Treatment of dedifferentiated chordoma: a retrospective study from a large volume cancer center

J Neurooncol. 2019 Sep;144(2):369-376. doi: 10.1007/s11060-019-03239-3. Epub 2019 Jul 23.


Objective: Dedifferentiated chordomas (DC) are genetically and clinically distinct from conventional chordomas (CC), exhibiting frequent SMARCB1 alterations and a more aggressive clinical course. We compared treatment and outcomes of DC and CC patients in a retrospective cohort study from a single, large-volume cancer center.

Methods: Overall, 11 DC patients were identified from 1994 to 2017 along with a cohort of 68 historical control patients with CC treated during the same time frame. Clinical variables and outcomes were collected from the medical record and Wilcoxon rank sum or Fisher exact tests were used to make comparisons between the two groups. Kaplan-Meier survival analysis and log-rank tests were used to compare DC and CC overall survival.

Results: DC demonstrated a bimodal age distribution at presentation (36% age 0-24; 64% age > 50). DC patients more commonly presented with metastatic disease than CC patients (36% vs. 3% p = 0.000). DC patients had significantly shorter time to local treatment failure after radiation therapy (11.1 months vs. 34.1 months, p = 0.000). The rate of distant metastasis following treatment was significantly higher in DC compared to CC (57% vs. 5%, p = 0.000). The median overall survival after diagnosis for DC was 20 months (95% CI 0-48 months) compared to 155 months (95% CI 94-216 months) for CC (p = 0.007).

Conclusion: DC patients exhibit significantly higher rates of both synchronous and metachronous metastases, as well as shorter overall survival rates compared to conventional chordoma. The relatively poor survival outcomes with conventional therapies indicate the need to study targeted therapies for the treatment of DC.

Keywords: Chordoma; Dedifferentiated chordoma; INI-1; Radiation; SMARCB1.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Cell Differentiation*
  • Child
  • Child, Preschool
  • Chordoma / pathology
  • Chordoma / radiotherapy*
  • Female
  • Follow-Up Studies
  • Hospitals, High-Volume / statistics & numerical data*
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / radiotherapy*
  • Radiotherapy / mortality*
  • Retrospective Studies
  • Spinal Neoplasms / pathology
  • Spinal Neoplasms / radiotherapy*
  • Survival Rate
  • Treatment Outcome
  • Young Adult