Primary Angiitis of the Central Nervous System Mimicking Tumor-Like Lesion  -Case Report-

Tanei, Takafumi; Nakahara, Norimoto; Takebayashi, Shigenori; Ito, Masafumi; Hashizume, Yoshio; Wakabayashi, Toshihiko

doi:10.2176/nmc.51.56

Cited by 20 publications

(13 citation statements)

References 19 publications

(45 reference statements)

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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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“…MR-angiography (MRA) is suggestive of vasculitis in 45% [22]. MRI of PACNS may be suspicious for brain tumour, and PACNS mimicking tumour-like lesions is often diagnosed by biopsy by chance [32]. Conventional cerebral angiography is indicative of vasculitis in up to 75% if performed repeatedly [1,8,11,31].…”

Section: Diagnosis Of Definitive Vasculitismentioning

confidence: 99%

Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls

Berlit

Kraemer

2014

Clinical and Experimental Immunology

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Cerebral vasculitis is a rare cause of juvenile stroke. It may occur as primary angiitis of the central nervous system (PACNS) or as CNS manifestation in the setting of systemic vasculitis. Clinical hints for vasculitis are headache, stroke, seizures, encephalopathy and signs of a systemic inflammatory disorder. Diagnostic work-up includes anamnesis, whole body examination, laboratory and cerebral spinal fluid (CSF) studies, magnetic resonance imaging (MRI), angiography and brain biopsy. Due to the rarity of the disease, exclusion of more frequent differential diagnoses is a key element of diagnostic work -up. This review summarizes the steps that lead to the diagnosis of cerebral vasculitis and describes the red flags and pitfalls. Despite considering the dilemma of angiography-negative vasculitis and false-negative brain biopsy in some cases, it is important to protect patients from 'blind' immunosuppressive therapy in unrecognized non-inflammatory differential diagnosis.

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“…Approximately 4% of PCNSV cases involved a solitary tumor-like lesion [14]. Excision was curative in some cases, and high-dose steroids, cyclophosphamide, azathioprine and immunoglobulins resulted in a favorable outcome in others [1257911]. …”

Section: Discussionmentioning

confidence: 99%

“…PCNSV has an annual incidence rate of 2.4 cases per million personyears and causes significant morbidity and mortality [123]. Cerebral vasculitis causes various neurological symptoms such as headache, hemiparesis, and mental disturbances, and diagnosis can be difficult using magnetic resonance imaging (MRI) with conventional sequences [345].…”

Section: Introductionmentioning

confidence: 99%

Primary Central Nervous System Vasculitis Mimicking a Cortical Brain Tumor: A Case Report

Lee

Jung

Lee

et al. 2017

Brain Tumor Res Treat

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We report a case of primary central nervous system vasculitis (PCNSV) mimicking a cortical brain tumor. A 25-year-old woman presented with a 2-week history of headache and transient right hemiparesis. Brain magnetic resonance imaging (MRI) revealed a cortical-involving lesion on the left frontal lobe. The 6-cm sized lesion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The lesion had continual linear enhancement on the subcortical white matter and leptomeninges. There was no evidence of hemorrhage on susceptibility-weighted images and no diffusion restriction on diffusion-weighted images. The regional cerebral blood volume was decreased on the MR perfusion images, and spectroscopy showed increased lactate and lipid peaks. The symptoms were aggravated by fever and seizures. Biopsy was performed to rule out tumorous or inflammatory lesions. Pathologically, lymphocytes were infiltrated on the vessels, and the arachnoid membrane was thickened with inflammatory cells. The patient did not have any underlying diseases, including immune disorders. After high-dose steroid administration, her symptoms improved. Two months later, brain MRI showed a reduction in the infiltration of the T2 hyperintensity lesion with subtle subcortical enhancement. We present a case of PCNSV involving the left frontal lobe, showing vasogenic edema, mass effect, and subcortical linear contrast enhancement without hemorrhage or infarction.

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Primary Angiitis of the Central Nervous System Mimicking Tumor-Like Lesion -Case Report-

Cited by 20 publications

References 19 publications

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

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

Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls

Primary Central Nervous System Vasculitis Mimicking a Cortical Brain Tumor: A Case Report

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