Solitary tumour-like mass lesions of the central nervous system: Primary angiitis of the CNS and inflammatory pseudotumour

Killeen, Tim; Jucker, Dario; Went, Philip; Muthurajah, Vanaja; Woon, Kelvin; Cesnulis, Evaldas; Czapliński, Adam

doi:10.1016/j.clineuro.2015.05.008

Cited by 8 publications

(4 citation statements)

References 9 publications

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“…Abnormal in ND 100% [18] 100% [12] ND ND 100% [47] 100% [19] 100% [31] 100% [5] 100% [ x Kumar, 2017 [30] x Takahashi, 2017 [31] x x x Lee, 2017 [32] x x Arif, 2017 [33] x x x Mizuno, 2016 [34] x x Sun, 2016 [35] x x x Benson, 2016 [36] x x Pillai, 2016 [37] x x x Fang, 2015 [38] x x x Kim, 2015 [39] x X x Bajaj, 2015 [40] x x x Kim SI, 2015 [41] x Killeen, 2015 [42] x x x Gan, 2015 [43] x x Huang, 2015 [44] x x x Okunomiya, 2014 [45] x x Safouris, 2014 [46] x x x Michiels, 2014 [47] x x x x Gaillard, 2014 [48] x x Rao, 2014 [49] x x Noh, 2014 [50] x x x Safouris, 2013 [46] x x x Orr, 2013 [51] x x x Lyra, 2013 [52] x x Rosenberg, 2013 [53] x x Okeda, 2012 [54] x x Yu, 2011 [55] x…”

Section: Black Blood Mrimentioning

confidence: 99%

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

et al. 2020

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Background Primary angiitis of the CNS (PACNS) is a process causing variously combined neurological disturbances. Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect. Objective (1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations. Methods Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as “major” or “minor” if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS. Results We reviewed 24 case series. “Major” clinical features were headache, stroke, cognitive impairment, focal neurological deficits; “minor” were seizures, altered consciousness, psychiatric disorders. “Major” neuroradiological features were multiple parenchymal lesions, parenchymal/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; “minor” were parenchymal/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major/minor) + one major neuroradiological feature; and (2) Two clinical (≥ 1 major) + one minor neuroradiological feature. Conclusion Our review provides a detailed clinical/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.

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Section: Black Blood Mrimentioning

confidence: 99%

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

et al. 2020

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“…PACNS manifests as a single large mass lesion in around 5 to 12% of cases. 5,6 The most common manifestations are headache, episodic encephalopathy, progressive cognitive impairment, stroke, transient ischemic attacks, cranial neuropathy, myelopathy, and ataxia. 7 CT, MRI, and angiography are all useful diagnostic imaging modalities.…”

Section: Discussionmentioning

confidence: 99%

Primary Central Nervous System Angiitis Mimicking a Space-Occupying Lesion

Gandham

Patel

Mathew

et al. 2022

Indian Journal of Neurosurgery

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Background and Purpose Intracranial space-occupying lesions are a sine qua non for neoplastic lesions; however, occasionally non-neoplastic lesions mimic neoplastic lesions, leading to diagnostic dilemmas. We report our experience with three patients who presented with a progressive hemispheric syndrome and the diagnostic considerations involved in the cases. Materials and Methods In this retrospective study, we included three patients with primary angiitis of central nervous system (PACNS) who underwent craniotomy and biopsy, suspecting it to be mass lesions. Demographic features, clinical features, radiological features, histopathology, treatment, and clinical outcomes were studied. Results Majority were males. The male:female ratio was 2:1. Lobar involvement was common. MR brain with contrast showed features of high-grade glioma. Despite hemispheric involvement, there was no mass effect. Perilesional edema was seen in all cases. All underwent craniotomy and biopsy; histopathology was consistent with PACNS. All patients were treated with corticosteroids and cyclophosphamide. Rituximab was used in addition to cyclophosphamide in one patient. At 2 years follow-up, two patients were in disease remission and one patient died due to disease progression. Conclusion PACNS has a protean clinical manifestation. A high index of suspicion is required in cases with atypical clinical presentations, radiological features, and normal angiograms. Early histological diagnosis and aggressive immunotherapy with high-dose corticosteroids combined with intravenous cyclophosphamide yields favorable outcomes.

