TY  - JOUR
A1  - Löhr, Mario
A1  - Kessler, Almuth F.
A1  - Monoranu, Camelia-Maria
A1  - Grosche, Jens
A1  - Linsenmann, Thomas
A1  - Ernestus, Ralf-Ingo
A1  - Härtig, Wolfgang
T1  - Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report
JF  - BMC Neurology
N2  - 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.
KW  - amyloidoma
KW  - neurooncology
KW  - brain tumor
KW  - neurodegenerative disease
KW  - neurovascular unit
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-200341
VL  - 19
ER  -