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Table of Contents
Year : 2021  |  Volume : 4  |  Issue : 2  |  Page : 56-60

Primary CNS vasculitis mimicking glioma: A case report and literature review

Department of Neurosurgery, LTMMC & LTMG Hospital, Mumbai, Maharashtra, India

Date of Submission16-Jun-2021
Date of Acceptance16-Feb-2022
Date of Web Publication20-Apr-2022

Correspondence Address:
Dr. Batuk D Diyora
Department of Neurosurgery, LTMMC & LTMG Hospital, Sion, Mumbai 400022, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJNO.IJNO_17_21

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Primary central nervous system vasculitis (PCNSV) is a rare disorder usually presenting with nonspecific complaints. Its incidence is estimated to be 2.4 per million population per year. Very few reported cases have developed into large tumor-like lesions mimicking glioma. It is a diagnosis of exclusion when systemic vasculitis is ruled out. High-dose steroids with or without the combination of cyclophosphamide (CYP) help in remission. Biopsy with histopathology helps in forming a definitive diagnosis. Excision of the mass lesion is required in some cases.

Keywords: Mimicking glioma, tumor-like lesion, vasculitis

How to cite this article:
Mullah M, More N, Devani K, Diyora BD. Primary CNS vasculitis mimicking glioma: A case report and literature review. Int J Neurooncol 2021;4:56-60

How to cite this URL:
Mullah M, More N, Devani K, Diyora BD. Primary CNS vasculitis mimicking glioma: A case report and literature review. Int J Neurooncol [serial online] 2021 [cited 2023 Jun 1];4:56-60. Available from: https://www.Internationaljneurooncology.com/text.asp?2021/4/2/56/343564

  Introduction Top

PCNSV (also known as primary angiitis of the central nervous system) is a rare entity that was first described in 1959.[1] As per a U.S. report, its incidence is 2.4 cases per million population.[1],[2] It describes inflammation of blood vessels in the brain and spinal cord without any features of systemic vasculitis. Its clinical presentation is variable. Very few cases have been reported where it has progressed to the large tumor-like lesion (TL, PCNSV) with a severe edema mimicking glioma. We describe a case of PCNSV in a young male patient and review the reported cases in the literature.

  Case history Top

A 25-year-old male patient presented with complaints of severe headache followed by three episodes of generalized tonic–clonic convulsions. It was followed by postictal transient aphasia. On examination, his consciousness, orientation, and speech were well preserved without any apparent neurodeficit. Baseline laboratory analysis was normal. Magnetic resonance imaging (MRI) brain showed a 4 × 4×5 cm, well-defined intra-axial heterogenous T2/FLAIR central hypointense and peripheral hyperintense lesion with patchy areas of restriction on diffusion-weighted images. The postcontrast study showed a thick ring of heterogenous peripheral enhancement. There was a significant mass effect, vasogenic edema with a midline shift toward the opposite side [Figure 1]. Magnetic resonance (MR) spectroscopy showed a raised choline and creatinine peak with Cho/NAA ratio > 2, and a lipid-lactate peak was also present. The first differential diagnosis was high-grade glioma due to heterogeneous enhancement, disproportionate edema, and choline peak. The second differential diagnosis was tuberculoma due to central T2 hypointense areas and lipid-lactate peak.
Figure 1: Magnetic resonance imaging (MRI) scan of the brain revealed about a 4 cm × 4 cm × 5 cm sized well-defined intra-axial lesion in the left frontoparietal region. The lesion appeared to be of a mixed intensity on T2-weighted (A) and FLAIR images (B) with significant perilesional edema. The patchy areas of diffusion restriction noted on diffusion-weighted images (C). Post-contrast images showing a thick ring of heterogenous peripheral enhancement on coronal (D), sagittal (E), and axial (F) plane

