Ewing sarcoma: a tumor with an uncanny predilection for uncommon sites

Abstract

Ewing sarcoma is one of the most common bone tumors diagnosed in pediatric age group second only to osteosarcoma. The tumor is known to involve most commonly pelvic bones and long bones while extra-skeletal Ewing sarcoma accounts for 15%. Ewing sarcoma of other sites like scapula, clavicle, hands, and feet are quite rare, accounting for only 3-5%. To determine the epidemiological profile of Ewing sarcoma of unusual sites treated in our department including age at diagnosis, site of origin and mode of management along with survival data. Details of Ewing sarcoma patients reported our department in last 9 years were collected and data was analysed. We report a total of 20 cases of Ewing sarcoma in last 9 years with 4 (20%) extra skeletal Ewing sarcoma arising from soft tissues of extremities and 30% from rare sites (3 scapula, 1 clavicle, 1 phalanx, 1 calcaneum). All patients were operated after 4-7 cycles of chemotherapy with 66% limb salvage rate. With an average necrosis of 40%, with a median follow up of 36 months, the overall survival of the group was found to be 77.7%. Ewing sarcoma is one of the rare tumors of bone and soft tissue with the predilection for unusual sites, with reasonable survival outcomes in localized disease. Though scapula, clavicle, hands and feet are rare sites for Ewing sarcoma, they accounted for 30% in our department, possibly because of the referral patterns.