Epidemiology
A rare molecular sarcoma, more common in adolescents and young adults, that usually arises in deep soft tissue. It can look like Ewing sarcoma, but it usually has a distinct biology; molecular tests are needed to name it correctly.
Basic features
It can look like Ewing sarcoma, but it has different genetics and behavior.
Usual treatment
It is managed at sarcoma centers with chemotherapy, surgery, and radiotherapy depending on extent and response. Before chemotherapy starts it is worth discussing fertility preservation with the team, because some of these treatments can affect it and the options have to be raised beforehand.
Outcomes and prognosis
It tends to be aggressive, with a more guarded prognosis than Ewing sarcoma; follow-up looks for early recurrence and metastases.
