title: Vemurafenib (BRAF Inhibitor) Therapy for Orbital Erdheim-Chester Disease.
abstract: Erdheim-Chester disease is a rare xanthogranulomatous systemic disease, which involves the orbit in some cases. Through this case report, the authors review the treatment modalities used in orbital Erdheim-Chester disease and explore a newer modality of treatment. Cases of orbital Erdheim-Chester disease were identified in the literature utilizing a PubMed search and all the treatment modalities were reviewed. The response to treatment of orbital Erdheim-Chester disease has been poor with the various medical and surgical treatment modalities used in the past. The authors report the use of BRAF inhibitor with a remarkable response in our case.
authors.affiliation: *Stein Eye Institute, Division of Orbital and Oculoplastic Surgery, University of California, Los Angeles, and †Doheny Eye Institute, Los Angeles, California, U.S.A.
authors.forename: Adit
authors.initials: A
authors.lastname: Gupta
authors.forename: Amir
authors.initials: A
authors.lastname: Yeganeh
authors.forename: Daniel
authors.initials: D
authors.lastname: Rootman
authors.forename: Robert
authors.initials: R
authors.lastname: Goldberg
mesh_terms: D001706:Biopsy; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D004791:Enzyme Inhibitors / Q000008:administration & dosage; D031249:Erdheim-Chester Disease / Q000175:diagnosis / Q000188:drug therapy*; D005500:Follow-Up Studies; D006801:Humans; D007211:Indoles / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D009915:Orbit / Q000000981:diagnostic imaging; D009916:Orbital Diseases / Q000175:diagnosis / Q000188:drug therapy*; D048493:Proto-Oncogene Proteins B-raf / Q000037:antagonists & inhibitors*; D035583:Rare Diseases; D013449:Sulfonamides / Q000008:administration & dosage*; D014057:Tomography, X-Ray Computed; D000077484:Vemurafenib
chemical_list: D004791:Enzyme Inhibitors; D007211:Indoles; D013449:Sulfonamides; D000077484:Vemurafenib; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf