Primary Central Nervous System Angiitis Mimicking a Space-Occupying Lesion

Abstract

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.

Keywords

primary CNS angiitis - craniotomy - corticosteroids - cyclophosphamide

Ethical Approval Statement

This study has been approved by institutional review board, Christian Medical College, Vellore, India.

Authors' Contribution

K.P.R. contributed in the concept and design of study; E.J.G., B.P., and V.M. in data collection and analysis; E.J.G. and V.M. in manuscript preparation; K.P.R. and V.M. in the critical revision of manuscript; and K.P.R. as guarantor.

Publikationsverlauf

Artikel online veröffentlicht:
16. Mai 2022

• References

• 1 Gan C, Maingard J, Giles L, Phal PM, Tan KM. Primary angiitis of the central nervous system presenting as a mass lesion. J Clin Neurosci 2015; 22 (09) 1528-1531
• 2 Omuro AM, Leite CC, Mokhtari K, Delattre JY. Pitfalls in the diagnosis of brain tumours. Lancet Neurol 2006; 5 (11) 937-948
• 3 Cunliffe CH, Fischer I, Monoky D. et al. Intracranial lesions mimicking neoplasms. Arch Pathol Lab Med 2009; 133 (01) 101-123
• 4 Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009; 66 (06) 704-709
• 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 (12) 1732-1735
• 6 Killeen T, Jucker D, Went P. et al. Solitary tumour-like mass lesions of the central nervous system: Primary angiitis of the CNS and inflammatory pseudotumour. Clin Neurol Neurosurg 2015; 135: 34-37
• 7 Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol 2011; 10 (06) 561-572
• 8 Geri G, Saadoun D, Guillevin R. et al. Central nervous system angiitis: a series of 31 patients. Clin Rheumatol 2014; 33 (01) 105-110
• 9 Giannini C, Salvarani C, Hunder G, Brown RD. Primary central nervous system vasculitis: pathology and mechanisms. Acta Neuropathol 2012; 123 (06) 759-772
• 10 Singh S, Soloman T, Chacko G, Joseph TP. Primary angiitis of the central nervous system: an ante-mortem diagnosis. J Postgrad Med 2000; 46 (04) 272-274
• 11 Singh S, John S, Joseph TP, Soloman T. Primary angiitis of the central nervous system: MRI features and clinical presentation. Australas Radiol 2003; 47 (02) 127-134
• 12 Lee Y, Kim JH, Kim E. et al. Tumor-mimicking primary angiitis of the central nervous system: initial and follow-up MR features. Neuroradiology 2009; 51 (10) 651-659
• 13 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 (01) 20-39
• 14 Alrawi A, Trobe JD, Blaivas M, Musch DC. Brain biopsy in primary angiitis of the central nervous system. Neurology 1999; 53 (04) 858-860
• 15 Sundaram S, Menon D, Khatri P. et al. Primary angiitis of the central nervous system: Clinical profiles and outcomes of 45 patients. Neurol India 2019; 67 (01) 105-112
• 16 Suri V, Kakkar A, Sharma MC, Padma MV, Garg A, Sarkar C. Primary angiitis of the central nervous system: a study of histopathological patterns and review of the literature. Folia Neuropathol 2014; 52 (02) 187-196
• 17 Salvarani C, Brown Jr RD, Christianson T. et al. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients. Medicine (Baltimore) 2015; 94 (21) e738
• 18 Boulouis G, de Boysson H, Zuber M. et al; French Vasculitis Group. Primary angiitis of the central nervous system: magnetic resonance imaging spectrum of parenchymal, meningeal, and vascular lesions at baseline. Stroke 2017; 48 (05) 1248-1255
• 19 Powers WJ. Primary angiitis of the central nervous system: diagnostic criteria. Neurol Clin 2015; 33 (02) 515-526
• 20 Miller DV, Salvarani C, Hunder GG. et al. Biopsy findings in primary angiitis of the central nervous system. Am J Surg Pathol 2009; 33 (01) 35-43
• 21 Elbers J, Halliday W, Hawkins C, Hutchinson C, Benseler SM. Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol 2010; 68 (05) 602-610
• 22 Salvarani C, Brown Jr RD, Christianson TJH, Huston III J, Giannini C, Hunder GG. Long-term remission, relapses and maintenance therapy in adult primary central nervous system vasculitis: A single-center 35-year experience. Autoimmun Rev 2020; 19 (04) 102497