Abstract
Endometrial stromal sarcomas (ESS) are rare tumors of endometrial stromal origin that account for approximately 1% of all uterine malignant neoplasms and are responsible for a significant proportion of mortality due to uterine malignancies. There are immense case reports on low-grade ESS, but there is a paucity of data on high-grade ones, probably, because these cases generally present in advanced stages and have a high case fatality rate. Moreover, there have been several refinements in the classification of these tumors. We, herein, provide an update on this topic and discuss the poignant outcome of a case of high-grade ESS.