Based on this evidence, treatment of most stage I and II uterine sarcomas should include hysterectomy, bilateral salpingo-oophorectomy, and treatment of the pelvic lymphatics by radiation or surgery. Strong consideration should be given to the use of adjuvant chemotherapy to decrease the incidence of distant metastases. Stage III uterine sarcomas are probably best treated by an aggressive combined approach of surgery, radiation therapy, and chemotherapy, while patients with stage IV disease are candidates for combination chemotherapy.
Surgery The first step in the treatment of early uterine sarcoma should be exploration. Because extirpative survey is the most important aspect of treatment, and knowledge of the extent and spread of the disease is important for further management, one should not forgo or delay surgery by using radiation therapy or chemotherapy first. At the time of surgery, the peritoneal cavity should be carefully explored and peritoneal washings obtained. Special attention should be given to the pelvic and para-aortic lymph nodes; lymphadenectomy should be performed with ESS and carcinosarcoma, but may be omitted in leiomyosarcoma, in which the risk of lymph node metastases is low and the therapeutic and diagnostic value is questionable (428). Total abdominal hysterectomy is the standard procedure, and bilateral salpingo-oophorectomy should be performed in all patients except premenopausal women with leiomyosarcoma. Based on the surgical and pathologic findings, additional therapy with radiation therapy or chemotherapy can be planned. Rarely, a patient may be cured by excision of an isolated pulmonary metastasis (455,456).
Radiation Therapy Several studies showed that adjuvant preoperative or postoperative radiation therapy is beneficial in decreasing pelvic recurrences and increasing quality of life in patients with localized ESS and carcinosarcoma, but not with leiomyosarcoma (457–465). Radiation was not demonstrated to improve survival. One trial randomized patients with uterine sarcomas to pelvic radiotherapy versus observation. Althoughthe risk of local relapse significantly decreased from 24% to 14%, there was no difference in survival between groups (466). The GOG randomized patients to whole abdomen radiation versus three cycles of cisplatin, ifosfamide, and mesna in patients with less than 1 cm of residual disease (467). Whole-abdomen radiation was associated with significant toxicity and the chemotherapy regimen appeared to confer a nonsignificant survival advantage.
Chemotherapy Several chemotherapeutic agents have activity in sarcomas, including vincristine, actinomycin D, cyclophosphamide, doxorubicin, dimethyl triazeno imidazole carboxamide (dacarbazine, DTIC), cisplatin, ifosfamide paclitaxel, gemcitabine, and liposomal doxorubicin (468). Doxorubicin appears to be the most active single agent in the treatment of leiomyosarcoma, producing a 25% response rate (469). Ifosfamide has a lesser degree of activity (470). Cisplatin and ifosfamide demonstrated clear activity in carcinosarcoma, with response rates of 18% to 42% and 32%, respectively (471–473). Doxorubicin demonstrated less than a 10% response rate in carcinosarcoma (469). Paclitaxel yielded an 18% response rate with carcinosarcoma, but had limited activity in leiomyosarcomas (474,475). Gemcitabine and liposomaldoxorubicin showed activity in leiomyosarcomas (476,477).