Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature

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.

Figure 1

Picture of initial presentation.

Figure 2

Left orbit: 2.0 × 1.6 × 1.0 cm nonenhancing soft tissue extraconal medial intraorbital lesion abutting the nasolacrimal duct, lateral and inferior to the globe. No thickening of the lateral rectus muscle or surrounding inflammation.

Figure 3

Pathology. (a) 100x, low power. Cellular neoplasm with microcystic areas associated with myxoid matrix deposition. Focally extensive fields of necrosis were also present in this case (bottom). (b) 400x, high power. Densely cellular neoplasm comprised variably cohesive cells with dense intracytoplasmic eosinophilic inclusions and eccentric nuclei with focally prominent nucleoli.

Figure 4

Picture after healing from left orbital exenteration surgery with closure.

Figure 5

Postoperative radiation therapy to the left orbit with IMRT. Graphic illustration of the radiotherapy dose cloud. The associated table shows the color map correlation with radiation dose in cGy.