Epidemiology
A primary bone sarcoma common in adolescents and young adults; typically around the knee. There is also a second, less common group of older adults, in whom it usually arises in a bone that was already diseased or previously irradiated (for example, Paget's disease). In a minority there is a hereditary predisposition (for example, Li-Fraumeni syndrome or hereditary retinoblastoma), so genetic counseling is sometimes offered. Before surgery, staging is completed —especially of the chest— because treatment combines chemotherapy with planned surgery.
Basic features
A malignant tumor that produces immature bone. It usually causes persistent pain, swelling, and aggressive changes on X-ray.
Usual treatment
Chemotherapy before and after surgery, plus wide resection with reconstruction or limb-salvage surgery when possible. 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
The outlook depends mainly on whether there are metastases, on how well the tumor responds to chemotherapy (assessed in the removed specimen as the degree of necrosis), and on achieving complete removal: it is more favorable in localized disease with a good response, and more guarded when metastases are present at diagnosis.