Surgery

• We recommend extrafascial abdominal hysterectomy with bilateral salpingo-oophorectomy and surgical staging (peritoneal washings for cytology and paraaortic lymph node dissection or sampling) as initial treatment for stage I through IVa uterine sarcomas if fertility is not an issue

• We suggest fertility-preserving surgery in young women who wish to preserve childbearing potential and have a low-grade leiomyosarcoma or a smooth muscle tumor of uncertain malignant potential .

Adjuvant therapy — Adjuvant radiation therapy (RT) appears to enhance local control following surgery; whether it provides a survival benefit remains unclear. Adjuvant chemotherapy is of uncertain benefit.

Stage I and II

• We suggest adjuvant pelvic RT for improved local control in patients with stage I and II  carcinosarcomas, high-grade leiomyosarcomas and undifferentiated uterine sarcomas. We suggest not using whole abdominal radiation therapy rather than pelvic RT for carcinosarcomas

• We suggest not using adjuvant chemotherapy for resected stage I or II uterine sarcomas, including carcinosarcoma. However, others disagree, citing the overall poor prognosis and retrospective studies that suggest improved survival from the use of adjuvant chemotherapy in this setting. Some clinicians (including one of the authors, SM) routinely offer systemic chemotherapy in addition to pelvic RT for medically fit patients who can tolerate this treatment. Guidelines from the NCCN recommend adjuvant chemotherapy for resected stage I or II carcinosarcomas but not for other subtypes

Stage III and IVa

• We suggest adjuvant systemic therapy rather than RT alone for most patients with stage III or IVa uterine sarcomas. In selected circumstances (ie, patients with pelvic nodal metastases and no evidence of disease in the paraaortic nodes or in the peritoneum), after a thorough discussion with a well-informed patient, we suggest RT alone

• There is no consensus as to the choice of adjuvant chemotherapy regimen. We suggest a platinum-based chemotherapy regimen for patients with carcinosarcoma and undifferentiated sarcomas. Our preferred regimen is paclitaxel, doxorubicin and cisplatin (the TAP regimen) as used in GOG 177. For leiomyosarcomas, we suggest docetaxel plus gemcitabine

• We suggest hormone therapy with megestrol acetate for resected stage III and IVa endometrial stromal sarcomas. We start with 40 mg twice daily initially, increasing to 80 mg twice daily if tolerated. We treat for at least six to 12 months, and if well-tolerated, treatment is continued indefinitely. As with the other histologies, pelvic radiation may be added for better local control.

• Pelvic radiotherapy may be added for improved local control; however, myelotoxicity may be severe in patients receiving cytotoxic chemotherapy followed by RT. If the paraaortic lymph nodes are involved with tumor, and there is no evidence of extranodal abdominal disease or disease outside of the abdomen or pelvis, we suggest extended field irradiation to encompass the sites of known nodal metastases in order to achieve better local control