Chordoma: update on disease, epidemiology, biology and medical therapies

DOI Web Site 1 Citations
  • Anna Maria Frezza
    Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale Tumori
  • Laura Botta
    Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan
  • Annalisa Trama
    Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan
  • Angelo P. Dei Tos
    Department of Pathology, Treviso Regional Hospital, Treviso
  • Silvia Stacchiotti
    Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale Tumori

Search this article

Description

<jats:sec> <jats:title>Purpose of review</jats:title> <jats:p>Chordoma is an exceedingly rare subtype of bone sarcoma. This review aims to provide a comprehensive insight into chordoma epidemiology, and an update on the recent advances in disease, biology and medical therapies.</jats:p> </jats:sec> <jats:sec> <jats:title>Recent findings</jats:title> <jats:p>The incidence of chordoma is approximately 0.08/100 000 and the 5-year overall age-adjusted relative survival is 72% in the United States and 61% in Europe. Over the last years, significant steps forwards have been done in the comprehension of chordoma complexity, with insights gained into the biology and morphology of this disease. New entities have been described and potentially druggable molecular targets identified. This is becoming all the more relevant today, as new potentially active agents are under development.</jats:p> </jats:sec> <jats:sec> <jats:title>Summary</jats:title> <jats:p>Chordoma is a complex disease because of its rarity, biological heterogeneity and peculiar clinical behaviour. Despite the progress done, the outcome in this disease remains unsatisfactory and the identification of active systemic treatments remains an urgent, unmet medical need.</jats:p> </jats:sec>

Journal

Citations (1)*help

See more

Details 詳細情報について

Report a problem

Back to top