Overview

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 +

AXL +

EWSR1 +

MET +

RET +

Disease Details

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

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 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.