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

Boysson, Hubert de; Boulouis, Grégoire; Dequatre, Nelly; Godard, Sophie; Néel, A.; Arquizan, Caroline; Detante, Olivier; Bloch-Queyrat, Coralie; Zuber, Mathieu; Touzé, Emmanuel; Bienvenu, Boris; Aouba, Achille; Guillevin, Loı̈c; Naggara, Olivier; Pagnoux, Christian

doi:10.1161/strokeaha.116.013917

Cited by 32 publications

(37 citation statements)

References 19 publications

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“…Conversely, PACNS presenting multiple lesions that also mimic aggressive multifocal cerebral neoplasm have been reported (26, 31–34). A retrospective study of 10 tumor-like PACNS cases in France found that the number of patients with single and multiple lesions was similar (4 and 3 patients, respectively) (35). The Mayo Clinic analysis showed that nearly 83% of the multiple lesions tended to be bilateral (7).…”

Section: Discussionmentioning

confidence: 99%

“…A study showed that 89% of PACNS patients with tumor-like lesions presented lymphocytic patterns, similar to other PACNS subtypes. Granulomatous patterns were next, accounting for approximately 14% (35). Unfortunately, immunohistochemistry and molecular analysis of T-cell and B-cell clonality was not completed in our patient because our neurosurgeons did not doubt the diagnosis of the patient's glioma before surgery.…”

Section: Discussionmentioning

confidence: 99%

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

Jin

Guo

et al. 2019

Front. Neurol.

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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.

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Section: Discussionmentioning

confidence: 99%

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.

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“…A combination of glucocorticoid and immunosuppressant were the recommended treatment of TLML-PACNS, and aggressive immunosuppressive therapy was associated with improved outcomes compared with glucocorticoids immunotherapy. [10,11] In our study, patients with severe lesion were improved with methylprednisolone and CyP therapy.…”

Section: Discussionmentioning

confidence: 52%

Seizure syndrome as a first manifestation of solitary tumor-like mass lesion of PACNS

Zhu

Yang

et al. 2017

Medicine

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Rationale:Primary angiitis of the central nervous system (PACNS) is an inflammatory disease involving cerebrovascular and parenchymal, and solitary tumor-like mass lesion of PACNS (TLML-PACNS) is frequently misdiagnosed as neoplastic or other inflammatory diseases. However, seizure syndrome as a first manifestation of TLML-PACNS has rarely reported before.Patient concerns:Here, we report 2 cases of seizure syndrome, which was the first sign that presented prior to the diagnosis of TLML-PACNS by brain biopsy.Diagnoses:A mass lesion in the white and gray matters was detected by magnetic resonance imaging. The pathology for leptomeningeal lesion biopsy observed a transmural inflammation of the artery, with T lymphocyte infiltration. Patients were diagnosed with PACNS and epileptic seizure by biopsy and electroencephalogram.Interventions:Patients were treated with glucocorticoid pulse therapy for 3 days, and subsequently oral prednisone was continued, in combination with immunosuppressant.Outcomes:Luckily, both two patients were improved after treatment, and only mild cognitive impairment remained without adverse event.Lessons:Patient with mass lesion in CNS, which is similar to tumor, presented with seizure, headache, or cerebrovascular events without any other risk factors for stroke or tumor, should be considered the feasible with the disease of TLML-PACNS.

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“…Magnetic resonance imaging is the preferred imaging in some hospitals, which shows single stenotic lesions in multiple blood vessels [35]. Steroids and immunomodulators are the preferred treatment that yields good outcomes in comparison to steroids alone [36][37]. There are no clear guidelines for the management of PACNS.…”

Section: Primary Central Nervous System Angiitismentioning

confidence: 99%

Association Between Cerebrovascular Accident and Vasculitis: Myth or Reality?

Aljanabi

Mamtani

Acharya

et al. 2019

Cureus

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This article aims to determine an association between vasculitis and cerebrovascular accidents (CVA) and the ideal management plan to decrease the chances of CVA in vasculitis patients. We also attempt to find a preferred treatment regimen that decreases the complications of CVAs in vasculitis patients, thereby resulting in reduced mortality and morbidity. We reviewed both free-access full-text articles and the abstracts of articles behind a paywall. We used the PubMed database and reviewed 89 articles that matched our inclusion and exclusion criteria. In all, 42 out of the 89 articles had the most relevant data for our article. We used the following keywords to search the database: vasculitis, stroke, cerebrovascular accident, ischemic, arteritis, and steroid. We found an association between subtypes of vasculitis, mostly large vessel vasculitis and CVA. We could not identify a specific cutoff value for specific inflammatory markers that can increase the risk of developing CVA. Besides, there are no formal guidelines for the dosage or the route of administration for corticosteroids, which are the cornerstone of treatment for most vasculitis. We found that male giant cell arteritis (GCA) patients have a higher chance of developing CVA than females, Also interestingly, anemia was found to be protective against CVA development in GCA patients. Sometimes, CVA can happen due to the effects of treatments of some types of vasculitis. We recommend establishing further studies about other subtypes of vasculitis and their associations with stroke.

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

Cited by 32 publications

References 19 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

Seizure syndrome as a first manifestation of solitary tumor-like mass lesion of PACNS

Association Between Cerebrovascular Accident and Vasculitis: Myth or Reality?

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