eISSN: 1509-572x
ISSN: 1641-4640
Folia Neuropathologica
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2/2021
vol. 59
 
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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

1.
Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
2.
Department of Pathology, University Medical Centre Maribor, Maribor, Slovenia
3.
Department of Neurosurgery, University Medical Centre Maribor, Maribor, Slovenia
Folia Neuropathol 2021; 59 (2): 205-211
Online publish date: 2021/06/30
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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:

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

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