Orbital Epithelioid Sarcoma

Abstract

Epithelioid sarcoma is a rare but often aggressive malignancy of soft tissue that usually occurs in young adults as a superficial lesion in the distal upper limbs. To date, there are only 4 case reports of epithelioid sarcoma primarily occurring in the orbit. Two of these patients were treated with primary exenteration only one of whom was alive 3 years after diagnosis. Radical surgical excision is thus the first treatment of choice for primary orbital epithelioid sarcoma. The authors present a patient with primary orbital epithelioid sarcoma who refused exenteration. Surgical debulking followed by local brachytherapy was performed. The patient remains tumor free 5 years after diagnosis. The literature remains limited regarding treatment options for primary orbital epithelioid sarcoma. However, based on reported cases and this case, the authors conclude that surgical excision combined with local iridium radiation therapy is an acceptable treatment when treating primary orbital epithelioid sarcoma.

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

“…The fourth case was reported in 2014 with treatment including orbit exenteration and chemotherapy, but the patient died 14 months after initial diagnosis [6]. The fifth reported case in 2016 is most similar to ours in that the patient underwent a macroscopic radical excision and postoperative radiation therapy treatment, and the patient was still in remission 5 years following the initial presentation [2].…”

“…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]. It is typically divided into two clinical subtypes including a distal type and proximal type.…”

“…ES is occasionally found in the scalp, ear, and hard palate. Even though the etiology is unclear for these rare malignancies, they are felt to originate from mesenchymal tissue [2, 5, 6]. ES has an unfavorable prognosis with reported 77% local recurrence and 45% distant metastasis rates, usually to regional lymph nodes and lungs [6].…”

“…To date, there are only 5 previously reported cases of ES of the orbit to our knowledge. 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]. Of note, only the patient who underwent excision with radiation therapy was still in remission 5 years following the initial presentation [2].…”

Epithelioid sarcoma arising from the temporal space

“…[ 2 ] Such tumors occur in the scalp, the auricle and periauricular area, the neck, the temporo-mandibular region, the oral cavity, the parotid gland, the nose, and the orbit. [ 6 , 9 – 13 ] Epithelioid sarcoma is often characterized by a high propensity toward regional lymph node metastasis, local recurrence, and distant metastasis. The prognosis in terms of overall survival is associated with all of tumor location; tumor size and depth; the number of mitotic figures; and the presence/absence of hemorrhage, necrosis, and vascular invasion.…”