Primary Ewing’s sarcoma of the orbit: case report

Abstract

A 22-year-old man presented with tenderness and swelling of the left lateral part of the orbit. Computed tomography revealed a left intraorbital mass measuring 3 cm x 3 cm involving the left lateral wall of the orbit and the greater wing of the left sphenoid bone. Magnetic resonance imaging revealed that the intraorbital mass was extraneuroaxial. During surgery, the tumor was seen to arise from the lateral wall of the orbit and infiltrate into the left temporal muscle. Following the surgery, the patient was administered radiation therapy for the whole cranium and chemotherapy for the residual tumors. However, the tumor recurred, and the patient died about 2 years following the first surgery because the tumor had metastasized to the lung. On light microscopy, the tumor cells were closely packed with uniform, small, and round cells. Immunohistochemical studies showed that the tumor cell membrane stained positive for MIC2. Furthermore, the MIB-1 labeling index was 36.2%. On electron microscopy, small quantities of cytoplasm containing glycogen accumulations without neurosecretory granules and neurofilaments were observed. Based on these results, the tumor was diagnosed to be primary Ewing's sarcoma. Primary orbital Ewing's sarcoma of the skull has been considered to be extremely rare, and a review of the literature was performed.

Primary orbital Ewing sarcoma family of tumors: a study of 12 cases

“…35 The poor prognostic factors for ESFTs are metastatic disease at presentation, large tumor 45 cm, older age, male gender, central tumor location including skull, clavicle, ribs, pelvis, vertebrae, and the upper extremities. 35,[36][37][38] Orbital ESFT is considered to be less aggressive than ESFT at other locations in the body, [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]39 as the survival has been more than 6 months in majority of the reported cases. Although extraorbital extension is frequently reported with primary orbital ESFTs, systemic metastases at presentation or during follow-up duration is rare as per the available literature.…”

“…3 Primary orbital ESFT is extremely rare with isolated case reports and small case series reported in literature. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] Herein we discuss the demographic profile, clinical features, radiological features, treatment modalities, and outcomes of primary orbital ESFTs.…”

CT and multimodal imaging findings of primary orbital Ewing's sarcoma involving the middle cranial fossa: a case report

“…Necrosis, hemorrhage and adjacent organ invasion could be partially detected, while calcification was never seen. As to the imaging features of orbital ES, only Kano, et al and Naqvi, et al mentioned it in their case reports that focused on the clinicopathologic features of orbital ES [2-3] . The two irregular orbit ES masses in their case reports were located in the left lateral wall of the orbit, and the greater wing of the sphenoid bone was involved.…”

Exenteration and Custom Implant Brachytherapy as a Treatment for Recurrent Primary Extraskeletal Orbital Ewing Sarcoma

“…14,16 A review of 22 cases of primary orbital ES found 7 patients died within an average of 17.1 months and none survived for more than 5 years. 9 To date, approximately 25 cases of primary orbital ES have been reported; however, no reports comment on favorable treatment of recurrent disease. The technique described in this report requires the multidisciplinary efforts of ophthalmologists, radiation oncologists, pediatric oncologists, dentists, radiation physicists, radiologists, and nurses.…”

“…12–14 Recurrent ES carries a poor prognosis 15 with no past reports of successful treatment of recurrent primary ES of the orbit, which often progresses rapidly and has a propensity to metastasize. 9,10,16,17 The authors report a case of primary extraskeletal ES of the orbit in a 6-year-old boy and successful treatment using a unique multimodal treatment approach. This case report is in compliance with the Health Information Portability and Accountability Act and followed the principles of the Declaration of Helsinki.…”