Article
Cerebral amyloidoma – intraoperative findings and review of the literature
Zerebrales Amyloidom – intraoperativer Befund und Literaturrecherche
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Published: | June 4, 2021 |
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Objective: Primary cerebral amyloidomas represent a benign but rare condition with about thirty published cases. Most published studies focus on radiological and histological characteristics. Here, we present the case of a 66-year old woman with an infiltrative process of the parietal lobe who underwent an open biopsy and show intraoperative findings.
Methods: Case Report and Literature Review
Results: Cerebral amyloidomas are a subtype of marginal cell lymphomas or lymphomas of the B-cell line, characterized by deposits of amyloid light chains. They mostly have a subcortical location and have been associated with neurodegenerative processes in the past. A review of the literature shows that most cases undergo a stable clinical course, but represent a certain risk for intracerebral hemorrhaging.
A 66-year old female presented initially with a pulsating noise in her left ear. Besides a hysterectomy for multiple myomas at an earlier age and an autoimmune thyroiditis, there were no other preexistent conditions. MRI showed a diffuse process of the left parietal lobe with moderate contrast enhancement and signal enhancement in the T2-Flair-sequences. Upon biopsy, the intraoperative aspect was one of a well vascularized process consisting out of diffusely spread follicular structures throughout the white matter, reaching to the left ventricle. Histology showed a massive protein agglutination among lymphocyte infiltrates and k- light- chain- mRNA- restriction, consistent with the diagnosis of amyloidoma.
Conclusion: Cerebral amyloidomas are easily misdiagnosed as neoplastic processes. A better knowledge of the characteristics of this entity is necessary to guide specific therapies.