Primary orbital Ewing's sarcoma: Report of a case and review of the literature

Primary orbital Ewing's sarcoma is a very rare condition. Since the first c ase was reported in 1950, only 7 other cases have been reported in the Engl ish literature. Herein we describe the ninth, the only bilateral and the yo ungest case of primary orbital Ewing's sarcoma occurring in a 2-year-old bo y, who presented to us with bilateral painless proptosis. Tissue biopsy of the tumour was obtained through the nasal sinus. Immunohistological studies of the biopsy tissue confirmed the diagnosis of Ewing's sarcoma. No distan t site of the tumour was found so this was considered a primary orbital tum our. Combined chemotherapy and radiotherapy without surgical resection achi eved an encouraging result in that the patient has remained in remission fo r 30 months after completion of treatment. The successful use of combined c hemotherapy and radiotherapy, without surgery, adds further support to evid ence that surgical excision may be avoided in selected cases of primary orb ital Ewing's sarcoma.