Research highlights Management of bone sarcomas in reference center improves compliance to guidelines and survival. Antiangiogenic tyrosine kinase inhibitors exert significant antitumor activity in most primary bone cancers. Second-line cytotoxic chemotherapy regimens have demonstrated activity in Ewing's sarcoma. Immunotherapy with…
Introduction Primary malignant tumors of the bone represent less than 1% of all cancer cases in the European population. The world age-standardized incidence rate is in the range of 0.5–2/100,000/year [ ]. In 2020, an estimated 3600 new cases and…
Introduction Close to 1% of all tumors are primary malignant bone tumors. Out of these, the chondrosarcoma is the second most common primary malignant bone tumor in adults with an incidence of 3–4/100,000 per year. Chondrosarcoma is the collective term…
Research highlights Regulated cell death (RCD) maintaining tissue homeostasis and integrity comprises apoptosis, autophagic cell death, and necroptosis. Chemoresistance involves deregulation of particular mechanisms/pathways of RCD. A better understanding of chemotherapy-induced RCD mechanisms may help to improve treatment options. New…
Introduction When a medical team is dealing with a primary malignant bone tumor, the standard goal of the treatment is to totally eradicate the disease with minimal treatment-related adverse effects. In that way, for more than three decades, complete surgical…
Introduction Bone sarcomas include a variety of primary, nonepithelial, malignant neoplasms with metastatic potential originating from bone cells or their precursors. Some are purely osteolytic, while others produce a calcified matrix (e.g., osteosarcoma), a cartilaginous matrix (e.g., chondrosarcoma), or a…
Introduction Sarcomas of the bone are relatively rare neoplasms with an estimated 2890 new cases in the United States in 2012 [ ]. Because of the rarity of primary bone sarcomas and interpretive uncertainty, management may be delayed from initial…
Research highlights The cartilage tumor family is a heterogeneous group of tumors, driven by mutations, translocations, or unknown factors. The biology of cartilage tumors is reflected by defective growth plate signaling pathways. Chondrosarcomas can arise within a benign precursor or…
Learning points ⇒ The H3-3A.Gly34Trp (G34W) missense mutation is essentially pathognomonic of giant cell tumor of bone, occasionally to p.Gly34Leu (G34L) alterations. There are good antibodies available for detecting this mutation by immunohistochemistry. Malignant giant cell tumor of bone harbors…
Introduction Since the mid-1990s when Ewing's sarcoma started to be studied at the molecular level, a very large number of investigations aimed at deciphering the biology of this disease, particularly by exploring the role of the Ewing's sarcoma–specific oncogene. Even…