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“…Radiologic features of t-PCNSV were not well described or recognized because it is extremely rare entity. 4 To our knowledge, apart from several case reports [11][12][13][14][15][16][17][18][19][20][21][22] and review articles, [23][24][25] only a few small case series have been published in the medical literature. [6][7][8]10 Molloy et al 7 reported 8 cases of masslike PCNSV with the proved histopathology including both "Ab -related" PCNSV and "non-Ab -related" PCNSV from 2 tertiary institutions.…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

mentioning

confidence: 99%

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

Suthiphosuwan¹,

Bharatha

Hsu

et al. 2020

AJNR Am J Neuroradiol

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Primary central nervous system vasculitis (PCNSV) is a poorly understood neuroinflammatory disease of the CNS affecting the intracranial vasculature. Although PCNSV classically manifests as a multifocal beaded narrowing of the intracranial vessels, some patients may not have angiographic abnormalities. A rare subset of patients with PCNSV present with masslike brain lesions mimicking a neoplasm. In this article, we retrospectively review 10 biopsy-confirmed cases of tumefactive PCNSV (t-PCNSV). All cases of t-PCNSV in our series that underwent CTA or MRA were found to have normal large and medium-sized vessels. T-PCNSV had a variable MR imaging appearance with most cases showing cortical/subcortical enhancing masslike lesion (70%), often with microhemorrhages (80%). Diffusion restriction was absent in all lesions. In summary, normal vascular imaging does not exclude the diagnosis of t-PCNSV. Advanced imaging techniques including MR perfusion and MR spectroscopy failed to demonstrate specific findings for t-PCNSV but assisted in excluding neoplasm in the differential diagnosis. Biopsy remains mandatory for definitive diagnosis.ABBREVIATIONS: PCNSV ¼ primary central nervous system vasculitis; t-PCNSV ¼ tumefactive PCNSV; ABRA ¼ amyloidb -associated angiitis; CAA-RI ¼ cerebral amyloid angiopathy-related inflammation; ESR ¼ erythrocyte sedimentation rate; MRP ¼ MR perfusion; CRP ¼ C-reactive protein; PCR ¼ polymerase chain reaction; VWI ¼ vessel wall imaging; PCNSL ¼ primary CNS lymphoma P rimary central nervous system vasculitis (PCNSV) is a poorly understood neuroinflammatory disease involving intracranial vessels. [1][2][3][4][5] The typical radiologic manifestation of PCNSV is multifocal beading of the large and medium-sized intracranial vessels. 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. CASE SERIES Case SelectionWe performed a retrospective pathology data base search by using the keywords "brain biopsy" and "vasculitis" in histopathology reports from July 2010-December 2018 at a single institution. Exclusion criteria included patients with histopathology findings of amyloid-beta (Ab )-associated angiitis (ABRA) or cerebral amyloid angiopathy-related inflammation (CAA-RI), and infectious CNS vasculitis. Finally, we identified 6 patients who had a final diagnosis of t-PCNSV from this institution. The other 4 patients with t-PCNSV were collected from the imaging and histopathology archives of the contributing authors from 2 other institutions.All 10 patients presented with masslike brain...

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Solitary tumour-like mass lesions of the central nervous system: Primary angiitis of the CNS and inflammatory pseudotumour

Cited by 8 publications

References 9 publications

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

“When should primary angiitis of the central nervous system (PACNS) be suspected?”: literature review and proposal of a preliminary screening algorithm

Primary Central Nervous System Angiitis Mimicking a Space-Occupying Lesion

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

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