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Primary Brain Amyloidoma, Both a Neoplastic and a Neurodegenerative Disease: A Case Report

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source: BMC Neurology

year: 2019

authors: M. Löhr, A.F. Kessler, C. Monoranu, J. Grosche, T. Linsenmann, R. Ernestus, W. Härtig

summary/abstract:

Background:
Scattered extracellular deposits of amyloid within the brain parenchyma can be found in a heterogeneous group of diseases. Its condensed accumulation in the white matter without evidence for systemic amyloidosis is known as primary brain amyloidoma (PBA). Although originally considered as a tumor-like lesion by its space-occupying effect, this condition displays also common hallmarks of a neurodegenerative disorder.
 
Case Presentation:
A 50-year-old woman presented with a mild cognitive decline and seizures with a right temporal, irregular and contrast-enhancing mass on magnetic resonance imaging. Suspecting a high-grade glioma, the firm tumor was subtotally resected. Neuropathological examination showed no glioma, but distinct features of a neurodegenerative disorder. The lesion was composed of amyloid AL λ aggregating within the brain parenchyma as well as the adjacent vessels, partially obstructing the vascular lumina. Immunostaining confirmed a distinct perivascular inflammatory reaction. After removal of the PBA, mnestic impairments improved considerably, the clinical course and MRI-results are stable in the 8-year follow-up.
 
Conclusion:
Based on our histopathological findings, we propose to regard the clinicopathological entity of PBA as an overlap between a neoplastic and neurodegenerative disorder. Since the lesions are locally restricted, they might be amenable to surgery with the prospect of a definite cure.
 
organization: University Hospital of Wuerzburg, Germany; University of Wuerzburg, Germany; University of Leipzig, Germany

DOI: 10.1186/s12883-019-1274-x

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