P20.19.A RARE CASE OF PRIMARY BRAIN AMYLOIDOMA

Abstract BACKGROUND Cerebral amyloidoma, composed of insoluble fibrillary proteins, is one of the rarest forms of amyloid deposition. It resembles a tumor, with a variable clinical presentation that can include vision loss, behavioral changes, cognitive issues, and recurrent headaches. It has a slow...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Neuro-oncology (Charlottesville, Va.) Va.), 2024-10, Vol.26 (Supplement_5), p.v120-v120
Hauptverfasser: Ivan, R, Signate, A, Puget, S, Sirbu, C A
Format: Artikel
Sprache:eng
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
Beschreibung
Zusammenfassung:Abstract BACKGROUND Cerebral amyloidoma, composed of insoluble fibrillary proteins, is one of the rarest forms of amyloid deposition. It resembles a tumor, with a variable clinical presentation that can include vision loss, behavioral changes, cognitive issues, and recurrent headaches. It has a slow evolution pattern, without characteristics of dissemination. MATERIAL AND METHODS We present the case of a 56-year-old patient, who presented with a sensory loss on the right side of the body, progressive memory troubles of one-year duration, and later a left homonym hemianopsia. She underwent a cerebral MRI that showed a right parietal-temporal heterogenous lesion, with contrast enhancement as well as a right parieto-occipital smaller lesion, with a differential diagnostic of glial tumor or cerebral lymphoma. A stereotactic biopsy of the mass was made by the neurosurgeon with a subsequent anatomopathological examination. RESULTS The histopathological result was consistent with a cerebral amyloidoma AL with lambda chains, without an associated lymphatic lesion. The systemic extension was also evaluated, without signs of systemic amyloidosis (salivary gland biopsy, thoracic abdominal pelvic scan, myelogram, and a urinary analysis). Hematological and ophthalmological consults were also made. CONCLUSION Given the age and the progressive clinical course, the treatment proposed was with Revlimid and Dexamethasone, along with a periodical surveillance with cerebral imaging and neurological consultations. The evolution remained stable at the moment of the publication of this report.
ISSN:1522-8517
1523-5866
DOI:10.1093/neuonc/noae144.406