Author/Authors :
Lee, Christopher M. Department of Radiation Oncology - Cancer Care Northwest - Spokane - WA, USA , Kaya, Erin A. Department of Radiation Oncology - Cancer Care Northwest - Spokane - WA, USA , Wagner, Aaron E. Department of Radiation Oncology - Cancer Care Northwest - Spokane - WA, USA , Thatcher, Steve H. Department of Radiation Oncology - Cancer Care Northwest - Spokane - WA, USA , Lamoreaux, Wayne T. Department of Radiation Oncology - Cancer Care Northwest - Spokane - WA, USA , Fairbanks, Robert K. Department of Radiation Oncology - Cancer Care Northwest - Spokane - WA, USA , Broadbent, Talmage J. Northwest Eyelid and Orbital Specialists - Spokane - WA, USA , Thomas, Cheddhi J. Incyte Diagnostics, Spokane - WA, USA
Abstract :
Epithelioid sarcoma is a rare high-grade malignancy identified by Enzinger in 1970. It accounts for 1% of all reported softtissue sarcomas and presents most commonly in distal upper extremities in young adults with a male predominance. Atthis time, there are only 5 previously reported cases of primary epithelioid sarcoma of the orbit. We present a primaryorbital epithelioid sarcoma case of a patient who underwent orbital exenteration followed by external beam radiationtreatment. Because the literature is limited, this is to our knowledge the largest descriptive analysis of cases of orbitalepithelioid sarcoma. We also provide a detailed review of all the previously reported primary orbital epithelioid sarcomacases, as well as a discussion on the use of postoperative radiation therapy for patients with epithelioid sarcoma. Surgicalresection followed by adjuvant radiation therapy appears to be a safe option for local treatment of this rare malignancy,but further future studies are needed of this rare clinical situation in order to better understand and optimize treatmentfor patients with orbital epithelioid sarcoma
