NCI Definition: A rare malignant neoplasm with melanocytic differentiation characterized by the presence of polygonal or spindle shaped clear cells. This sarcoma usually affects the tendons and aponeuroses and is associated with a poor prognosis due to recurrences and metastases. [1]

Clear cell sarcoma of soft tissues most frequently harbor alterations in EWSR1, ATF1, CREB1, CDKN2A, and SRSF2 [2].

Most Commonly Altered Genes in Clear Cell Sarcoma of Soft Tissue

EWSR1 Fusion, EWSR1-ATF1 Fusion, ATF1-EWSR1 Fusion, EWSR1-CREB1 Fusion, and CREB1-EWSR1 Fusion are the most common alterations in clear cell sarcoma of soft tissue [2].

Top Alterations in Clear Cell Sarcoma of Soft Tissue

Significant Genes in Clear Cell Sarcoma of Soft Tissue

ATF1 +





Disease Details

Clear cell sarcoma - not kidney, Malignant Melanoma of Soft Parts, Soft tissue clear cell sarcoma, Clear cell sarcoma/Malignant melanoma of soft parts (excluding Clear cell sarcoma of the kidney), Malignant Melanoma of the Soft Parts, Clear Cell Sarcoma of Soft Parts, Chordoid Sarcoma
Soft Tissue Sarcoma
OncoTree Name
Clear Cell Sarcoma
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.