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Because of signs of raised intracranial pressure and mass effect, the decision was made to perform craniotomy and excision of the tumor. Intraoperatively, the tumor was unusual. It had a rubbery consistency with areas of necrosis and hemorrhage seen. The tumor was not easily suckable by high-pressure suction. The histopathological examination showed vessels inside the necrotic tissue with increased perivascular lymphocytes and lymphocytic infiltration in the vessel wall. Features of myolysis and endotheliitis were also seen. Some vessels were thrombotic, with recanalization seen in a few [Figure 2]. Several reviews of histopathology slides did not show any evidence of granuloma, malignancy, or any organism, nor was there any positivity on Congo Red. Systemic analysis was done with ESR, CSF analysis, Toxoplasma antibodies, RA factor, ANA, ANCA, and CT scan of chest and abdomen; all of these were normal. The diagnosis of PCNSV of a lymphocytic variety was established based on the histopathological findings and the absence of systemic disease. Pulse steroid (CS) therapy with intravenous methyl prednisolone was given 1gm per day for three days, later tapering to every three days. Then, the patient was shifted on to oral prednisolone 1 mg/kg/day and later tapered weekly. In the event of relapses of seizure episodes, antiepileptics were added and Cyclophosphamide (CYP) pulse therapy was given once a month for three pulses. The CYP pulse was stopped later as the patient developed disseminated tuberculosis and received antituberculosis treatment for nine months. Follow-up MRI brain that was performed at three months and 16 months after surgery showed no new lesion [Figure 3] and [Figure 4]. The patient was seizure-free on four antiepileptics for two years as of now with no sign of relapse or recurrence.
Figure 2: H and E-stained section of the post-surgical specimen showing perivascular diffuse infiltration of lymphocytes around necrotic tissue (shown by arrow A) and thrombosed vessels with intravascular infiltration of lymphocytes with endotheliitis and myolysis (B)

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Figure 3: Magnetic resonance imaging (MRI) scan of the brain (at 3 months’ follow-up) showing a significant reduction in the size of the lesion on FLAIR images (A) and on post-contrast images (B)

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Figure 4: Magnetic resonance imaging (MRI) scan of the brain (at 16 months’ follow-up) showing no evidence of residual or recurrence of lesion on axial T1-weighted images (A), T2-weighted images (B), and post-contrast images (C)

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  Discussion Top

PCNSV is a rare disease with approximately 2.4 cases per million population, as per the estimation of a U.S. study.[1] Its overall world prevalence is unknown. Its diagnosis is usually established once other suspected factors are ruled out. It is most common in the age group of 30–50.[2],[3] There are very few case reports and large series published of PCNSV and very few of them present with a TL[2],[4],[5],[6],[7],[8],[9],[10],[11],[12] [Table 1]. The most common presentation is headache or seizures. PCNSVs have shown slight male preponderance. The patients who were diagnosed with PCNSV were subjected to CT angiogram and a detailed MRI evaluation. The ones with a high index of suspicion of a nontumor-like lesion underwent biopsy, with stereotactic-guided biopsy being the most common method of biopsy. The majority underwent medical management with very low relapse rates and comparable cure rates.
Table 1: Case series with tumor-like presentation in primary CNS Vasculitis

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The French vasculitis study group had 10 patients with TL presentation out of a total of 85 patients with PCNS. Out of these, seven underwent stereotactic biopsy and three underwent craniotomy. In this study, TL-PCNSV cases were found in younger populations and had a greater incidence of seizure as compared with those without TL lesions.[8]

Headache was the most common presentation followed by seizures, neurological deficits, and altered sensorium.[13] Sometimes, it may also present with features of raised intracranial pressure, meningoencephalitis, and stroke. Other uncommon symptoms were a transient ischemic attack, ataxia, aphasia visual disturbance, and subarachnoid hemorrhage. Most of the cases are idiopathic.[13] Association with a viral infection such as Herpes Zoster, Hepatitis virus, or Retrovirus and bacterial association with leptomeningitis or meningoencephalitis are also found in some cases. CNS Amyloidosis or Hodgkin’s disease is also rarely associated with PCNSV. PCNSV is histologically of three types: granulocytic (58%), necrotizing (22%), and lymphocytic (20%).[14] It is usually a diagnosis of exclusion. Histopathological findings such as granulocytic or lymphocytic infiltration inside vessel walls and perivascular area with thrombotic vessels help to form a definitive diagnosis. Our patient showed lymphocytic variety. Depending on the type of vessel involved, it can be classified into small vessel (SV) PCNSV or medium vessel (MV) PCNSV.[13] The only MR spectroscopy that helps in diagnosis is glutamate/glutamine uptake.[5] MRI perfusion scan may also aid in diagnosis.[10] Angiography in CT/MRI or digital subtraction form may show some thrombotic vessels, especially in MV diseases.[11] SV PCNSV is difficult to diagnose on angiography.

