Epidemiology
A very rare vascular sarcoma of bone, usually in adults, that can be multifocal. Because of its aggressiveness and possible multifocality, it is assessed with staging imaging before surgery is defined.
Basic features
It tends to destroy bone and form a soft-tissue mass, with progressive pain or a pathologic fracture (a break through weakened bone).
Usual treatment
Complete resection with negative margins is the central goal when feasible, because achieving full local control is the main factor linked to a better outcome; radiotherapy and systemic treatment are added depending on the extent of disease or when surgery is not possible.
Outcomes and prognosis
It carries a high risk of metastasis and recurrence and an unfavorable prognosis, related to the extent of disease and to local control.
