A Rare Case of Cerebral Amyloidoma Mimicking a Hemorrhagic Malignant Central Nervous System Neoplasm

Abstract

Cerebral amyloidoma is a rare form of amyloidosis with a localized tumor like an amyloid deposition in the brain composed of insoluble fibrillary protein with cross beta-sheet conformation. Its usual presentation includes vision loss, seizures, behavioral changes, cognitive decline, and recurrent headaches. It has a benign course with a slow progression, and it is not associated with dissemination. We report a case of a 65-year-old Caucasian woman who presented with symptoms of progressively worsening cognitive dysfunction of six months' duration. From CT of the brain, it was found that she had a right frontal and left parietal hemorrhagic mass with a large amount of vasogenic edema and a midline shift. MRI showed heterogeneously enhancing hemorrhagic mass of 5.2 cm x 2.6 cm x 3.6 cm in size, with a satellite lesion. Initially, this was suspected to be a high-grade glioma vs. metastatic hemorrhagic lesions. She underwent stereotactic biopsy of the mass, and histopathology was consistent with cerebral amyloidoma with marked IgA lambda plasma cell differentiation. She did not have any evidence of systemic amyloidosis, and therefore, she is being clinically observed with a regular follow-up and annual CT surveillance. She has remained stable over the past two years, although she has residual cognitive dysfunction. Cerebral amyloidoma can mimic malignant central nervous system (CNS) neoplasms and should be considered as a differential of any single or multiple mass lesions occurring in the white matter region of the brain with a characteristic appearance of "hyperdense lesions" on CT. It is a benign disease with no metastatic potential that usually resolves entirely after resection.

Primary cerebral immunoglobulin light chain amyloidoma in a patient with multiple sclerosis

“…One case of a patient with a follicular lymphoma with plasmacytic differentiation was associated with AL amyloidosis located in the ileum 10. In some articles, a lymphoid neoplasm was mentioned as underlying cause of the cerebral amyloidoma 6 11–16. However, to diagnose, an underlying lymphoid neoplasm the infiltrate must have been typed.…”

“…However, to diagnose, an underlying lymphoid neoplasm the infiltrate must have been typed. Only a few articles performed immunohistochemistry on the infiltrate with demonstration of B-cell clonality 14–16. The article of Heß et al describes seven cases with cerebral amyloidoma and B-cell clonality.…”

Amyloids and brain cancer: molecular linkages and crossovers

“…This classic example of primary cerebral amyloidosis characterized by localized tumor-like amyloid deposition was later called as primary brain amyloidoma. These amyloidic deposits of glycoproteins remain benign; however they may mimic malignant brain tumors as evident in a recently reported case in a 65-year-old Caucasian woman [ 92 ]. Primary brain amyloidoma is a rare tumor-like lesion, which consists of deposition of amyloid within the brain parenchyma without evidence for systemic amyloidosis.…”