Dedifferentiated Chondrosarcoma from Molecular Pathology to Current Treatment and Clinical Trials

Abstract

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.

FGF23 Expression Is a Promising Immunohistochemical Diagnostic Marker for Undifferentiated Pleomorphic Sarcoma of Bone (UPSb)

“…Common sites include the femur, pelvis, and humerus, as well as the scapula, rib, and tibia. Additionally, it may affect other bone types, with a predilection for pelvic bones and scapulae [9]. It is usually characterised by two distinctive histopathological components: a low-grade tumour, characterised by the deposition of nonosseous hyaline cartilage matrix, closely intersected with a high-grade noncartilaginous sarcoma tumour.…”

“…Better outcomes are associated with wide surgical margins, as they lead to higher survival rates and a decreased risk of recurrence. Palliative treatment options, such as immunotherapy and chemotherapy, are employed, even though dedifferentiated chondrosarcoma tends to exhibit relative resistance to these therapies [8,9]. Dedifferentiated chondrosarcoma is associated with a poor prognosis and an aggressive course, leading to a 5-year survival rate of 7% to a maximum of 24%.…”