Long-term control of primary cerebral ALH amyloidoma with focal radiation therapy

ABSTRACT Primary cerebral amyloidoma is an exceedingly rare condition that refers to localized tumor-like deposition of amyloid in the brain without evidence of an underlying systemic process such as multiple myeloma. Reported cases of primary cerebral amyloidoma to date have described restricted light-chain deposition (AL amyloid); we report two cases of primary cerebral amyloidoma with additional heavy-chain deposition (ALH amyloid). Overall, when localized disease is present, therapy such as surgery or radiation therapy is considered to limit the risk of progression. However, in part due to the rarity of the condition, defining optimal treatment has been problematic. Here we discuss definitive management of ALH primary cerebral amyloidoma treated with focal radiation therapy.