NCI Definition: A rare malignant soft tissue neoplasm of uncertain differentiation, characterized by the presence of chondroblast-like cells in a myxoid stroma and a multinodular growth pattern. The most common sites of involvement are the deep soft tissues of the extremities, particularly the thigh. It usually presents as an enlarging soft tissue mass. Patients may have long survivals, but local recurrences and metastases occur in approximately half of the cases. The most common site of metastasis is the lungs. [1]

Extraskeletal myxoid chondrosarcomas most frequently harbor alterations in NR4A3, EWSR1, TP53, SMAD2, and GPS2 [2].

Most Commonly Altered Genes in Extraskeletal Myxoid Chondrosarcoma

NR4A3-EWSR1 Fusion, NR4A3 Fusion, EWSR1-NR4A3 Fusion, EWSR1 Fusion, and TP53 Loss are the most common alterations in extraskeletal myxoid chondrosarcoma [2].

Top Alterations in Extraskeletal Myxoid Chondrosarcoma

Disease Details

Extraosseous Chondrosarcoma, Myxoid Extraskeletal Chondrosarcoma, Extraskeletal Chondrosarcoma, EMC, Myxoid Extraosseous Chondrosarcoma
Soft Tissue Sarcoma
Intracranial Extraskeletal Myxoid Chondrosarcoma
OncoTree Name
Extraskeletal Myxoid 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.