Abstract #9

by | Sep 19, 2022 | 2022 Abstracts

Abstract 9- 0915-0930
Category: Clinical

At the end of the session,
participants will be able to:

  1. Gaining insight into the diagnostic challenges of a rare neuropathological condition from clinic and imaging to brain biopsy and autopsy.
  2. Defining the pathological findings of cerebral amyloidoma over the course of the disease.
Presenter

Shervin Pejhan is a first year Neuropathology resident at Western University.

Authors

Shervin Pejhan 1, Adrian Budhram 2, Joseph Megyesi 2, Lee Cyn Ang 1,2,
Robert Hammond 1,2, Qi Zhang 1,2

1 Department of Pathology & Lab Medicine, University of Western Ontario, ON, Canada

2 Department of Clinical Neurological Sciences, University of Western Ontario, ON, Canada

Target Audience:

Pathologists, Residents, Medical Students, Medical Laboratory technologists, Graduate students

CanMEDS:

Medical Expert (the integrating role), Communicator, Collaborator, Scholar, Professional

Cerebral amyloidoma: pathological findings from diagnostic biopsy to autopsy

Abstract

Amyloidoma is a rare condition defined by the aggregation of amyloid proteins forming a mass-like lesion. While brain-restricted amyloidomas have been reported, their course and pathogenesis are poorly understood.

We are reporting a case of multifocal brain amyloidoma in a 71-year-old man, which is significant for its protracted clinical course and progression of neurological symptoms. A pair of stereotactic biopsies revealed the underlying pathology.

The pathological findings were unusual with a dual picture of perivascular lymphoplasmacytic infiltration in the frontal area, accompanied by amyloid deposits in occipital white matter. Proteomic analysis of the amyloid protein by mass spectrometry was also curious as it characterised both lambda light chain and amyloid beta-protein without evidence of monoclonality. Further assessments found no involvement of other organ systems. With the progression of symptoms and lack of definitive treatment, the patient underwent palliative whole-brain irradiation, with no improvement, and passed away shortly thereafter.

Assessment of post-mortem brain tissue showed massive amyloid deposition in the periventricular white matter with sparse perivascular lymphocytes and fewer plasma cells than the biopsy samples. Special staining, immunohistochemistry, electron microscopy, and mass spectrometry confirmed multifocal cerebral amyloidoma, lambda light chain type. The collection of the biopsy and autopsy findings may be the result of lesions observed at different stages of progression, treatment effects, or a not-previously described pattern of brain amyloidoma.

Studying this case is beneficial to gaining insight into the pathobiology of this rare condition and to inform future studies and therapeutic interventions.