The diagnostic criteria for labeling PCNSV are unexplained neurologic deficit after thorough clinical and laboratory evaluation, the arteritic process demonstrated by cerebral angiogram and histopathologic examination in CNS, and the absence of evidence of systemic vasculitis.[15]

Treatment with steroid therapy (CS) shows remission in a few cases and may need an adjuvant immunosuppressant. There is no randomized controlled trial for this and treatment is mainly based on the guidelines of systemic vasculitis.[13] Methylprednisolone (CS) pulse therapy 1gm/day tapered over three days was used, and this later shifted to oral prednisolone tapered weekly. In our case, the patient showed relapsing seizures on monotherapy; hence, pulse therapy of CYP 1gm monthly was started. CYP can also be given for 125 mg/day for two weeks. Molloy et al.[6] opined that if TL-PCNSV is entirely resected, there is usually no need for adjuvant CYP. However, as per the French vasculitis study group, the CS+CYP combination has a better remission rate than CS monotherapy in TL-PCNSV even if excision is done.[8]Chang et al.[11] from China had a total of 28 patients with TL-PCNSV, of whom three underwent excision, and the remaining underwent navigation, stereotactic or robotic biopsy. In this study, 27 patients received CS+CYP. CYP might have the disadvantage of immunosuppression leading to superinfection, as in our case with disseminated TB. Recent pieces of evidence have shown promising results with Rituximab.[16] Other drugs that can also be used as adjunctive therapy are mycophenolate mofetil, methotrexate, and tocilizumab.

  Conclusion Top

PCNSV is a rare pathology that is also masquerading as a tumor or glioma-like lesion. Histopathology gives a definitive diagnosis. Preoperative angiography, glutamate spectroscopy, MR perfusion may help in diagnosis of this condition. If resected, tumor-like lesions with mass effects have a better outcome. Pulse steroid or CYP is the treatment of choice. This treatment may affect the patient’s immunity, leading to secondary infections such as tuberculosis and should be watched out for.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Godasi R, Pang G, Chauhan S, Bollu PC . Primary central nervous system vasculitis. 2021 Sep 20. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022.  Back to cited text no. 1
Salvarani C, Brown RD Jr, Christianson TJH, Huston J 3rd, Morris JM, Giannini C, et al. Primary central nervous system vasculitis mimicking brain tumor: Comprehensive analysis of 13 cases from a single institutional cohort of 191 cases. J Autoimmun 2019;97:22-8.  Back to cited text no. 2
Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, et al. Primary central nervous system vasculitis: Analysis of 101 patients. Ann Neurol 2007;62:442-51.  Back to cited text no. 3
Singh S, John S, Joseph TP, Soloman T. Primary angiitis of the central nervous system: MRI features and clinical presentation. Australas Radiol 2003;47:127-34.  Back to cited text no. 4
Panchal NJ, Niku S, Imbesi SG. Lymphocytic vasculitis mimicking aggressive multifocal cerebral neoplasm: MR imaging and MR spectroscopic appearance. AJNR Am J Neuroradiol 2005;26:642-5.  Back to cited text no. 5
Molloy ES, Singhal AB, Calabrese LH. Tumour-like mass lesion: An under-recognised presentation of primary angiitis of the central nervous system. Ann Rheum Dis 2008;67:1732-5.  Back to cited text no. 6
You G, Yan W, Zhang W, Li S, Li G, Jiang T. Isolated angiitis of the central nervous system with tumor-like lesion, mimicking brain malignant glioma: A case report and review of the literature. World J Surg Oncol 2011;9:97.  Back to cited text no. 7
de Boysson H, Boulouis G, Dequatre N, Godard S, Néel A, Arquizan C, et al; French Vasculitis Study Group. Tumor-like presentation of primary angiitis of the central nervous system. Stroke 2016;47:2401-4.  Back to cited text no. 8
Kumar PP, Rajesh A, Kandadai RM, Purohit AK, Sundaram C. Primary CNS vasculitis masquerading as glioblastoma: A case report and review. Asian J Neurosurg 2017;12:69-71.  Back to cited text no. 9
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Muccio CF, Tedeschi E, Elefante A, Caranci F, Cerase A. Primary central nervous system vasculitis mimicking a brain tumor on conventional magnetic resonance imaging: The usefulness of perfusion-weighted imaging: A case report. Acta Neurol Belg 2020;120:475-7.  Back to cited text no. 10
Chang HB, Gao M, Zhang JN, Cao WD, Guo SL, Wang P, et al. Retrospective analysis of 28 cases confirmed for primary angiitis of the central nervous system by biopsy. J Stroke Cerebrovasc Dis 2020;29:105400.  Back to cited text no. 11
Murthy JMK, Challa S, Parida S, Murthy TVRK, Basha Paspala SA. Primary angiitis of central nervous system-tumor-like mass lesions: Report of six biopsy confirmed cases. Neurol India 2021;69:451-6.  Back to cited text no. 12
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Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 1988;67:20-39.  Back to cited text no. 15
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]


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