Back to Diseases List
Associated Genetic Biomarkers
NCI Definition: A high-grade malignant bone tumor arising from the remnants of the notochord. It is characterized by a lobulated growth pattern, myxoid stroma formation, the presence of physaliphorous cells, and a sarcomatous component. 
Dedifferentiated chordomas most frequently harbor alterations in WAS, TP53, STAT5B, ERBB3, and EP300 .
WAS P394Q, WAS Mutation, TP53 X224_splice, STAT5B Mutation, and STAT5B M137T are the most common alterations in dedifferentiated chordoma .
There is 1 clinical trial for dedifferentiated chordoma, of which 1 is open and 0 are completed or closed. Of the trial that contains dedifferentiated chordoma as an inclusion criterion, 1 is phase 2 (1 open).
SMARCB1 is the most frequent gene inclusion criterion for dedifferentiated chordoma clinical trials .
Ipilimumab and nivolumab are the most common interventions in dedifferentiated chordoma clinical trials.
Significant Genes in Dedifferentiated Chordoma
SMARCB1 is an inclusion eligibility criterion in 1 clinical trial for dedifferentiated chordoma, of which 1 is open and 0 are closed. Of the trial that contains SMARCB1 status and dedifferentiated chordoma as inclusion criteria, 1 is phase 2 (1 open) .
1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].
2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.
3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.