Journal Basic Info
- Impact Factor: 1.995**
- H-Index: 8
- ISSN: 2474-1647
- DOI: 10.25107/2474-1647
Major Scope
- Pediatric Surgery
- Gynecological Surgery
- Plastic Surgery
- Vascular Surgery
- Thoracic Surgery
- Surgical Oncology
- Ophthalmic Surgery
- Gastroenterological Surgery
Abstract
Citation: Clin Surg. 2016;1(1):1230.Case Report | Open Access
Sustained Efficacy of Targeted Therapy with Vemurafenib in BRAF(V600e) - Mutated Erdheim - Chester Disease
Kimberly D. Tran, Juan Ayala-Haedo, Oliver Fischer, Sander R. Dubovy and Sara T. Wester
Department of Ophthalmology, University of Miami, USA
*Correspondance to: Sara T. Wester
PDF Full Text DOI: 10.25107/2474-1647.1230
Abstract
The authors report the case of a 45-year-old African American female with complex history of pituitary mass, diabetes insipidus, anasarca, congestive heart failure, right atrial mass, cardiac tamponade, pericardial effusions, retroperitoneal fibrosis, and lower extremity arthralgia, who presented for evaluation of bilateral exophthalmos and intraconal orbital masses. Orbital biopsy revealed histiocytic infiltrate with immunohistochemical staining positively for CD 68, and negatively for S100 and CD1a, as well as genetic testing positive for the proto-oncogene B-Raf protein (BRAFV600E) mutation. The presentation was suggestive of Erdheim-Chester Disease, and the patient was treated with Vemurafenib with significant sustained clinical improvement.
Keywords
Cite the article
Tran KD, Ayala-Haedo J, Fischer O, Dubovy SR, Wester ST. Sustained Efficacy of Targeted Therapy with Vemurafenib in BRAF (V600e) - Mutated Erdheim - Chester Disease. Clin Surg. 2016; 1: 1230.
