High-Grade Endometrial Stromal Sarcoma
Associated Genetic Biomarkers
NCI Definition: A rare, high grade sarcoma that arises from the endometrial stroma. It is characterized by round cell morphology. It was previously also known as undifferentiated uterine sarcoma. In 2014, high grade endometrial stromal sarcoma was reclassified and is currently considered a distinct and rare neoplasm. It appears to have a prognosis that falls between low grade endometrial stromal sarcoma and undifferentiated sarcoma. 
High-grade endometrial stromal sarcomas most frequently harbor alterations in ZRSR2, CDKN2A, YWHAE, PTEN, and PBRM1 .
CDKN2A Loss, ZRSR2 Loss, YWHAE-NUTM2B Fusion, PBRM1 Mutation, and NUTM2B-YWHAE Fusion are the most common alterations in high-grade endometrial stromal sarcoma .
There is 1 clinical trial for high-grade endometrial stromal sarcoma, of which 1 is open and 0 are completed or closed. Of the trial that contains high-grade endometrial stromal sarcoma as an inclusion criterion, 1 is phase 2 (1 open).
Deficient, MLH1, and MSH2 are the most frequent gene inclusion criteria for high-grade endometrial stromal sarcoma clinical trials .
Nivolumab is the most common intervention in high-grade endometrial stromal sarcoma clinical trials.
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.