Primary central nervous system vasculitis mimicking brain tumour: case report and literature review

Abstract

Primary central nervous system vasculitis (PCNSV) is a rare inflammatory disease causing significant morbidity and mortality. We present a detailed history and clinical course of a patient with PCNSV along with a literature review. A 50-year-old Chinese female presented with a 6-month history of mild to moderate headache and sudden onset of visual loss. Early computed tomography of the brain showed multifocal infarctions. Over 3 weeks after admission, her clinical presentations changed considerably and her condition deteriorated. She became confused then fell in to semicoma, and developed left-sided paralysis and quadriparesis. At about 7 months after her illness, a magnetic resonance imaging of the brain revealed a tumour-like mass lesion within the right parietal lobe resulting in compressed posterior horn of the right lateral ventricle. In the end, a brain biopsy identified extensive vessel inflammation, which established the diagnosis of PCNSV. Steroid and immunosuppressive therapy dramatically improved the patient's condition. Our report highlights the importance of considering PCNSV as a differential diagnosis in patients who are suspected with encephalitis, multiple sclerosis, cerebral haemorrhage, cerebral thrombus, leukoencephalopathy, or brain tumour, etc. while their clinical presentations are exceptionally so changeable. This case also gives emphasis to the value of a brain biopsy in consolidating the diagnosis, and the efficacy of steroid and immunosuppressive therapy in PCNSV.

Tumor-Like Presentation of Primary Angiitis of the Central Nervous System

“…[3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Focal deficit was the most frequent presenting symptom in 12 (54%) patients, followed by headaches in 11 (50%). Imaging revealed a single mass in 13 (62%) patients, supratentorial in 11 4, 6-10, 13, 17, 19, 20 and involving the spinal cord in two.…”

Pseudotumoral presentation of primary central nervous system vasculitis

“…PLACNS is a rare infl ammatory vasculitis that aff ects the parenchyma and meningeal arteries and veins [1][2][3][4] . An even less common subtype of PLACNS is the lymphocytic vasculitis 1,5 .…”

“…Th e pathogenesis, diagnosis, and treatment of the disease are challenging with few reports in the literature 3,5 . Its cause remains unclear, but a viral etiology, as well as an association with lymphoma, has been suggested 1,3 . If not treated, progressive neurological decline or even death may occur 1,4 .…”

“…PLANCS is usually diagnosed by exclusion based on radiological, pathological, and immunohistochemical fi ndings [5][6][7] . Th ree main histological patterns have been reported, the granulomatous infl ammatory pattern, the lymphocytic pattern, and the acute necrotizing one 3,8 . MRI, MRA, and DSA can help in the diagnosis 1,4 .…”

Tumefactive Primary Central Nervous System Vasculitis: Imaging Findings of a Rare and Underrecognized Neuroinflammatory Disease

“…An underrecognized and rarer subset of PCNSV, approximately 5%-29%, can present with "masslike" or "tumefactive" lesions mimicking a neoplasm. 3,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] To date, imaging features of tumefactive PCNSV (t-PCNSV) have not been well described and definitive diagnosis can only be made confidently on histopathology. [5][6][7][8][9][10] In this article, we performed a retrospective review of 10 histopathologically proved cases of t-PCNSV and analyzed pertinent imaging features with histopathologic correlation.…”