Treatment of Orbital Desmoid-type Fibromatosis With Sorafenib. Issue 5 (25th April 2022)

• Record Type: Journal Article
• Authors: Downie, Elaine M.; Amend, Christina E.; Miranda, Alexander; Burkat, Cat N.
• Is Part Of: Ophthalmic plastic and reconstructive surgery. Volume 38:Issue 5(2022)
• Page Start: e144
• Page End: e147
• Publication Date: 2022-04-25
• Subjects: Eye -- Surgery -- Periodicals; Ophthalmic plastic surgery -- Periodicals; Reconstructive Surgical Procedures -- Periodicals; Ophthalmologic Surgical Procedures -- Periodicals; Ophthalmology -- Periodicals; Surgery -- Periodicals; 617.70592
• DOI: 10.1097/IOP.0000000000002186
• Languages: English
• ISSNs: 0740-9303

Abstract

Abstract : Desmoid-type fibromatosis is a rare tumor, particularly in the orbit, with fewer than 10 cases of primary orbital desmoid-type fibromatosis reported in the literature. The authors present a case of an infant who presented with rapid onset of OD proptosis, disc edema, and hyperopic shift who was found to have a retrobulbar desmoid-type fibromatosis. After initial biopsy, due to risk of vision loss with complete excision, the tumor was treated with sorafenib, a tyrosine kinase inhibitor. During the course of treatment with sorafenib, the tumor stabilized and then regressed in size. To the authors' knowledge, this is the first reported case of orbital desmoid-type fibromatosis to be treated with sorafenib. Abstract : Presentation of a case of orbital desmoid-type fibromatosis in a pediatric patient that was treated with sorafenib; to the authors' knowledge, this is the first case reported of orbital desmoid-type fibromatosis treated in this manner.