Primary Orbital Ewing Sarcoma in a Middle-aged Woman

Ewing′s sarcoma of the orbit with intracranial extension: A rare cause of unilateral proptosis

“…Separate cases of involvement of either the orbit or the cranial compartment have been reported,[4–9] but a combination of the two has been a rarity. Syed has reported a case of intracranial Ewing's sarcoma of the calvarium that presented with features of mass effect and was excised in toto .…”

Primary orbital Ewing's sarcoma presenting with unilateral proptosis

“…Primary EWSs of the orbit have been recognized, accounting for 2–3% of all EWSs. Symptoms and signs of orbital involvement include pain, headache, reduced vision, lower eyelid swelling, palpable mass, proptosis, ophthalmoplegia, papilloedema and blindness 5 . The main differential diagnosis for unilateral proptosis in a 14‐year‐old patient includes rhabdomyosarcoma, granulocytic sarcoma and possibly Langerhans cell histiocytosis.…”

Magnetic Resonance Imaging of Primary Orbital Ewing’s Sarcoma

“…It represents about 6–9% of all malignant bone tumours and has a predilection for long bones, ribs and the pelvis [8, 9]. A literature search revealed only eleven cases of primary cranial Ewing’s sarcoma [1,2,3,4,5,6,7,8,9,10,11,12,13] of which only seven were involving the orbit [1,2,3,4,5,6,7]. Orbital tumours usually grow outwards, leading to commonly seen localized swelling.…”

“…Preoperative imaging could thus prove crucial in such a scenario. Surprisingly, of the seven cases of primary orbital Ewing’s sarcoma described in the literature [1,2,3,4,5,6,7], none had a magnetic resonance imaging (MRI) study performed. We report a case of primary orbital Ewing’s sarcoma, the MR findings in which were very unusual and suggest that the MR findings of orbital Ewing’s sarcoma could be different from those of Ewing’s sarcoma elsewhere in the body.…”