Primary Ewing’s sarcoma of the orbit with intracranial extension abutting the temporal lobe: a rare case report

Abstract

Ewing's sarcoma is a small, blue, round-cell tumor of mesenchymal origin which typically presents itself during the first and the second decades of life. Typically, it is noticed in the long bones of the limbs, pelvis, or ribs. There have been few reports worldwide with none in Pakistan of this rare phenomenon. We report here the case of a 16-year-old female diagnosed with Ewing's sarcoma with intracranial extension abutting the temporal lobe resulting in proptosis and partial loss of vision in the left eye. The purpose of this case is to discuss the clinicoradiological presentation, microscopic description, and management of the patient.

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

“…Of the reported 41 cases in the literature, disease-related mortality was noted in 9 (22%) patients over a mean follow-up period of 27 months (median, 17 months; range, 3-84 months). [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]39 However, in our series, disease-related mortality was noted in 11 (92%) patients over a mean follow-up period of 21 months (median, 7 months; range, 1-152 months) despite using a standard treatment protocol. The high rate of mortality in our series could be attributed to referral bias or advanced disease including intracranial extension (n = 7), and/or systemic metastasis (n = 5) at presentation.…”

“…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.…”

“…Skeletal Ewing’s sarcoma typically involves the long bones and rarely occurs in the orbit. To date, fewer than 20 cases of orbital ES have been reported in the English literature and most reports focused on pathological or clinical finding [1-3] . To our knowledge, the CT or MR imaging (MRI) findings of orbital ESs have not been reported.…”