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

Mario Löhr1, Almuth F Kessler2, Camelia-Maria Monoranu3

  • 1Department of Neurosurgery, University Hospital of Wuerzburg, Josef-Schneider-Str. 11, 97080, Würzburg, Germany. loehr_m1@ukw.de.

BMC Neurology
|April 12, 2019
PubMed
Abstract

Insights

Primary brain amyloidoma (PBA) is a rare condition involving amyloid buildup in the brain. Surgical removal of PBA can lead to significant clinical improvement, suggesting a potential cure.

Area of Science:

  • Neuropathology
  • Neurodegenerative Diseases
  • Neurosurgery

Background:

  • Primary brain amyloidoma (PBA) involves localized amyloid deposits in the brain's white matter.
  • PBA presents with tumor-like mass effects but shares features with neurodegenerative disorders.

Observation:

  • A 50-year-old woman with cognitive decline and seizures had a temporal lobe mass.
  • Resection revealed amyloid AL λ deposits and perivascular inflammation, not glioma.
  • Post-surgery, her cognitive impairments improved, with stable condition over 8 years.

Findings:

  • Histopathology confirmed amyloid aggregation within brain parenchyma and vessels.
  • The lesion exhibited characteristics of both neoplastic and neurodegenerative processes.
  • Amyloid deposits partially obstructed vascular lumina, suggesting a vascular component.

Implications:

  • PBA may represent an overlap between neoplastic and neurodegenerative conditions.
  • Surgical resection of localized PBA lesions offers a potential for cure.
  • This case highlights the importance of considering PBA in differential diagnoses of brain masses.
Keywords:
AmyloidomaBrain tumorNeurodegenerative diseaseNeurooncologyNeurovascular unit

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