title: Systemic therapy for endometrial stromal sarcomas: current treatment options.
abstract: Uterine endometrial stromal sarcomas including true low-grade endometrial stromal sarcoma (LG-ESS) and high-grade (HG-ESS) or undifferentiated endometrial sarcoma (UES) constitute a group of rare, aggressive malignancies. Most LG-ESSs express steroid receptors. Surgery is the principal primary therapy for endometrial stromal sarcomas and should be considered in all cases. These malignancies are relatively radio- and chemoresistant. Chemotherapy is used in recurrent and advanced HG-ESS and UES. Currently, the combination of gemcitabine and docetaxel is considered the most effective regimen, but at the expense of substantial toxicity. In steroid receptor positive advanced LG-ESS hormonal therapy, mainly with progestins, allows in some patients for a long-term survival. Aromatase inhibitors seem to be equally effective as first- and subsequent-line of treatment, and are well tolerated. The role of molecular-targeted therapies in endometrial stromal sarcomas remains to be established.
authors.affiliation: Department of Oncology and Radiotherapy, Medical University of Gdansk, Poland. kserkies@gumed.edu.pl.
authors.forename: Krystyna
authors.initials: K
authors.lastname: Serkies
authors.forename: Ewa
authors.initials: E
authors.lastname: Pawłowska
authors.forename: Jacek
authors.initials: J
authors.lastname: Jassem
mesh_terms: D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D000074322:Antineoplastic Agents, Immunological / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016889:Endometrial Neoplasms / Q000601:surgery / Q000628:therapy*; D005260:Female; D006801:Humans; D058990:Molecular Targeted Therapy; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy; D010166:Palliative Care; D018203:Sarcoma, Endometrial Stromal / Q000601:surgery / Q000628:therapy*
chemical_list: D018931:Antineoplastic Agents, Hormonal; D000074322:Antineoplastic Agents, Immunological
keywords: chemotherapy; endocrine therapy; endometrial stromal sarcoma; targeted therapy