Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report

BMC Neurol. 2019 Apr 10;19(1):59. doi: 10.1186/s12883-019-1274-x.

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.

Keywords: Amyloidoma; Brain tumor; Neurodegenerative disease; Neurooncology; Neurovascular unit.

Publication types

  • Case Reports

MeSH terms

  • Amyloidosis / pathology*
  • Brain Diseases / pathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Middle Aged
  • Neurodegenerative Diseases / etiology*
  • Neurodegenerative Diseases / pathology*