, glucocorticoids, and mycophenolate mofetil | No recurrence on 21 month follow up |
| Lee et al. (26) | 24M | Seizure | Not mentioned | Not mentioned | Lymphocytic | High signal on T2, low signal on T1, increased ADC, normal angiogram | The left frontal lobe | Corticosteroids and lesionectomy | Disappeared multiple-enhancing lesions on 8.1 year follow up |
| Lee et al. (26) | 37F | Sleeping tendency, poor oral intake, and decreased verbal output | Not mentioned | Not mentioned | Lymphocytic | 2.3 cm enhancing mass with edema, decreased ADC, MCA M1 occlusion, and left distal ICA stenosis on angiogram | Suprasellar area | Cyclophosphamide and corticosteroids | Decreased size on 1 year follow up |
| You et al. (27) | 35F | Headache and left-sided weakness | Normal | Increased total protein: 170 mg/dl; MBP 2.23 nmol/l; increased IgG in CSF index 20.5 mg/dl | Lymphocytic | A tumor-like mass with edema and enhancement | The right parietal lobe | Subtotal resection, prednisolone, and cyclophosphamide | Neurological symptoms disappeared on 6 week follow up |
| Tanei et al. (24) | 60M | Slight disorientation, left hemiparesis, and motor aphasia | Normal | Normal | Granulomatous | A slightly enhanced mass lesion with surrounding edema | The right parietal lobe | Surgical removal only | Improvement with no new lesions on 6 month follow up |
| Muccio et al. (25) | 46F | Progressive confusion, headache, dizziness, and nystagmus | Not mentioned | Not mentioned | Lymphocytic | Increased signal on FLAIR, low signal on T1, and signal intensification with contrast | The right temporal lobe | Steroids | Regression of the lesion on 3 month follow up |
| Pizzanelli et al. (28) | 50F | Speech disorder, headache, and apathy | Not mentioned | Not mentioned | Lymphocytic | Mixed hyperintensity on T2 with conspicuous edema | The left frontal lobe | Surgical removal, steroids, and cyclophosphamide | Recurrence after one year and 5 months |
| Killeen et al. (29) | 51M | Headache and left homonymous hemianopia | Normal | Mononuclear pleocytosis: 58/L elevated protein: 1400 mg/L | Lymphohistiocytic | Extensive perifocal edema, ring-enhancing and central necrotic mass on MRI | The white matter adjacent to the posterior horn of the right lateral ventricle | Oral prednisolone and cyclophosphamide | Suffered no recurrence on 4 year follow up |
| Kim et al. (22) | 20F | Right facial focal motor seizures | Elevated CRP: 9.80 mg/dL | Not mentioned | Lymphocytic | High signal intensity on T2 and heterogeneous enhancement with contrast | The left posterior frontal area | Surgical removal | No new lesions on 3 year follow up |
| Sun et al. (30) | 42M | Headache, convulsions, and aphasia | Not mentioned | Normal | Lymphocytic | A tumor-like mass with edema and high signal intensity on FLAIR | The left frontotemporal lobe | Surgical removal, methylprednisolone, and subsequent oral prednisolone | Only aphasia on 1 year follow up |
| Zhu et al. (17) | 22M | Jacksonian epilepsy and weakness in right thumb | Elevated CRP: 10.52 mg/L; elevated ESR: 22 mm/h; ANCA, ANA, and RF (-) | Elevated protein: 0.85g/L | Lymphocytic | Hypodensity on T1, hyperintensity on T2, no enhanced signal on Gd-enhanced image, and mild hyperintensity on FLAIR | The left temporal lobe | Methylprednisolone and subsequent oral prednisone | Most symptoms were relieved on 50 day follow up |
| Zhu et al. (17) | 31M | Recurrent unconsciousness, right limb convulsions, aconuresis or encopresis, headache, and nausea | Elevated CRP: 11.78 mg/L; elevated ESR: 18 mm/h ANCA, ANA, and RF (–) | Slightly elevated protein: 0.48 g/L | Lymphocytic | Mixed hyperintensity on T1 and T2, hyperintensity on FLAIR, and DWI and Gd-enhanced images | The left temporal lobe | Methylprednisolone and subsequent oral prednisone | Only mild memory impairment remained without adverse events on 7 month follow up |
| Zhang et al. (20) | 35F | Severe headache and gait disturbances | Not mentioned | Not mentioned | Lymphocytic | A tumor-like mass with edema | The left cerebellum | Corticosteroids | Recovered well without residual deficits on 3 month follow up |