Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature

Alenka Matjašič; Karmen Wechtersbach; Rajko Kavalar; Matjaž Voršič; Jernej Mlakar; Janez Ravnik; Mara Popović

doi:10.5114/fn.2021.106405

eISSN: 1509-572x
ISSN: 1641-4640

Folia Neuropathologica

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abstract:

Case report

Brain aggregoma with clonal B-cell perivascular proliferation detected by next-generation sequencing. A case report and review of the literature

Alenka Matjašič

¹

,

Karmen Wechtersbach

¹

,

Rajko Kavalar

²

,

Matjaž Voršič

³

,

Jernej Mlakar

¹

,

Janez Ravnik

³

,

Mara Popović

¹

Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Department of Pathology, University Medical Centre Maribor, Maribor, Slovenia
Department of Neurosurgery, University Medical Centre Maribor, Maribor, Slovenia

Folia Neuropathol 2021; 59 (2): 205-211

DOI: https://doi.org/10.5114/fn.2021.106405

Online publish date: 2021/06/30

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AMA

Matjašič A, Wechtersbach K, Kavalar R, et al. Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature. Folia Neuropathologica. 2021;59(2):205-211. doi:10.5114/fn.2021.106405.

APA

Matjašič, A., Wechtersbach, K., Kavalar, R., Voršič, M., Mlakar, J.,  & Ravnik, J. et al. (2021). Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature. Folia Neuropathologica, 59(2), 205-211. https://doi.org/10.5114/fn.2021.106405

Chicago

Matjašič, Alenka, Karmen Wechtersbach, Rajko Kavalar, Matjaž Voršič, Jernej Mlakar, Janez Ravnik,  and Mara Popović. 2021. "Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature". Folia Neuropathologica 59 (2): 205-211. doi:10.5114/fn.2021.106405.

Harvard

Matjašič, A., Wechtersbach, K., Kavalar, R., Voršič, M., Mlakar, J., Ravnik, J.,  and Popović, M. (2021). Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature. Folia Neuropathologica, 59(2), pp.205-211. https://doi.org/10.5114/fn.2021.106405

MLA

Matjašič, Alenka et al. "Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature." Folia Neuropathologica, vol. 59, no. 2, 2021, pp. 205-211. doi:10.5114/fn.2021.106405.

Vancouver

Matjašič A, Wechtersbach K, Kavalar R, Voršič M, Mlakar J, Ravnik J et al. Brain aggregoma with clonal B-cell perivascular proliferation
detected by next-generation sequencing. A case report
and review of the literature. Folia Neuropathologica. 2021;59(2):205-211. doi:10.5114/fn.2021.106405.

PlumX metrics:

Light-chain deposition disease (LCDD), a rare type of monoclonal immunoglobulin deposition disease, can be presented as systemic or localized, very rarely affecting central nervous system (CNS). Only 10 cases of CNS-LCDD have been described so far. We present an eleventh case of cerebral tumour-like LCDD, called aggregoma, and compare it with previously reported cases. A 49-year-old patient was admitted to the hospital due to a first generalized epileptic seizure. Magnetic resonance imaging (MRI) showed focal lesion in the right occipital lobe. Abundant parenchymal aggregates of pale eosinophilic material were observed, Congo red negative, Thioflavin T moderately positive, and l-light chain positive, but k negative in immunofluorescence with mild perivascular lymphoplasmacytic infiltrates in the intervening brain tissue. Clonality testing by next-generation sequencing showed the monoclonal nature of B-lymphocytes. Electron microscopy showed a finely granular ultrastructure of the aggregates without deposition in the vessel walls. A whole-body workup did not show any extra-cerebral immune dyscrasias.

keywords:

Brain aggregoma with clonal B-cell perivascular proliferation detected by next-generation sequencing. A case report and review of the literature

Alenka Matjašič 1 , Karmen Wechtersbach 1 , Rajko Kavalar 2 , Matjaž Voršič 3 , Jernej Mlakar 1 , Janez Ravnik 3 , Mara Popović 1

aggregoma, CNS-LCDD, immunoglobulin light chains, next-generation sequencing, paraproteinaemia

Alenka Matjašič

¹

,

Karmen Wechtersbach

¹

,

Rajko Kavalar

²

,

Matjaž Voršič

³

,

Jernej Mlakar

¹

,

Janez Ravnik

³

,

Mara Popović

¹