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…
Introduction First described by James Ewing in 1921 [ ], Ewing's sarcoma (ES) represents a family of morphologically similar small round cell sarcomas (SRCSs) including “classic” Ewing's sarcoma of bone, extraskeletal Ewing's sarcoma [ ], small cell tumor of the…
Abbreviation OS Osteosarcoma Acknowledgments This work has been supported in part by funds provided by the Foundation AECC “Proyectos Estratégicos” to F.L. Introduction Classical pediatric human osteosarcoma (OS) is considered a heterogeneous entity characterized by inactivation of tumor suppressors, altered…
Acknowledgments This work has been supported in part by funds provided by the Haley's Hope Memorial Support Fund for Osteosarcoma Research at the University of Alabama at Birmingham. Introduction Although osteosarcoma is a relatively rare neoplasm, it is the most…
Introduction The classification of bone sarcomas is still today mainly based on the comparison of histological, clinical, and radiological criteria. This classification evolves with the improvement of knowledge on the mechanisms of carcinogenesis of these tumors which results in the…
Research highlights Recurrent genomic rearrangements and specific somatic mutations in bone tumors are leading to new diagnostic tests. Deregulated fusion transcription factors are revealing how epigenomic changes may underlie Ewing's sarcoma oncogenesis. Deep sequencing studies have contributed toward improved understanding…
Introduction After the discovery of the Philadelphia chromosome in patients with chronic myeloid leukemia [ ], investigators in the cancer field tried to find other associations between chromosomes and cancer, including bone tumors [ ]. The cytogenetic findings of bone…
Acknowledgments K.P. is supported by European Research Council Advanced Investigator Grant “INJURMET”; C.A-P. is supported by the National Institute of Cancer (INCa, ) and SIRIC Montpellier Cancer Grant INCa_Inserm_DGOS_12553; K.P. and C.A-P. are both supported by the ERA-NET TRANSCAN…