This review provides an overview of the histopathology, classification, diagnostic procedures, and
therapy of skeletal
chondrosarcoma.
Chondrosarcomas that arise de novo are primary
chondrosarcomas, whereas
chondrosarcomas developing superimposed on pre-existing benign cartilage
neoplasms such as
enchondromas or
osteochondromas are referred to as secondary
chondrosarcomas. Conventional
chondrosarcomas can be categorized according to their location in bone into central, peripheral, and juxtacortical
chondrosarcomas. Histological grading is related to prognosis; however, it is also subject to interobserver variability. Rare subtypes of
chondrosarcoma, including dedifferentiated, mesenchymal, and
clear cell chondrosarcoma, are discussed as well. Magnetic resonance imaging is necessary to delineate the extent of the intraosseous and soft tissue involvement preoperatively. Computed tomography is especially recommended in the pelvis and other flat bones where it may be difficult to discern the pattern of bone destruction and the presence of matrix mineralization. Wide, en-bloc excision is the preferred surgical treatment in intermediate- and high-grade
chondrosarcoma. In low-grade
chondrosarcoma confined to the bone, extensive intralesional
curettage followed by local adjuvant treatment and filling the cavity with bone graft has promising long-term clinical results and satisfactory local control.
Chondrosarcomas are relatively
radiotherapy resistant; therefore, doses >60 Gy are needed in attempts to achieve local control after incomplete resection. Irradiation with
protons or other charged particles seems beneficial in this curative situation.
Chemotherapy is only possibly effective in
mesenchymal chondrosarcoma, and is of uncertain value in dedifferentiated
chondrosarcoma. Potential new systemic treatment targets are being discussed.