Epidemiology
A sarcoma with skeletal muscle differentiation. It is the commonest soft-tissue sarcoma in children and adolescents, but it also occurs in adults, where other subtypes predominate (pleomorphic and spindle cell/sclerosing) and management is closer to that of an adult high-grade sarcoma. In children and adolescents, management is organized with pediatric oncology, because chemotherapy and radiotherapy are usually a central part of the plan.
Basic features
It can grow quickly and cause pain, obstruction, or limitation depending on the site.
Usual treatment
Systemic chemotherapy and local control with surgery, radiotherapy, or both, depending on the site, the risk group, the age, resectability, and response; the plan is coordinated by the oncology team (pediatric when applicable). 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 depends mainly on the molecular subtype (the presence or absence of the FOXO1 fusion), along with the site, the age, and the extent: localized cases in a favorable-risk group have a relatively high survival, while metastatic disease has a considerably more guarded prognosis.