Pseudotumoral presentation of primary central nervous system vasculitis

Lyra, Tatiana Goyanna; Martin, Maria da Graça Morais; Carvalho, Rodrigo do Carmo; Oliveira, Cláudia Regina Gomes Cardim Mendes de; Godoy, Luís Filipe de Souza; Delgado, Daniel de Sousa; Cerri, Giovanni Guido; Leite, Cláudia da Costa

doi:10.1590/0004-282x20130032

Cited by 6 publications

(4 citation statements)

References 10 publications

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“…[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.…”

Section: Data From the Literaturementioning

confidence: 99%

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

Boysson

Boulouis

Dequatre

et al. 2016

Stroke

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Background and Purpose— We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. Methods— We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. Results— The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30–48] years versus 46 [18–79] years; P =0.008; 9 [90%] with seizures versus 22 [29%], P <0.001; respectively). All 10 patients had a biopsy (stereotactic procedure in 7 and open-wedge surgery in 3). Histological findings suggestive of vasculitis were observed in 9 patients in whom conventional cerebral angiography and magnetic resonance angiography were negative. In the remaining patient, vascular imaging demonstrated diffuse bilateral large- and medium-sized vessel involvement (biopsy did not reveal vasculitis). All patients with tumor-like presentation received glucocorticoids, combined with cyclophosphamide in 9 cases. With a median follow-up of 27 (12–130) months, 5 (50%) patients relapsed, but achieved remission again after treatment intensification. Conclusions— Patients with tumor-like presentation of primary angiitis of the central nervous system represent a subgroup characterized with mainly small-sized vessel disease that requires histological confirmation because vascular imaging is often normal. Although relapses are not uncommon, global outcomes are good under treatment with glucocorticoids and cyclophosphamide.

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Section: Data From the Literaturementioning

confidence: 99%

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

Boysson

Boulouis

Dequatre

et al. 2016

Stroke

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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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“…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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Pseudotumoral presentation of primary central nervous system vasculitis

Cited by 6 publications

References 10 publications

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

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

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

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

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