Gynecologic Sarcomas

Abstract

Overview Sarcomas are extremely rare and account for less than 1.5% of gynecologic cancers. Carcinosarcoma and leiomyosarcoma each account for 35–40% of uterine sarcomas, with endometrial stromal sarcoma (ESS) accounting for 10–15% and other sarcomas including adenosarcomas comprising 5–10%. Uterine carcinosarcoma should be classified as a metaplastic carcinoma of the uterus. Most adenosarcomas and ESSs have good prognosis and respond to hormonal therapy. Undifferentiated endometrial sarcoma (UES) and adenosarcomas with sarcomatous overgrowth are rare and have poor prognosis and require chemotherapy. ESS are histologically and clinically distinct from UES and each have distinct gene rearrangements. More than 50% of Stage I leiomyosarcomas (LMSs) and carcinosarcomas patients will recur. Chemotherapy is required for advanced or recurrent disease. In LMSs, the active drugs are doxorubicin, ifosfamide, gemcitabine, and docetaxel. For uterine carcinosarcomas, the drugs of choice are ifosfamide, cisplatin/carboplatin, and paclitaxel. Adjuvant radiation therapy may provide locoregional control in select cases of uterine carcinosarcomas. Hormonal therapy, including progestational agents, GnRH analogues, and aromatase inhibitors, has a role in the treatment of advanced or recurrent low‐grade ESSs and adenosarcomas.