Why the study?
Primary Ewing's sarcomas of the orbit with intracranial extension are extremely rare, with few cases reported to date.
Total tumour excision with adjuvant radiation and chemotherapy may be helpful in survivability in patients with primary orbital Ewing's sarcoma with intracranial extension, though overall prognosis remains poor.
May support multimodality therapy in rare orbital Ewing sarcoma; hypothesis-generating and should not yet change practice.
Primary Ewings sarcomas of orbit with intracranial extension are extremely rare with few cases reported to date. They form an important differential diagnosis in aggressive orbital tumours presenting in young males. We presented the case report of a young male presenting with right eye proptosis. The patient underwent complete tumour excision. Biopsy was suggestive of Ewings sarcoma. Metastatic workup showed extensive lesions in lungs and bones. Six months post-operative follow-up with radiation and chemotherapy showed disease remission. CD99 is a key IHC marker in differentiating from other similar tumours. Total tumour excision with adjuvant radiation and chemotherapy may be helpful in survivability in such patients but overall prognosis still remains poor.
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Ghosh et al. (2023) studied this question.
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