A giant metastatic low-grade endometrial sarcoma requiring surgical management

Low-grade endometrial stromal sarcomas are relatively rare tumors. We here report a case of a woman presenting with a giant metastatic low-grade endometrial stromal sarcoma with thromboembolic complications requiring urgent surgical management.

A 58-year-old obese female was admitted, with a voluminous abdominopelvic mass, due to complications related to its size and extent. The tumor derived from the uterus and invaded the ureters, bladder and rectum. It compressed the right iliac vessels causing both deep vein thrombosis and pulmonary embolism. She developed a painful irreducible umbilical. We proceeded with a debulking surgery (hysterectomy with bilateral salpingo-oophorectomy). Histological findings were consistent with a low-grade endometrial stromal sarcoma.

Low-grade endometrial stromal sarcomas are generally low-grade malignant neoplasms with an indolent clinical course. Surgery is the cornerstone of treatment. In low-income countries, malignancies are more often diagnosed at a late stage, which limits therapeutic options. Cytoreduction is recommended in advanced tumors with extrauterine manifestation, depending on symptoms and with palliative intent.

Low-grade endometrial stromal sarcomas are indolent uterine malignancies with metastatic potential. Even in advanced cases, cytoreduction must be considered.