A multi-institutional review of outcomes of endometrial stromal sarcoma

The Ohio State University, Columbus, Ohio, United States
Gynecologic Oncology (Impact Factor: 3.77). 07/2007; 105(3):630-4. DOI: 10.1016/j.ygyno.2007.01.031
Source: PubMed


To compare the clinical behavior and outcomes of low- and high-grade endometrial stromal sarcomas (LGESS and HGESS), respectively.
Patients with endometrial stromal sarcoma from five different institutions were identified and reviewed for clinicopathologic variables, surgical management and outcomes. Statistical calculations including Chi-square, t-test and survival using the Kaplan-Meier method with the log rank test were performed.
One hundred and five patients were identified with 72 having LGESS, 31 with HGESS and 2 having unclassified tumors. The mean age was 50 years for patients with LGESS and 64 years for those with HGESS (p<0.0001). In patients with LGESS, 68% (49 patients) had disease confined to the uterine corpus or cervix compared to 39% (12 patients) in HGESS (p=0.002). The median overall survival was 53 months for HGESS and had not yet been reached in LGESS with 87.8% alive at 80 months (p<0.0001). In HGESS patients with extrauterine disease, the presence of residual disease greater than 2 cm had a significant effect on median survival. Median survival was 52 months for those who underwent optimal cytoreduction versus 2 months for those with suboptimal residual disease (p=0.007). The impact of cytoreduction was not seen in LGESS patients with extrauterine disease with 82.1% alive at 78 months.
Low-grade and high-grade endometrial stromal sarcomas represent two distinct clinical entities and should be treated as such. Survival in patients with high-grade tumors appears to be related to amount of residual disease at the completion of initial surgery and would suggest the need for aggressive cytoreduction. The role of surgical staging and optimal adjuvant therapy remains unclear.

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    • "Recurrence occurs in up to half of the patients, sometimes many years after the initial diagnosis. The most frequent sites of recurrence include pelvis and abdomen.2,14 In the present study, 5 year survival rate was 88.6% and 10-year survival rate was 74.2 % (Fig.1). "
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    ABSTRACT: Objective: To evaluate clinico-pathological features and prognostic valuses of Endometrial stromal sarcomas (ESS) through comparison of the two grade groups (low- and high-grade disease). Methodology: We retrospectively analyzed the medical records of 27 patients who were diagnosed with ESS at a single institute between March 1988 and November 2009. Our retrospective chart review was approved by our local institutional Review Board (IRB). Results: The median age of the patients was 44.0 years, the median follow-up period was 101.0 months and the 10-year survival rate was 74.2%. The median uterine weight was 215.0 gm. Twenty-three (70.4%) and four patients (29.6%) had low- and high-grade disease, respectively. As primary treatment, twenty-four (70.4%) and three patients (11.1%) underwent type I hysterectomy and type III hysterectomy, respectively. Total six cases were recurred and two cases of the six-recurred patients were distant metastasis (lung) and four cases were died of the disease. Univariate analysis revealed that the histologic grade and the uterine tumor weight were significantly related with longer disease-free survival (p=0.025 and 0.043 respectively). Conclusion: ESSs with high-grade or larger tumor size have to be carefully and sufficiently managed, because of its rarity and aggressive behavior. To determine the proper adjuvant treatment of ESS with high risks, further clinical data should be collected and studied.
    Full-text · Article · Mar 2013 · Pakistan Journal of Medical Sciences Online
    • "The receptor concentration and the predominant isoform may vary in ESS originating in the uterus versus extra uterine sites, such as to make the latter less hormone responsive.[35–38] Tamoxifen, as well as hormonal replacement therapy containing estrogens are contraindicated in patients after treatment of ESS.[1739–41] Advanced/metastatic ESS also can be treated with antioestrogen therapy, with an aromatase inhibitor or progestagen.[18] Maluf FC and Petal S suggest a dose of 2.5 mg letrazole daily for recurrent cases of ESS.[42] Due to the rarity of ESS, it is difficult to conduct prospective randomized clinical trials for determining the optimal treatment regimen. "
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    ABSTRACT: Endometrial stromal sarcomas are rare malignant tumors of the uterus, and most of the information available in literature is based on small series or case reports. A proper preoperative diagnosis is difficult and in most cases the diagnosis is confirmed after hysterectomy for a presumed benign disease. Endometrial sampling, ultrasound, and magnetic resonance imaging can provide diagnostic clues. Total hysterectomy with bilateral salpingo-oopherectomy is the main line of management and for early disease complete cure is a reality. Ovarian conservation may be possible in young women with early stage disease and the role of lymphadenectomy is controversial. Adjuvant hormone therapy in the form of progesterone, gonadotropin releasing hormone analogues, and aromatase inhibitors are found to be effective in preventing recurrences. Hormone therapy, radiotherapy and surgical excision of the metastasis are recommended for recurrences.
    No preview · Article · Mar 2012 · Indian journal of medical and paediatric oncology
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    • "Endometrial stromal sarcomas represent 0.2% of all genital tract malignancies and were previously subdivided into low- or high-grade categories based upon their mitotic index. High-grade endometrial stromal sarcomas are now termed poorly differentiated or undifferentiated uterine sarcomas due to its aggressive clinical course [5–10]. "
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    ABSTRACT: Endometrial stromal sarcoma (ESS) formerly classified as low-grade endometrial stromal sarcoma is a rare uterine malignancy with a good prognosis despite a tendency to recur. Primary surgical management for ESS includes total abdominal hysterectomy and bilateral salpingo-oophorectomy. Patients with ESS have long disease-free survival rates when treated with primary surgical therapy, but nearly fifty percent of these patients will recur. We present the case of a patient with recurrent ESS who had an excellent response to combined therapy with megestrol and leuprolide.
    Full-text · Article · Jun 2010 · Sarcoma
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