Epidémiologie des sarcomes Réseaux de référence 11 mars 2016 Dr Sarah Dumont Oncologue médicale, MD, PhD Olivier Mir and Axel Le Cesne Origine du sarcome? du grec “sarx” = “chair” 1er cancer de l’Histoire David AR et al., Nat Rev Cancer. 2010 Oct Capasso LL, Int J Cancer. 2005 Jan Tout site possible Adipocytic tumours Well deifferentiated / dedifferentiated liposarcoma Myxoid / round cell liposarcoma Pleomorphic liposarcoma ….. Fibroblastic / myofibroblastic tumours Fibromatosis (desmoid) Solitary fibrous tumour / haemangiopericytoma Low grade myofibroblastic tumour Infantile fibrosarcoma Adult fibrosarcoma Mixofibrosarcoma ….. So-called fibrohistiocytic tumours Pleomorphic MFH / Undifferentiated pleomorphic sarcoma ….. Smooth muscle tumours Leiomyosarcoma ….. Skeletal muscle tumours Embryonal rhabdomyosarcoma Alveolar rhabdomyosarcoma Pleomorphic rhabdomyosarcoma Vascular tumours Epithelioid haemangioendothelioma Angiosarcoma of soft tissue ….. Chondro-osseous tumours Mesenchymal chondrosarcoma Extraskeletal osteosarcoma Tumours of uncertain differentiation Synovial sarcoma Epithelioid sarcoma Alveolar soft part sarcoma Clear cell sarcoma of soft tissue Extraskeletal myxoid chondrosarcoma Extraskeletal Ewing tumour Desmoplastic small round cell tumour Extra-renal rhabdoid tumour Malignant mesenchymoma Neoplasms with perivascular epithelioid cell differentiation (PEComa) Intimal sarcoma • Sarcome 20 % des cancers solides pédiatriques • Moins de 1% des cancers de l’adulte • Sarcomes de tissus mous surtout • 13% sarcomes osseux • Age moyen 60 ans SEER data 1973‐2008 et Burningham Z et al. Clin Sarcoma Res. 2012 Plus de 80 histotypes de sarcomes… Others OsteoS Solitary fibrous tumors Fibrosarcomas MPNST Fibromyxoid Sarcomas Angiosarcomas Ewing sarcomas Synovialosarcomas Rhabdomyosarcomas Myxofibrosarcomas MFH Uterine LMS DermatofibroS Kaposi Leiomyos non ut. Sarcoma NOS Liposarcomas GIST Environ 4000 nouveaux cas par an en France 0 0.2 0.4 0.6 0.8 Incidence / 100,000 / yr 1 1.2 Survie à 5 ans d’un sarcome métastatique: autour de 10% SEER Data Intersarc Intergroupe coopérateur