Orbital angiosarcoma — case report and treatment options

Abstract

Orbital angiosarcoma is an exceedingly rare subgroup of angiosarcoma. Angiosarcoma makes up less than 1 % of all soft tissue sarcomas. Fifteen reported cases to date of orbital angiosarcoma demonstrate its size-dependent, aggressive, widely infiltrative nature, poor circumscription, multifocality, tendency for local recurrence, predilection for pediatric age groups, and dismal prognosis. Hufnagel and Koo [18] in 1987 eloquently and comprehensively reviewed the literature regarding this unusually rare subset of angiosarcoma. Proptosis associated with blepharoptosis was the most common presenting symptom. Further, swelling was noted in approximately one-half of the patients, and ophthalmoplegia was noted in approximately one-fourth. In addition, Hufnagel and Koo [18] reviewed the treatment options for orbital angiosarcoma. The options range from excision alone, excision followed by radiating therapy (RT), orbital exenteration, exenteration with RT and exenteration with RT and chemotherapy. They noted no apparent correlation between treatment modality and long-term outcome. Only three of the fifteen patients were noted to be alive without evidence of disease at 14 months, 1.5 years, and 2.5 years. The purpose of this paper is to document this rare case, to demonstrate the utility of en block resection with staged reconstruction and to report long-term survival.