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Uterine sarcoma neoplasms that are responsible for a large majority of uterine cancer–associated deaths.
The subtypes include leiomyosarcomas, endometrial stromal tumors, and adenosarcomas.
Standard treatment includes complete surgical resection.
Adjuvant treatment with chemotherapy, hormonal therapy, or radiation may be considered in patients with high-risk disease.
The ability of adjuvant treatment to improve overall survival in patients with uterine sarcomas is unclear, and there is no standard recommendation regarding adjuvant therapy.
Adjuvant radiation appears to improve local control but has no significant impact on survival.
Sarcomas comprise less than 5% of all uterine corpus tumors.
They account for a disproportionate percentage of deaths from uterine cancers.
Mortality rate from uterine sarcomas was reported to be as high as 29%.
Survival is dependent on stage.
The estimated 5-year survival rate for stage I uterine leiomyosarcoma is 76%, while it is 60% for stage II, 45% for stage III, and 29% for stage IV disease.
WHO classification system divides uterine sarcomas into mesenchymal tumors, and mixed epithelial and mesenchymal tumors.
Mesenchymal tumors include leiomyosarcomas, endometrial stromal sarcomas, undifferentiated endometrial sarcomas, and smooth muscle tumors of uncertain malignant potential.
Mixed epithelial and mesenchymal tumors include carcinosarcomas, Müllerian adenosarcomas, carcinofibromas, adenofibromas, and adenomyomas.
Leiomyosarcomas are the most common uterine sarcomas, followed by endometrial stromal sarcomas, undifferentiated endometrial sarcomas, and Müllerian adenosarcomas.
The incidence rate for leiomyosarcoma of 0.4 per 100,000 women,.
The incidence rate for endometrial stromal sarcomas of 0.3 per 100,000 women.
Patients do not have identifiable risk factors, however, there are germline mutations, including p53 and Rb, that are associated with an increased risk for leiomyosarcoma.
Studies suggest an increased risk for uterine sarcoma in women with a history of obesity, diabetes mellitus, younger age at menarche, or exposure to tamoxifen.
Preoperative diagnosis of uterine sarcoma is often difficult as patients with early-stage uterine sarcoma will present with the same set of symptoms associated uterine leiomyomata including: postmenopausal bleeding, abnormal uterine bleeding, abdominal or pelvic pain and/or pressure, bloating or distention, constipation, enlarging abdomen, urinary symptoms, and abnormal or foul-smelling vaginal discharge.
Some women are totally asymptomatic.
Endometrial sampling is considered a standard preoperative diagnostic test in uterine neoplasms, but uterine sarcomas originate in the myometrium and performance of endometrial biopsies has a low predictive value for identification of uterine sarcomas.y
In patients with inconclusive MRI who underwent needle biopsy demonstrated sensitivity, specificity, and positive and negative predictive values for the procedure of 91.7%, 100%, 100%, and 96.2%, respectively.
Imaging with ultrasound, CT, or MRI may identify a uterine mass, but no imaging modality has been shown to accurately differentiate between leiomyomatas and uterine sarcomas.
Uterine masses that increase in size after menopause are concerning for malignancy.
Elevation of LDH isoenzyme type 3, may be useful in making a diagnosis of leiomyosarcoma.
Surgery for uterine sarcoma is to remove all sites of disease and for purposes of accurate staging.
In most cases, surgery is the pref2241ed initial management.
For patients with early-stage leiomyosarcoma, surgery consists of total hysterectomy and surgical cytoreduction of intra-abdominal and retroperitoneal disease.
Routine lymphadenectomy is unnecessary, and only suspicious lymph nodes should be removed.
A bilateral salpingo-oophorectomy is usually performed at the time of total hysterectomy, particularly in perimenopausal and postmenopausal women, but has no survival advantage
With locally advanced or limited metastatic disease, an attempt to resect all disease may improve survival.
Radiation is used for local control.
Patients who undergo morcellation for presumed benign disease, and who are subsequently diagnosed with leiomyosarcoma, have worse outcomes compared with women who undergo hysterectomy.
Power morcellation is associated with an increased risk of tumor recurrence.
Morcellation is associated with fragmentation and spread of leiomyosarcoma in the intraperitoneal cavity.
Stage I and II leiomyosarcomas have a 50% to 70% risk of recurrence.
More than half of recurrences are extrapelvic recurrences, often multisite and terminal.
Aduvant RT for Stage I and II May yield improved local control of disease; however, they have not demonstrated a change in overall survival.
For stage I and II leiomyosarcoma, studies have not shown a survival benefit for adjuvant chemotherapy.
The combination of fixed-dose-rate gemcitabine plus docetaxel, followed by doxorubicin, was evaluated in (Sarcoma Alliance for Research through Collaboration (SARC) protocol 005) as adjuvant therapy for resected stage I and II leiomyosarcoma: Median follow-up was 39.8 months, 46% of patients developing recurrent disease.