Downie EM (1), Amend CE (2), Miranda A (3), Burkat CN (1).
(1) Oculoplastic, Facial Cosmetic, and Orbital Surgery, Department of Ophthalmology and Visual Sciences. (2) Division of Pediatric Hematology, Medical Oncology and Palliative Care. (3) Pediatric Ophthalmology, Department of Ophthalmology and Visual Sciences, University of Wisconsin-Madison, Madison, Wisconsin, U.S.A.
Publication
Ophthalmic Plast Reconstr Surg
Summary
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.
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