Orbital angiosarcoma ? case report and treatment options

Abstract

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.Key words: Orbit -Angiosarcoma -Reconstruction Case reportA 71-year-old male presented to the Institute for Craniofacial and Reconstructive Surgery at Providence Hospital, Southfield, Michigan in November of 1991, stating a six week history of swelling of the left side of the nose and medial canthal region. He was otherwise asymptomatic without ocular, nasal or lacrimal system-related symptoms. The patient underwent fine needle aspiration cy- En block resection of the left medial canthus, medial orbit including roof and floor, as the medial aspect of both the upper and lower eyelids was then performed. Initial reconstruction utilized a lower eyelid switch flap (Mustarde) for the first stage upper eyelid reconstruction and a forehead flap for nasal and medial canthal reconstruction. Six weeks later, second stage reconstruction was accomplished by insetting the lower eyelid switch flap into the upper eyelid defect. A nasal chondromucosal graft was then harvested, sutured into place in the lower eyelid, and a cheek flap was then raised to complete the lower eyelid reconstruction. The patient has been followed closely postoperatively, both with clinical exams and follow-up scans, and shows no evidence of recurrent nor metastatic disease at 41/2 years. DiscussionAngiosarcoma is a rare entity comprising less than 1% of all soft tissue sarcomas. The etiology of angiosarcoma has been speculatively linked to a number of elements. For example: 1) connective tissue dystrophy secondary to chronic lymphedema [27], 2) upper extremity lymphedema associated with the post-mastectomy patient [6,15,16], radiotherapy in a variety of different clinical settings [2,11,14,2...