Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report
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 cons...
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| Format: | Article |
| Language: | English |
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BMC
2019-04-01
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| Series: | BMC Neurology |
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| Online Access: | http://link.springer.com/article/10.1186/s12883-019-1274-x |
| _version_ | 1828474729410854912 |
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| author | Mario Löhr Almuth F. Kessler Camelia-Maria Monoranu Jens Grosche Thomas Linsenmann Ralf-Ingo Ernestus Wolfgang Härtig |
| author_facet | Mario Löhr Almuth F. Kessler Camelia-Maria Monoranu Jens Grosche Thomas Linsenmann Ralf-Ingo Ernestus Wolfgang Härtig |
| author_sort | Mario Löhr |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-11T06:03:31Z |
| format | Article |
| id | doaj.art-a2210cf4cba0425cb1276b7ce0ec9d4a |
| institution | Directory Open Access Journal |
| issn | 1471-2377 |
| language | English |
| last_indexed | 2024-12-11T06:03:31Z |
| publishDate | 2019-04-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj.art-a2210cf4cba0425cb1276b7ce0ec9d4a2022-12-22T01:18:22ZengBMCBMC Neurology1471-23772019-04-011911510.1186/s12883-019-1274-xPrimary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case reportMario Löhr0Almuth F. Kessler1Camelia-Maria Monoranu2Jens Grosche3Thomas Linsenmann4Ralf-Ingo Ernestus5Wolfgang Härtig6Department of Neurosurgery, University Hospital of WuerzburgDepartment of Neurosurgery, University Hospital of WuerzburgDepartment of Neuropathology, Institute of Pathology, University of WuerzburgPaul Flechsig Institute for Brain Research, University of LeipzigDepartment of Neurosurgery, University Hospital of WuerzburgDepartment of Neurosurgery, University Hospital of WuerzburgPaul Flechsig Institute for Brain Research, University of LeipzigAbstract 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.http://link.springer.com/article/10.1186/s12883-019-1274-xAmyloidomaNeurooncologyBrain tumorNeurodegenerative diseaseNeurovascular unit |
| spellingShingle | Mario Löhr Almuth F. Kessler Camelia-Maria Monoranu Jens Grosche Thomas Linsenmann Ralf-Ingo Ernestus Wolfgang Härtig Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report BMC Neurology Amyloidoma Neurooncology Brain tumor Neurodegenerative disease Neurovascular unit |
| title | Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report |
| title_full | Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report |
| title_fullStr | Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report |
| title_full_unstemmed | Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report |
| title_short | Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report |
| title_sort | primary brain amyloidoma both a neoplastic and a neurodegenerative disease a case report |
| topic | Amyloidoma Neurooncology Brain tumor Neurodegenerative disease Neurovascular unit |
| url | http://link.springer.com/article/10.1186/s12883-019-1274-x |
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