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Biom-03. Dabrafenib and Steroids for the Treatment of Erdheim-chester Disease With Extensive Central Nervous System Involvement

Year: 2024

Type: conference-abstract

Abstract: Erdheim-Chester disease (ECD) is an exceedingly rare non–Langerhans cell CD68+ CD1a- S100- histiocytic multi-organ disease. Diagnosis of ECD is often delayed due to non-specific radiographic findings and heterogeneous lesional tissue. Increasingly, the role of genomic alterations is being recognized for both diagnosis and treatment of ECD. More than half of ECD patients harbor the BRAFV600E mutation. Evaluation for this mutation be can falsely negative on immunohistochemical staining and confirmation with molecular analyses is recommended. We present a case of the 44 year-old male with BRAFV600E-positive ECD treated successfully with steroids followed by single-agent dabrafenib.

Source: Neuro-Oncology

DOI: 10.1093/neuonc/noae165.0076

Authors: Carlen Yuen, Silin Bao, Mya Sandi Aung, Rhea Shishodia, Xiao-Tang Kong

Institutions: University of California, Irvine, Community Regional Medical Center, California Health Sciences University

Language: English

Topic: Histiocytic Disorders and Treatments

Subfield: Physiology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

OpenAlex ID: W4404237252

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