Issue 3/2026
Dimitrova, K., Milev, Z.
PSAGBAL «St. Sofia» – Sofia
Endometrial stromal sarcoma (ESS) is a rare mesenchymal tumor (less than 1% of all uterine malignancies) that usually affects peri- and postmenopausal women (40–55 years). The current WHO classification divides these tumors into four categories based on histopathological and molecular criteria. Low-grade ESS (LG-ESS) usually has an indolent course and expresses estrogen and progesterone receptors, while high-grade ESS (HG-ESS) is highly aggressive and usually does not express these receptors. The diagnosis of ESS remains a serious challenge for clinical practice, as D&C is often insufficient to distinguish a benign endometrial stromal nodule from LG-ESS, necessitating immunohistochemical testing.
The presented clinical case of a 25-year-old patient, with a life-threatening anemic syndrome and persistent bleeding, illustrates that ESS can manifest itself at an extremely young age, often mimicking benign conditions. The performed immunohistochemical testing played a key role in establishing the proper diagnosis – hormone-sensitive LG-ESS. Despite the patient’s young age, the severity of the symptoms and the high risk of recurrence justified the radical surgical treatment performed in this case.