"Extraskeletal Ewing sarcoma (EES) is included in the Ewing sarcoma family of tumours (ESFT) along with Ewing sarcoma of bone, peripheral primitive neuroectodermal tumour (pPNET), peripheral neuroepithelioma, and Ewing sarcoma of the chest wall (previously known as Askin tumour). When compared with Ewing sarcoma of bone, extraskeletal Ewing sarcoma is rare."
"A rapidly growing, solitary, superficial or deep mass in the soft tissues is generally seen between 5 and 10 cm at the initial presentation 8. Depending on where the tumour is located, adult patients generally present with a painless mass or vague abdominal or chest pain 1. If the mass is seen to be paravertebral, the patient can present with symptoms of cord compression 10. Between 35% and 43% of adult patients have metastatic disease at presentation 3,5. The lung is the most common site of metastasis 4."