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. [1]

High-grade endometrial stromal sarcomas most frequently harbor alterations in ZRSR2, CDKN2A, YWHAE, PTEN, and PBRM1 [2].

Most Commonly Altered Genes in High-Grade Endometrial Stromal Sarcoma

CDKN2A Loss, ZRSR2 Loss, YWHAE-NUTM2B Fusion, PBRM1 Mutation, and NUTM2B-YWHAE Fusion are the most common alterations in high-grade endometrial stromal sarcoma [2].

Top Alterations in High-Grade Endometrial Stromal Sarcoma

Disease Details

Uterine sarcoma, favor high-grade endometrial stromal sarcoma, YWHAE-FAM22 ESS, Uterine Sarcoma Favor High-Grade Endometrial Stromal Sarcoma, Uterine Corpus High Grade Endometrial Stromal Sarcoma, YWHAE-FAM22 Endometrial Stromal Sarcoma, High Grade ESS, Uterine Corpus High Grade ESS
Uterine Corpus Endometrial Stromal Sarcoma
OncoTree Name
High-Grade Endometrial Stromal 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.