Primary angiitis of the central nervous system presenting as a mass lesion

Gan, Calvin; Maingard, Julian; Giles, Lauren; Phal, Pramit M.; Tan, K. Meng

doi:10.1016/j.jocn.2015.03.030

Cited by 6 publications

(5 citation statements)

References 30 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…MR spectroscopy may be very beneficial for the evaluation of patients presenting with tumefactive lesions of the CNS. Typical findings in glioma include high choline, low N-acetyl-aspartate (NAA) peaks, and high lactate levels (23). Elevated glutamate and/or glutamine peaks were observed in three case reports, the local accumulation of which was due to the breakdown of both neural and glial elements and the associated adjacent astrocytic response under inflammatory conditions (25, 33, 39).…”

Section: Discussionmentioning

confidence: 99%

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

Jin

Guo

et al. 2019

Front. Neurol.

View full text Add to dashboard Cite

Primary angiitis of the central nervous system (PACNS) is a rare disorder resulting in idiopathic inflammation affecting the parenchymal and leptomeningeal vessels confined to the central nervous system (CNS), of which a tumor-like mass lesion is an even rare subtype. We described a case of PACNS initially misdiagnosed as glioblastoma. The patient was a 35 year-old female with right-sided weakness and expressive dysphasia. Brain MRI showed a tumor-like lesion highly suggestive of glioblastoma, therefor surgical removal was done. After a resection and an exhaustive workup, PACNS was ultimately diagnosed. The case illustrates a type of imaging presentation of PACNS that is often misdiagnosed as high-grade glioma. Differentiation between tumor-like PACNS lesions and actual CNS tumors is challenging due to similar MR images. To avoid unnecessary surgical interventions, we summarized previously reported mass-forming PACNS cases in adults from January 1, 2000, to December 31, 2018 and the imaging characteristics of PACNS. Some less commonly used diagnostic methods such as MR spectroscopy may also help clinicians distinguish PACNS from its mimics.

show abstract

Section: Discussionmentioning

confidence: 99%

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

Jin

Guo

et al. 2019

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

“…PACNS is restricted only to CNS. [1][2][3][4] Accurate diagnosis is challenging due to the rarity, presentation, and absence of any specific serological tests. Differential diagnoses include secondary angiitis, reversible cerebral vasoconstriction syndrome, and noninflammatory vasculopathies.…”

Section: Introductionmentioning

confidence: 99%

“…Differential diagnoses include secondary angiitis, reversible cerebral vasoconstriction syndrome, and noninflammatory vasculopathies. 1 It has various subsets of presentations, of which tumor-like presentation is seen in 9% of these patients. 1,2…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Primary Central Nervous System Angiitis Mimicking a Space-Occupying Lesion

Gandham

Patel

Mathew

et al. 2022

Indian Journal of Neurosurgery

View full text Add to dashboard Cite

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.

show abstract

Primary angiitis of the central nervous system presenting as a mass lesion

Cited by 6 publications

References 30 publications

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review

“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

Contact Info

Product

Resources

About