Orbital angiosarcoma — case report and treatment options

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. 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. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Journal of Plastic Surgery Springer Journals

Orbital angiosarcoma — case report and treatment options

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Publisher
Springer-Verlag
Copyright
Copyright © 1997 by Springer-Verlag
Subject
Medicine & Public Health; Plastic Surgery
ISSN
0930-343X
eISSN
1435-0130
D.O.I.
10.1007/BF01419140
Publisher site
See Article on Publisher Site

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.

Journal

European Journal of Plastic SurgerySpringer Journals

Published: Mar 1, 1997

References

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