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Advances in the diagnosis and treatment of uterine sarcomas

Abstract: Uterine sarcomas account for less than 10% of all uterine neoplasms (Tropé et al., 2012). The most common include uterine leiomyosarcoma and the endometrial stromal neoplasms. The diagnosis requires pathologic review of the uterus in order to characterize extent of myometrial invasion. However, molecular diagnosis has aided the classification of endometrial stromal neoplasms, especially in helping to discriminate between endometrial stromal and undifferentiated endometrial sarcoma. The prognosis of these tumors following surgery varies, with endometrial stromal sarcoma associated with a better prognosis compared to leiomyosarcoma or undifferentiated endometrial sarcoma. For aggressive sarcomas, there is interest in adjuvant treatment, which has focused on the evaluation of systemic agents. However, the rarity of these tumors makes the conduct of prospective trials difficult and no consensus adjuvant regimen has emerged. In the absence of Level I data, the use of chemotherapy is based on institutional preferences. Ongoing clinical trials will help inform the standard treatment approach for these tumors, and we encourage patients with uterine sarcoma to participate in well-designed clinical trials.

Complete remission achieved by oophorectomy for recurrent endometrial stromal sarcoma after laparoscopic morcellation


Preoperative differentiation of endometrial stromal sarcoma (ESS) from myoma is challenged by the similarities in their clinical presentations and the unreliability of imaging studies. Often, ESS is diagnosed postoperatively after laparoscopic power morcellation of a presumed myoma (Amant et al., 2014 and Park et al., 2011). In approximately 80% of ESS cases diagnosed postoperatively after morcellation, preoperative diagnosis was either leiomyoma or adenomyosis (Park et al., 2011). Laparoscopic morcellation of ESS with an electromechanical morcellator can result in the dissemination of the tumor (Park et al., 2011 and Badia and Karini, 2010), but there has been no report of the spread of ESS after morcellation with a single incision on the uterus using a harmonic scalpel. Furthermore, if the ovaries are preserved at the time of the initial surgery, bilateral salpingo-oophorectomy (BSO) for recurrence is recommended (Amant et al., 2014 and Yoon et al., 2014), but the effect of BSO for recurrent ESS has not been reported. Here, we present a case where morcellation with a simple incision in total laparoscopic hysterectomy for a presumed myoma led to the peritoneal dissemination of ESS; and complete remission was achieved and laparoscopically confirmed after BSO for recurrent ESS.

Current treatment options in ESS

Source: Ihnen M, Mahner S, Jänicke F, Schwarz J. Int J Gynecol Cancer 2007; 17:957-963.

Uterine sarcomas are a rare form of uterine cancer. They occur in women from 40 to 60 years and are generally characterized by poor prognosis, a high rate of local recurrence, and distant metastases. Endometrial stromal sarcoma (ESS) accounts for 0.2% of all gynecological malignancies. Forms of possible treatment include surgery, radiotherapy, chemotherapy, and endocrine treatment. Randomized trials analyzing these treatment options are limited due to the rarity of this disease; therefore, a standard therapy could not be established thus far. To present an overview of the current treatment options of ESS, a search of Medline, Embase, and the Cochrane Library was performed and the results concluded. We report the case of a 32-year-old woman who presented with FIGO stage II ESS. Initial treatment with tamoxifen and local perfusion with cisplatin resulted in disease progression and were discontinued. A novel, therapeutic approach using two cycles of combination chemotherapy with doxorubicin and ifosfamide followed by surgery was applied. Five years after surgery, the patient is still in complete remission. Thus, we conclude that although there is no data from randomized trials available, chemotherapy in advanced or metastatic ESS can provide an opportunity for surgical treatment and can lead to long-term remission.

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Management of patients with uterine sarcoma: A debated clinical challenge

From PubMed

Uterine sarcomas include a heterogeneous group of rare tumours that usually have an aggressive clinical behaviour and a poor prognosis. Total abdominal hysterectomy and bilateral salpingo-oophorectomy represents the standard surgical treatment. Pelvic and/or para-aortic lymphadenectomy is indicated for carcinosarcoma, but not for leiomyosarcoma and undifferentiated endometrial sarcoma. Some recent data on low numbers of patients with low-grade endometrial stromal sarcoma appear to show an incidence of nodal involvement higher than previously expected, thus suggesting a role for lymphadenectomy in this malignancy. Carcinosarcoma also requires a comprehensive surgical peritoneal staging. Postoperative treatment of uterine sarcomas has been long debated.

Adjuvant pelvic radiotherapy appears to improve local control without any significant impact on overall survival. There is little evidence in the literature supporting the use of adjuvant chemotherapy in any gynaecological sarcomas except for carcinosarcomas. However, uterine sarcomas have a high tendency to develop distant recurrences, and recent data on adjuvant chemotherapy in soft tissue sarcomas are promising.

Potential alternative progestin therapy for low-grade endometrial stromal sarcoma: A case report


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Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine tumor that sometimes recurs and advances. Hormonal treatment, especially high-dose progestins and aromatase inhibitors (AIs), has demonstrated efficacy against these tumors. Because the standard treatment period is uncertain and hormonal treatment is effective, hormonal agents are likely to be used long-term, especially when a residual tumor is present. However, the long-term use of high-dose progestins and AIs may cause thromboembolism, as well as musculoskeletal stiffness and pain. Dienogest, a relatively new progestin, has demonstrated safety after long-term administration; it also appears to have a more favorable long-term safety profile compared with other progestins and AIs. We encountered a young patient with recurrent LG-ESS that metastasized to the liver and exhibited resistance to high-dose medroxyprogesterone acetate (MPA). The patient was successfully treated with dienogest monotherapy. This is the first report describing the efficacy of dienogest against recurrent and metastatic LG-ESS that is resistant to MPA and other agents.

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