Overview

NCI Definition: A malignant cartilaginous matrix-producing mesenchymal neoplasm arising from the bone and soft tissue. It usually affects middle-aged to elderly adults. The pelvic bones, ribs, shoulder girdle, and long bones are the most common sites of involvement. Most chondrosarcomas arise de novo, but some may develop in a preexisting benign cartilaginous lesion. [1]

Chondrosarcomas most frequently harbor alterations in IDH1, TP53, CDKN2A, IDH2, and ATRX [2].

Most Commonly Altered Genes in Chondrosarcoma

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

Top Alterations in Chondrosarcoma

Significant Genes in Chondrosarcoma

AKT1 +

AKT2 +

AKT3 +

CCND1 +

CCND2 +

CCND3 +

CDK4 +

CDK6 +

CDKN2A +

CRKL +

EGFR +

ERBB2 +

IDH1 +

IRS2 +

KIT +

MET +

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

MTOR +

PDGFRA +

PIK3CA +

PIK3CG +

PIK3R1 +

PIK3R2 +

PMS1 +

PMS2 +

PTEN +

RICTOR +

RNF43 +

RPTOR +

TSC1 +

TSC2 +

Disease Details

Synonyms
CHONDROSARCOMA, MALIGNANT, Soft tissue chondrosarcoma
Parent(s)
Malignant Connective and Soft Tissue Neoplasm
Children
Clear Cell Chondrosarcoma, Localized Chondrosarcoma, Periosteal Chondrosarcoma, Myxoid Chondrosarcoma, Mesenchymal Chondrosarcoma, and Dedifferentiated Chondrosarcoma
OncoTree Name
Chondrosarcoma
OncoTree Code
CHS

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.