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. [1]

Dedifferentiated chordomas most frequently harbor alterations in WAS, TP53, STAT5B, ERBB3, and EP300 [2].

Most Commonly Altered Genes in Dedifferentiated Chordoma

WAS P394Q, WAS Mutation, TP53 X224_splice, STAT5B Mutation, and STAT5B M137T are the most common alterations in dedifferentiated chordoma [2].

Top Alterations in Dedifferentiated Chordoma

Significant Genes in Dedifferentiated Chordoma


Disease Details

Sarcomatoid Chordoma
OncoTree Name
Dedifferentiated Chordoma
OncoTree Code


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.