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Amyloid Fibrils

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...

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Cerebral amyloidomas: Perspective on unusual morphological features.

Bette K Kleinschmidt-DeMasters

Clinical Neuropathology

|December 27, 2021

Summary

Cerebral amyloidomas (CAs) are rare brain lesions. This study highlights unusual features in three CA cases, including mineralization, giant cell reactions, and lymphoma, emphasizing diverse presentations of λ light chain amyloidosis.

Area of Science:

Neuropathology
Immunopathology
Oncology

Background:

Cerebral amyloidomas (CAs) are mass-forming lesions characterized by congophilic amyloid deposits, typically involving lambda (λ) light chains.
Distinguishing CAs from light chain deposition disease (LCDD) is crucial, as LCDD often involves kappa (κ) light chains and shows non-polarizable deposits.

Observation:

Three recent CA cases with atypical morphological findings were analyzed.
Case 1: CA with congophilic, polarizable deposits, dystrophic mineralization, and plasma cells.
Case 2: CA exhibiting multinucleated giant cell reaction and suspicion of marginal zone B-cell lymphoma.
Case 3: Autopsy-identified CA with nodular/concentric amyloid, perivascular plasma cell cuffs, and erythematous lesions.

Findings:

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Mass spectrometry confirmed λ light and alpha (α) heavy chain amyloid deposits in all three cases.

The cases demonstrated unusual gross and microscopic features not typically associated with CAs.

Histological findings included prominent dystrophic mineralization, significant giant cell response, and co-occurrence with B-cell lymphoma.

Implications:

These findings expand the understanding of the morphological spectrum of cerebral amyloidomas.
Recognizing these unusual features is vital for accurate diagnosis and appropriate patient management.
The study underscores the importance of detailed histopathological examination and advanced techniques like mass spectrometry in diagnosing complex amyloidosis cases.