Abstract
Epithelioid sarcoma is a rare high-grade malignancy identified by Enzinger in 1970. It accounts for 1% of all reported soft tissue sarcomas and presents most commonly in distal upper extremities in young adults with a male predominance. At this time, there are only 5 previously reported cases of primary epithelioid sarcoma of the orbit. We present a primary orbital epithelioid sarcoma case of a patient who underwent orbital exenteration followed by external beam radiation treatment. Because the literature is limited, this is to our knowledge the largest descriptive analysis of cases of orbital epithelioid sarcoma. We also provide a detailed review of all the previously reported primary orbital epithelioid sarcoma cases, as well as a discussion on the use of postoperative radiation therapy for patients with epithelioid sarcoma. Surgical resection 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 treatment for patients with orbital epithelioid sarcoma.
Highlights
Epithelioid sarcoma (ES) is a rare high-grade malignancy identified by Enzinger in 1970 [1]
Of the five primary orbital ES cases reported, two patients were treated with orbital exenteration [5], two were treated with surgical debulking followed by chemotherapy [6, 7], and one was treated with surgical excision followed by local radiation therapy [2]
We present a unique case of a patient with recently diagnosed primary orbital ES who underwent orbital exenteration followed by adjuvant external beam radiation treatment
Summary
Epithelioid sarcoma (ES) is a rare high-grade malignancy identified by Enzinger in 1970 [1]. It accounts for 1% of all reported soft tissue sarcomas [2]. Of the five primary orbital ES cases reported, two patients were treated with orbital exenteration [5], two were treated with surgical debulking followed by chemotherapy [6, 7], and one was treated with surgical excision followed by local radiation therapy [2]. We present a unique case of a patient with recently diagnosed primary orbital ES who underwent orbital exenteration followed by adjuvant external beam radiation treatment. We provide a detailed review of all other reported primary orbital ES cases in the literature
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