Epidemiology
A rare sarcoma of adults, usually in the deep tissues of the limbs. Despite the name, it does not arise in bone; it is usually deep and slow-growing, with possible late recurrences.
Basic features
It grows slowly and has a myxoid matrix; the name does not mean that it produces typical cartilage. It is defined by a rearrangement of the NR4A3 gene, which confirms the diagnosis against other myxoid-looking tumors.
Usual treatment
Wide resection is central; radiotherapy and systemic treatment are considered according to risk or advanced disease.
Outcomes and prognosis
It usually follows a prolonged course: the short-term prognosis is favorable, but it declines over the long term because of late recurrences and metastases.
