Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report
Please always quote using this URN: urn:nbn:de:bvb:20-opus-200341
- 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,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.…
Author: | Mario Löhr, Almuth F. Kessler, Camelia-Maria Monoranu, Jens Grosche, Thomas Linsenmann, Ralf-Ingo Ernestus, Wolfgang Härtig |
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URN: | urn:nbn:de:bvb:20-opus-200341 |
Document Type: | Journal article |
Faculties: | Medizinische Fakultät / Neurochirurgische Klinik und Poliklinik |
Medizinische Fakultät / Pathologisches Institut | |
Language: | English |
Parent Title (English): | BMC Neurology |
Year of Completion: | 2019 |
Volume: | 19 |
Pagenumber: | 59 |
Source: | BMC Neurology (2019) 19:59 https://doi.org/10.1186/s12883-019-1274-x |
DOI: | https://doi.org/10.1186/s12883-019-1274-x |
Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
Tag: | amyloidoma; brain tumor; neurodegenerative disease; neurooncology; neurovascular unit |
Release Date: | 2020/03/11 |
Collections: | Open-Access-Publikationsfonds / Förderzeitraum 2019 |
Licence (German): | CC BY: Creative-Commons-Lizenz: Namensnennung 4.0 International |