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.

A rare case of metastatic proximal-type epithelioid sarcoma of the ischioanal fossa: case report and literature review

“…17 ES has an unfavourable prognosis with reported 77% local recurrence and 45% distant metastasis rates, usually to regional lymph nodes, lungs, skin, scalp, bone, brain and other soft tissue parts. [18][19][20] Factors that are associated with a worse prognosis for ES are proximal and deep location, rhabdoid features, large size, older age, male sex, necrosis, and vascular invasion. 15 Due to their rare incidence and the absence of dedicated clinical trials, specific recommendations and guidelines on the optimal management of ES are almost inexistent.…”

Clinicopathological Characteristics, Treatment, and Survival in Patients Diagnosed With Proximal-Type Epithelioid Sarcoma: A Case Report and Systematic Review

“…However, 17 studies did not include this variable [10,17]. Thirty-seven patients (67.28%) presented with an initial sign of a rapidly growing palpable mass, mostly painful [3,9,[18][19][20][21][22]. Other symptoms and signs were presented according to the affected area like headaches, neck pain, nausea, vomiting, abdominal pain, abdominal fullness, visual disturbances, palpebral ptosis, eye pain and protrusion, ectropion, epiphora, anorexia, weight loss, dyspnea, edema in the affected area, constipation, fever, rectal bleeding, vaginal bleeding, and epistaxis.…”

“…Regarding treatment, 19 patients were treated surgically by wide local excision [1,4,11,12,19,[25][26][27][28][29][30][31][32][33][34], five with surgery plus lymphadenectomy [18,24,[35][36][37], seven underwent surgery and adjuvant radiotherapy [17,20,21,36,38,39], one underwent surgery and chemotherapy [26], six had surgery, chemotherapy, and adjuvant radiotherapy [13,16,17,[40][41][42], four underwent surgery, lymphadenectomy, and radiotherapy [23,36,43,44], one had surgery, lymphadenectomy, and chemotherapy [15], two underwent surgery, lymphadenectomy, radiotherapy, and chemotherapy, three received palliative radiotherapy and chemotherapy [36,45,46], and four underwent treatment with chemotherapy alone [9,10,33]. However, one patient, with metastases at presentation, received only palliative care [3] and two patients refused treatment...…”

“…Histologically, most cases were characterized by oval, polygonal, and pleomorphic epithelioid cells with abundant eosinophilic cytoplasm and enlarged vesicular nuclei with prominent nucleoli. In 22 cases, rhabdoid-looking areas were reported [4,9,11,13,17,20,21,25,[34][35][36][38][39][40][41][42]46,47].…”

“…The presence of hemorrhage [15,17,22,33,40,42] and necrosis [10,[15][16][17][18][19][21][22][23][25][26][27][32][33][34]36,37,[40][41][42]47] were common findings present in eight and 23 cases, respectively. High mitotic activity was reported in 18 tumors [3,4,10,12,16,18,19,22,24,25,31,32,35,37,[40][41][42], and in four of them, more than 20 mitoses were observed per 10 high-power fields [4,22,35,40].…”