Update on chondrosarcomas.

PURPOSE OF REVIEW: This paper reviews recent molecular, biologic, developmental therapeutic, and clinical findings in conventional and variant chondrosarcomas. RECENT
FINDINGS: The prognosis of chondrosarcomas traditionally correlates with histologic grade and adequacy of surgery. Newer markers of cell differentiation, activation, genetics, and cell signaling may offer important prognostic information. Translational research has validated platelet-derived growth factor receptor, estrogen signaling, matrix metalloproteinase-1, histone deacetylase, methylthioadenosine phosphorylase, and vascular endothelial growth factor-A as potential therapeutic targets. Bisphosphonates may also possess important antitumoral effects. Molecular studies have established that extraskeletal myxoid chondrosarcoma is a unique entity defined by the presence of a fusion gene between the orphan nuclear receptor, CHN/NOR1, and a promiscuous partner, most commonly EWSR1. Clinical studies have shown that development of second malignancies is an uncommon but real risk for chondrosarcoma survivors; the benefit of chemotherapy for dedifferentiated chondrosarcomas remains questionable; and late recurrences of clear cell chondrosarcomas emphasize the need for long-term follow up.
SUMMARY: Chondrosarcomas are a heterogeneous group of bone and soft tissue tumors. Recent advances in molecular diagnostics, pathobiology, and developmental therapeutics will aid both scientists and clinicians in improving the classification and therapy of this diverse family of cartilaginous tumors.