Typically affects patients in their 30s to 60s with a male predilection . They may account for as many as 12% of chondrosarcomas of bone .
Extraskeletal lesions tend to present with a palpable mass .
They are the most common extraskeletal chondrosarcomas, typically occurring in the deep soft tissues of the extremities, with the thigh being most common.
Conventional intramedullary chondrosarcomas can often demonstrate some degree of myxoid degeneration, leading to difficulty in accurate diagnosis of this entity .
Extensive myxoid stroma accounts not only for its name but also its high water content. Histologically as well as on imaging, these tumors are difficult to distinguish from chordomas and accounting for its alternative name of chordoid chondrosarcoma.
Going along with its more aggressive clinical course, the lesion is predominantly lytic, with the high water content giving it high signal on T2 weighted images, thus mimicking chordomas on imaging also. Enhancement is usually present but mild.