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

Anna Maria Frezza, Laura Botta, Annalisa Trama, Angelo P. Dei Tos, Silvia Stacchiotti

Research output: Contribution to journalArticlepeer-review


Purpose of reviewChordoma 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.Recent findingsThe incidence of chordoma is approximately 0.08/100000 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.SummaryChordoma 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.

Original languageEnglish
Pages (from-to)114-120
Number of pages7
JournalCurrent Opinion in Oncology
Issue number2
Publication statusPublished - Mar 1 2019


  • chordoma
  • disease
  • epidemiology
  • epigenetics
  • molecular target agents

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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