Unusual presentation of giant cell angiofibroma of the eyelids

PURPOSE. To describe a case of bilateral eyelid-confined giant cell angiofibroma (GCAF) in a patient with a slowly progressive bilateral eyelid swelling. METHODS. A 40-year-old man with a 5-year history of slowly progressive bilateral eyelid swelling, severe functional impairment, and bilateral cosmetic deformity was studied. An extensive ophthalmologic evaluation, laboratory examinations, and orbital magnetic resonance imaging were carried out. RESULTS. Clinical examination showed nonpitting lymphedema affecting both upper and lower eyelids, with orange peel skin. Orbital magnetic resonance imaging revealed diffuse thickening of the preseptal structures in the eyelids without extension to the orbit. Histologic specimen revealed the presence of spindle and multinucleated giant cells surrounding pseudovascular spaces strongly positive to CD34 and vimentin. A diagnosis of GCAF was made and radiation therapy was performed 3 weeks after surgical debulking with partial recovery of visual and anatomic function. CONCLUSIONS. Giant cell angiofibroma involving the eyelid is rare and can represent a diagnostic and therapeutic challenge to the ophthalmologist.