Dabrafenib and steroids for the treatment of Erdheim-Chester disease with extensive CNS involvement: a case report

Author:

Yuen Carlen A1ORCID,Bao Silin2,Aung Mya Sandi3,Shishodia Rhea3,Kong Xiao-Tang1

Affiliation:

1. Department of Neuro-Oncology, University of California, Irvine, CA 92868-3201, USA

2. Department of Internal Medicine. Community Regional Medical Center, Fresno, CA 93721, USA

3. California Health Sciences University, College of Osteopathic Medicine, Clovis, CA 93611, USA

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 BRAF V600E-positive ECD treated successfully with steroids followed by single-agent dabrafenib.

Publisher

Future Medicine Ltd

Subject

Pharmacology,Molecular Medicine,General Medicine

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