ORBITAL ANGIOSARCOMA - CASE-REPORT AND TREATMENT OPTIONS

Orbital angiosarcoma is an exceedingly rare subgroup of angiosarcoma. Angiosarcoma makes up less than 1% of all soft tissue sarcomas. Fiftee n reported cases to date of orbital angiosarcoma demonstrate its size- dependent, aggressive, widely infiltrative nature, poor circumscriptio n, multifocality, tendency for local recurrence, predilection for pedi atric 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 bleph aroptosis was the most common presenting symptom. Further, swelling wa s 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 opti ons 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 treat ment modality and long-term outcome. Only three of the fifteen patient s 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 r are case, to demonstrate the utility of en block resection with staged reconstruction and to report long-term survival.