Overview

NCI Definition: An aggressive morphologic variant of chondrosarcoma. It is composed of a low grade chondrosarcoma and a high grade non-cartilagenous sarcomatous component. Due to the aggressive nature of the disease, its prognosis is poor. [1]

Dedifferentiated chondrosarcomas most frequently harbor alterations in TP53, IDH1, IDH2, CDKN2B, and CDKN2A [2].

Most Commonly Altered Genes in Dedifferentiated Chondrosarcoma

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, IDH1 Mutation, and IDH1 Codon 132 Missense are the most common alterations in dedifferentiated chondrosarcoma [2].

Top Alterations in Dedifferentiated Chondrosarcoma

Significant Genes in Dedifferentiated Chondrosarcoma

CCND1 +

CCND2 +

CCND3 +

CDK4 +

CDK6 +

CDKN2A +

EWSR1 +

Disease Details

Parent(s)
Chondrosarcoma
OncoTree Name
Dedifferentiated Chondrosarcoma
OncoTree Code
DDCHS

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 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.