Chordoma: Current status, problems, and future directions

Curr Probl Cancer. 2021 Aug;45(4):100771. doi: 10.1016/j.currproblcancer.2021.100771. Epub 2021 Jul 1.


Chordoma is a rare tumor that occurs along the axial spine in pediatrics and adults, with an incidence of approximately 350 cases per year in the United States. While typically described as slow-growing, many patients will eventually develop loco-regional relapse or metastatic disease with few treatment options. Despite numerous efforts over the last 10+ years, effective treatments for patients are lacking. As subtypes of chordoma are identified and described in more detail, further knowledge regarding the natural history of each type, tumor location, age differences, genomic variability, and an overall better understanding of chordoma may be the key to developing meaningful clinical trials and effective therapies for patients with chordoma.

Trial registration: NCT03595228 NCT04134312 NCT02383498 NCT04246671 NCT03910465.

Keywords: Chordoma; Conventional chordoma; Dedifferentiated chordoma; Immunotherapy; Poorly differentiated chordoma.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Chordoma* / epidemiology
  • Chordoma* / pathology
  • Chordoma* / radiotherapy
  • Chordoma* / surgery
  • Humans
  • Skeleton / pathology
  • Spinal Neoplasms* / pathology
  • Spinal Neoplasms* / radiotherapy
  • Spinal Neoplasms* / surgery

Associated data