Sarcomas of the Uterus
Sarcomas of the uterus are rare; they comprise only 3-5% of all uterine tumors. These tumors primarily arise from two tissues: endometrial sarcomas from endometrial glands and stroma and leiomyosarcomas from the uterine muscle itself. Other sarcomas arise in supporting tissues and are very rare. Sarcomas have been classified as pure (composed of one cell type only) and mixed (composed of more than one cell type). The site of origin has also been used in the classification of sarcomas, with homologous tumors defined as those that contain tissue elements entirely indigenous to the uterus and heterologous tumors defined as those that contain tissue elements foreign to the uterus. Although several classifications have been suggested and used over the years, a simplified classification is endorsed by the Gynecologic Oncology Group and has been used successfully:
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• Leiomyosarcomas
• Endometrial stromal sarcomas
• Mixed homologous müllerian sarcomas (carcinosarcomas)
• Mixed heterologous müllerian sarcomas (mixed mesodermal sarcoma)
• Other uterine sarcomas
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Particularly with leiomyosarcomas, the diagnosis of this entity is determined almost solely on the basis of mitotic index (the number of mitoses per 10 high-power fields). Cellular atypia has also been used by some investigators.
Sarcomas are usually found in postmenopausal women; leiomyosarcomas are identified about 10 years earlier than mixed mesodermal sarcomas and endometrial stromal sarcoma. Menorrhagia and postmenopausal bleeding are common symptoms. Abdominal pain or mass is a frequent complaint. Particularly in the postmenopausal patient, a rapidly enlarging uterus should suggest sarcoma as a possible etiology. A large friable polypoid mass extending through a dilated cervix into the vagina also should raise suspicion of sarcoma, particularly in the postmenopausal patient. A mixed mesodermal tumor and endometrial stromal sarcoma can usually be diagnosed with a directed or endometrial biopsy, whereas a leiomyosarcoma may or may not be identified preoperatively.
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Total abdominal hysterectomy and bilateral salpingo-oophorectomy are considered the hallmarks of therapy for uterine sarcomas. In many instances, patients with sarcomas tend to have extrauterine disease, which may be present intraperitoneally or in the lymph nodes. This is particularly true for homologous and heterologous mixed müllerian tumors. Although radiation therapy has been used for a long time in patients with uterine sarcoma, it has not been associated with any improvement in survival compared with surgery alone. There does appear to be less local recurrence after radiation therapy, but because most patients that have recurrence have it at distant sites, the overall survival has not been affected. Adjunctive therapy with chemotherapeutic agents has been evaluated; however, none have been shown to improve survival even in patients with limited disease. Unfortunately, many patients with stage I uterine sarcomas will develop a recurrence. The use of adjuvant chemotherapy has to date minimally affected the survival of these patients. Preliminary data regarding the use of ifosfamide, mesna, and cisplatin suggest encouraging response rates, but their impact on survival awaits further evaluation.
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