Overview

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]

Significant Genes in Dedifferentiated Chordoma

SMARCB1 +

Disease Details

Synonyms
Sarcomatoid Chordoma
Parent(s)
Chordoma
OncoTree Name
Dedifferentiated Chordoma
OncoTree Code
DDCHDM

References

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 6. 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.