A rare case of primary orbital Ewings sarcoma with intracranial extension

Abstract

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.

Keywords

• non-axial proptosis
• small round blue cell tumours
• skeletal metastases
• extraosseous Ewing’s sarcoma
• orbital decompression surgery