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

  • Diagnostic Neuroradiology
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Abstract

Introduction

Primary angiitis of the central nervous system (PACNS) is an extremely rare vasculitis of unknown etiology. The purpose of this study was to describe the initial and follow-up magnetic resonance (MR) imaging features of the tumor-mimicking PACNS.

Methods

We retrospectively reviewed a total of 21 initial and follow-up brain MR images obtained in four patients with biopsy-proven PACNS mimicking brain tumor on MR images during the periods from 1 to 8.1 years. In the initial study, diffusion-weighted imaging (DWI; n = 4), MR angiogram (n = 4), conventional catheter angiogram (n = 3), perfusion MR (n = 1), and computed tomography (n = 1) and proton MR spectroscopy (MRS; n = 2) were included. The lesions of the brain were qualitatively assessed in terms of location, number, size, shape, signal intensity, absence or presence of hemorrhage, enhancement pattern, and changes on the follow-up studies.

Results

Initially, the lesion manifested as single suprasellar (n = 1) and frontal hemispheric (n = 1) mass and as multiple-enhancing lesions in the unilateral supratentorial hemisphere (n = 2). A patient showed steno-occlusive lesions in the internal carotid and middle cerebral arteries. DWI, perfusion imaging, and MRS revealed inconsistent findings among the patients. On the follow-up studies, a patient had two relapses but there was either significant decrease in size and extent or disappearance of the lesions with immunosuppressive therapy in all patients.

Conclusion

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

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References

  1. Salvarani C, Brown RD Jr, Calamia KT et al (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 62:442–451

    Article  PubMed  Google Scholar 

  2. Greenan TJ, Grossman RI, Goldberg HI (1992) Cerebral vasculitis: MR imaging and angiographic correlation. Radiology 182:65–72

    PubMed  CAS  Google Scholar 

  3. Pomper MG, Miller TJ, Stone JH et al (1999) CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography. AJNR Am J Neuroradiol 20:75–85

    PubMed  CAS  Google Scholar 

  4. Harris KG, Tran DD, Sickels WJ et al (1994) Diagnosing intracranial vasculitis: the roles of MR and angiography. AJNR Am J Neuroradiol 15:317–330

    PubMed  CAS  Google Scholar 

  5. Salvarani C, Brown RD Jr, Calamia KT et al (2008) Primary central nervous system vasculitis with prominent leptomeningeal enhancement: a subset with a benign outcome. Arthritis Rheum 58:595–603

    Article  PubMed  Google Scholar 

  6. Kuker W (2007) Cerebral vasculitis: imaging signs revisited. Neuroradiology 49:471–479

    Article  PubMed  Google Scholar 

  7. Finelli PF, Onyiuke HC, Uphoff DF (1997) Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease. Neurology 49:1696–1699

    PubMed  CAS  Google Scholar 

  8. Ehsan T, Hasan S, Powers JM et al (1995) Serial magnetic resonance imaging in isolated angiitis of the central nervous system. Neurology 45:1462–1465

    PubMed  CAS  Google Scholar 

  9. Ay H, Sahin G, Saatci I et al (2002) Primary angiitis of the central nervous system and silent cortical hemorrhages. AJNR Am J Neuroradiol 23:1561–1563

    PubMed  Google Scholar 

  10. Panchal NJ, Niku S, Imbesi SG (2005) Lymphocytic vasculitis mimicking aggressive multifocal cerebral neoplasm: mr imaging and MR spectroscopic appearance. AJNR Am J Neuroradiol 26:642–645

    PubMed  Google Scholar 

  11. Kristoferitsch W, Jellinger K, Bock F (1984) Cerebral granulomatous angiitis with atypical features. J Neurol 231:38–42

    Article  PubMed  CAS  Google Scholar 

  12. Beppu T, Inoue T, Nishimoto H et al (2007) Primary granulomatous angiitis of the central nervous system: findings of magnetic resonance spectroscopy and fractional anisotropy in diffusion tensor imaging prior to surgery. Case report. J Neurosurg 107:873–877

    Article  PubMed  Google Scholar 

  13. Berger JR, Romano J, Menkin M et al (1995) Benign focal cerebral vasculitis: case report. Neurology 45:1731–1734

    PubMed  CAS  Google Scholar 

  14. Johnson M, Maciunas R, Dutt P et al (1989) Granulomatous angiitis masquerading as a mass lesion. Magnetic resonance imaging and stereotactic biopsy findings in a patient with occult Hodgkin's disease. Surg Neurol 31:49–53

    Article  PubMed  CAS  Google Scholar 

  15. Molloy ES, Singhal AB, Calabrese LH (2008) Tumour-like mass lesion: an under-recognised presentation of primary angiitis of the central nervous system. Ann Rheum Dis 67:1732–1735

    Article  PubMed  CAS  Google Scholar 

  16. Campi A, Benndorf G, Filippi M et al (2001) Primary angiitis of the central nervous system: serial MRI of brain and spinal cord. Neuroradiology 43:599–607

    Article  PubMed  CAS  Google Scholar 

  17. Singh S, John S, Joseph TP et al (2003) Primary angiitis of the central nervous system: MRI features and clinical presentation. Australas Radiol 47:127–134

    Article  PubMed  Google Scholar 

  18. Lie JT (1992) Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol 23:164–171

    Article  PubMed  CAS  Google Scholar 

  19. Aviv RI, Benseler SM, Silverman ED et al (2006) MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol 27:192–199

    PubMed  CAS  Google Scholar 

  20. Calabrese LH, Furlan AJ, Gragg LA et al (1992) Primary angiitis of the central nervous system: diagnostic criteria and clinical approach. Cleve Clin J Med 59:293–306

    PubMed  CAS  Google Scholar 

  21. Yuh WT, Ueda T, Maley JE (1999) Perfusion and diffusion imaging: a potential tool for improved diagnosis of CNS vasculitis. AJNR Am J Neuroradiol 20:87–89

    PubMed  CAS  Google Scholar 

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We declare that we have no conflict of interest.

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Correspondence to Ji-hoon Kim.

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Lee, Y., Kim, Jh., Kim, E. et al. Tumor-mimicking primary angiitis of the central nervous system: initial and follow-up MR features. Neuroradiology 51, 651–659 (2009). https://doi.org/10.1007/s00234-009-0546-3

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  • DOI: https://doi.org/10.1007/s00234-009-0546-3

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