Dedifferentiated chondrosarcoma is a rare type of cancer that is very aggressive and has a poor prognosis with poor survival rates. This disease can affect anyone of any age, but it is usually diagnosed among people 50 years of age or older. There is no standard treatment available; usually it is based on surgery, however most patients are diagnosed with an advanced stage when radical treatment is not possible. We present the most up–to–date data on genetics, diagnostic procedures, and treatment options for localised and advanced diseases.

Dedifferentiated chondrosarcoma (DDCS) is a rare subtype of chondrosarcoma, a primary cartilaginous malignant neoplasm. It accounts for up to 1–2% of all chondrosarcomas and is generally associated with one of the poorest prognoses among all chondrosarcomas with the highest risk of metastasis. The 5-year survival rates range from 7% to 24%. DDCS may develop at any age, but the average presentation age is over 50. The most common locations are the femur, pelvis humerus, scapula, rib, and tibia. The standard treatment for localised disease is surgical resection. Most patients are diagnosed in unresectable and advanced stages, and chemotherapy for localised and metastatic dedifferentiated DDCS follows protocols used for osteosarcoma.