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]

Significant Genes in Clear Cell Sarcoma of Soft Tissue

ATF1 +




MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

PMS1 +

PMS2 +


Disease Details

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