Management of sarcomas of the uterus

Uterine sarcomas are rare malignancies accounting for 8-10% of all uterine malignancies, but are significantly more aggressive and have worse prognosis. Management of uterine sarcomas including leiomyosarcoma (LMS), endometrial stromal sarcoma, high-grade undifferentiated sarcoma and adenosarcoma is reviewed.

Uterine carcinosarcomas are staged and treated similarly to high-grade epithelial endometrial carcinomas and are no longer considered uterine sarcomas. Gemcitabine/docetaxel with doxorubicin holds promise for the treatment of LMS. A recently developed nomogram was demonstrated to predict disease recurrence in patients with LMS which may allow us to identify a subset of patients who are likely to recur and target this population for adjuvant systemic therapy. Cytogenetic abnormalities have been identified that allow differentiation of endometrial stromal sarcomas from high-grade undifferentiated uterine sarcomas which may be useful in pathologically difficult cases.

Uterine sarcomas are a heterogeneous group of tumors. To date, limited advancements have been made in discovering targeted therapies to these tumors. Chemotherapy with gemcitabine/docetaxel followed by doxorubicin holds promise in the treatment of LMS. Given the rarity of these tumors and the lack of clinical trials to guide management, patients with uterine sarcomas should be encouraged to enroll on clinical trials.