Epidemiology
A rare primary sarcoma of bone, now diagnosed with more caution because of the molecular reclassification of many tumors. That is why pathology review at an experienced center is decisive before treatment is settled.
Basic features
It produces a destructive lesion with no obvious bone or cartilage matrix, so an expert biopsy is key.
Usual treatment
It is treated with wide resection and consideration of radiotherapy or chemotherapy according to grade, site, and extent. 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 risk of recurrence and metastasis depends on the grade and on achieving negative margins; the prognosis varies widely according to the grade.
