Uterine sarcoma

Uterine sarcomas are a rare and heterogeneous group of aggressive tumors originating from the mesenchymal tissues of the uterus. They encompass several several histological subtypes, including leiomyosarcomas, endometrial stromal sarcomas, undifferentiated uterine sarcomas, and adenosarcomas.

Uterine sarcomas are characterized by recurrent genetic events, most commonly chromosomal rearrangements that create oncogenic gene fusions. Molecular markers such as RACGAP1 have been identified as metastatic drivers in uterine carcinosarcomas, mediating motility and invasion via regulation of STAT3 phosphorylation and survivin expression. S6S240 phosphorylation (reflecting mTOR pathway activation) is associated with higher grade and recurrence.

Uterine sarcomas are rare, comprising 2-6% of all uterine malignancies with an incidence of 0.5-4.4 per 100,000 women-years, and most cases occur after age 50.

Risk factors for uterine sarcomas include a history of tamoxifen use, pelvic irradiation, obesity, oral contraceptives, and menopausal use of estrogens and progestins.

Most patients with uterine sarcomas are symptomatic at diagnosis, with abnormal vaginal bleeding being the most frequent presenting symptom. Other symptoms may include vaginal discharge, pelvic pain, and pelvic mass. Uterine sarcomas typically appear as solid masses with inhomogeneous echogenicity on ultrasound, sometimes with irregular cystic areas, with moderate or very strong vascularization.

Uterine sarcomas are highly aggressive, but a subset of patients may experience long-term survival. The epithelial component of uterine carcinosarcomas determines prognosis by causing the majority of metastases and vascular invasion. The 5-year survival ranges from 20% to 75%, depending on the subtupe. Low-grade endometrial stromal sarcomas have the most favorable prognosis, with an overall survival of 80-100%.