Cerebral AL λ-amyloidoma: Clinical and pathomorphological characteristics. Review of the literature and of a patient

Abstract

Complete surgical removal of cerebral AL amyloidoma seems to be the only way to prevent progression or recurrence of such a brain lesion.

Cerebral immunoglobulin light chain amyloid angiopathy-related hemorrhages

“…In our patient, we failed to demonstrate systemic AL amyloidosis, B-cell or plasma cell dyscrasia or detectable circulating monoclonal protein suggesting that AL amyloid deposition was localized in the brain. Unlike previously reported patients with localized cerebral amyloidoma, our patient had not a tumor-like presentation but had recurrent ICH (Fischer et al, 2007). In a few patients suffering ICH, the co-localization of A-beta and Ig-lambda light chain AL in the walls of cerebral blood vessels has been reported (Galuske et al, 2004).…”

“…Hemorrhages secondary to Ig amyloid deposition in the vascular wall may be a rare clinical presentation of systemic AL amyloidosis (Spier et al, 2008). Localized cerebral AL amyloidoma are extremely rare with usually a tumor-like presentation (Fischer et al, 2007).…”

The role of advanced MR methods in the diagnosis of cerebral amyloidoma

“…Diagnosis is usually established by brain biopsy and histopathological examination. Tumorlike amyloid formation within the brain has been described in a total of 27 cases so far [2].…”

“…This major subunit protein in all primary amyloidomas is derived from immunoglobulin light chain (AL-l subtype) [1]. Tumor-like amyloid formation within the brain has been described in 27 cases so far [2]. In several reports the neuroimaging aspects of this condition [3,4] had been described.…”

“…In several reports the neuroimaging aspects of this condition [3,4] had been described. Amyloidoma can mimic other brain space occuping lesions in terms of neuroimaging and clinical neurological evaluation [2]. Advanced MR studies including MRS and diffusion were described in three case reports.…”