NCI Definition: A morphologic variant of chondrosarcoma arising from bone and soft tissue. It is characterized by the presence of malignant small round cells, biphasic growth pattern, and well differentiated hyaline cartilage. Clinical presentation includes pain and swelling. The clinical course is aggressive, with local recurrences and distant metastases. [1]

Mesenchymal chondrosarcomas most frequently harbor alterations in NCOA2, ATRX, INSR, HEY1, and YES1 [2].

Most Commonly Altered Genes in Mesenchymal Chondrosarcoma

NCOA2 Fusion, ATRX Mutation, INSR T557M, HEY1-NCOA2 Fusion, and YES1 Amplification are the most common alterations in mesenchymal chondrosarcoma [2].

Top Alterations in Mesenchymal Chondrosarcoma

Significant Genes in Mesenchymal Chondrosarcoma

HEY1 +


Disease Details

Small Cell Sarcoma
Central Nervous System Mesenchymal Chondrosarcoma and Mesenchymal Chondrosarcoma of Bone
OncoTree Name
Mesenchymal Chondrosarcoma
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.