Long-Term Progression of a Residual Cerebral Amyloidoma: An Illustrative Case and Systematic Review

Abstract

Cerebral amyloidomas (CA) are exceptionally rare tumor-like lesions composed of cerebral amyloid-beta, which is derived from the cleavage of the amyloid precursor protein. We presented a case of recurrent CA and performed a systematic review, comparing their initial presentation, imaging features, neurosurgical treatment, and natural history of the disease. A 65-year-old male with a history of right homonymous hemianopsia, who underwent subtotal resection of a CA 19 years before, presents to the emergency department with right hemiparesis, dysarthria, and a new onset of clonic seizures. Imaging revealed a left parieto-occipital lesion with calcifications and vasogenic edema. A gross-total resection was performed. Histopathology revealed a hypocellular eosinophilic lesion consistent with CA. Postoperatively, the patient recovered without new neurological deficits. One-year follow-up magnetic resonance imaging showed no residual or recurrence lesion. Eighty-seven cases, including ours, revealed that 65.5% (n= 57) were females with a median age of 54 years (IQR: 46-62). Most lesions were solitary (82.7%; 72 of 87 lesions). Frontal and parietal lobes were most commonly affected with 32.9% (n= 28) and 30.5% (n= 26), respectively. Seizures were the most common symptom followed by visual compromise. Calcifications were present in 19.5% (n= 17) of the lesions. This systematic review provides insights into the epidemiological, clinical, and neurosurgical characteristics, as well as the long-term prognosis of CA. This marks the first case in the reviewed literature with a 19-year period of follow-up where the patient had reoperation due to disease progression.