Low-Grade Endometrial Stromal Sarcoma

Epidemiology

• Endometrial stromal sarcoma (ESS) is rare; it accounts for less than 0.5% of all uterine malignancies and only 10% to 15% of mesenchymal uterine malignancies.

• ESS tends to occur in younger women more than other mesenchymal uterine tumors and is typically seen in women in their 40s and 50s.

• The so-called low-grade endometrial stromal sarcomas (LGESSs) account for the majority of these tumors; high-grade and undifferentiated types are rare and are discussed separately.