Tumor-mimicking primary angiitis of the central nervous system: initial and follow-up MR features

Lee, Youkyung; Kim, Ji Hoon; Kim, Eun‐Hee; Park, Shin Young; Yim, Younghee; Sohn, Chul Ho; Chang, Kee Hyun

doi:10.1007/s00234-009-0546-3

Cited by 34 publications

(64 citation statements)

References 15 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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“…5,8,15) Single or multiple mass lesions are also known in PACNS. 8,11,12,19) Approximately 5% of cases with PACNS present as a tumor-like mass lesion. 11) One of the mechanisms of formation of mass lesion is breakdown of the blood-brain barrier of the small vessels by infiltration of inflammatory cells in the perivascular and parenchymal regions, resulting in the mass mimicking enhanced lesion.…”

Section: Discussionmentioning

confidence: 99%

“…11) One of the mechanisms of formation of mass lesion is breakdown of the blood-brain barrier of the small vessels by infiltration of inflammatory cells in the perivascular and parenchymal regions, resulting in the mass mimicking enhanced lesion. 8) Cerebral angiography is useful in making the presumptive diagnosis of cerebral vasculitis, but abnormal angiographic findings were reported in only 25% of patients with PACNS. 4) Nine of 14 patients who were diagnosed with PACNS by biopsy had normal angiography findings.…”

Section: Discussionmentioning

confidence: 99%

“…15,17,19) The MR imaging findings of PACNS are multiple bilateral asymmetrical supratentorial lesions representing infarctions, which predominantly occur in the subcortical and deep white matter. 5,8,15) Single or multiple mass lesions are also known in PACNS. 8,11,12,19) Approximately 5% of cases with PACNS present as a tumor-like mass lesion.…”

Section: Discussionmentioning

confidence: 99%

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

Tanei

Nakahara

Takebayashi

et al. 2011

Neurol. Med. Chir.(Tokyo)

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A 60-year-old man presented with a rare case of primary angiitis of the central nervous system mimicking a tumor-like lesion and manifesting as slight disorientation, left hemiparesis, and motor aphasia. Computed tomography showed multiple low density lesions in the left frontal lobe, brain stem, and right parietal lobe. Magnetic resonance images revealed a slightly enhanced mass lesion in the right parietal lobe with surrounding brain edema. Serum, cerebrospinal fluid, and other image examinations did not show any abnormal findings, so surgical removal of the right parietal mass was performed. Histological examination revealed that the mass consisted of hemorrhagic infarction without cellular atypia. Proliferations of endothelial cells in small and medium arteries, and infiltration of macrophages in the perivascular space were detected in the infarction tissues. The histological diagnosis was primary angiitis of the central nervous system.

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“…MRI usually demonstrates high signal white matter foci in fluid-attenuated inversion recovery (FLAIR) and T2-weighted images [42]. Tumor mimic lesions are reported mainly in primary CNS vasculitis [43]. MRI can demonstrate post contrast enhancement of the arterial wall, which is an indicator of active inflammation [44].…”

Section: Central Nervous System Findings In Childhood Systemic Vasculmentioning

confidence: 98%

Imaging of systemic vasculitis in childhood

et al. 2015

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The term "systemic vasculitis" encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children.

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Tumor-mimicking primary angiitis of the central nervous system: initial and follow-up MR features

Abstract: Tumor-mimicking PACNS shows variable features on initial MR images but shows good responses to appropriate immunosuppressive therapy on follow-up MR images.

Cited by 34 publications

References 15 publications

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

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

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

Imaging of systemic vasculitis in childhood

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