Uterine sarcomas comprise endometrial stroma tumors (EST) and uterine leiomyosarcomas
(ULMS), with ESTs accounting for less than 2% and ULMS accounting for
approximately 1% of uterine neoplasms. Recent classifications of ESTs denote as many as
four categories: benign endometrial stromal nodule (ESN), low-grade endometrial stromal
sarcomas (ESS), high-grade endometrial stromal sarcomas (HGESS), and undifferentiated
uterine sarcomas (UUS). The designation UUS has been suggested to encompass undifferentiated endometrial
sarcomas (UES) and undifferentiated uterine sarcomas of other tissue origins. The prognosis for ESS is good.
Although a third of cases recur, the 5-year survival rate for Stage I disease can be as high as 98%. In contrast
the prognosis for UES is poor, with a 5-year survival rate for Stage I disease at 57%. Few patients with UES
survive two years. ULMS represents the most common single type of uterine sarcoma and is associated with a
poor prognosis. Recurrence rates are estimated at 50-70% and the 5-year survival rate for Stage I approximates
51%. The authors present a review of these tumors from the vantage point of clinical work up and diagnosis,
treatment options, and adjuvant therapy including hormonal therapy, radiation therapy, and chemotherapy.
Included are accounts of pertinent phase II and III clinical trials. The article concludes with commentary
on future treatment directions for these rare tumors.
Keywords: Stromal, sarcoma, leiomyosarcoma, undifferentiated, TP53, JAZF1/SUZ12, YWHAEFAM22,
hormone, chemotherapy, pazopanib.
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