Ovary and uterus-sparing procedures for low-grade endometrial stromal sarcoma: A retrospective study of 153 cases☆,☆☆
Introduction
Endometrial stromal sarcoma (ESS) is a rare tumor that represents approximately 7–15% of all uterine sarcomas but only 0.2% of all uterine malignancies [1]. Based on the mitotic rate, ESS is histologically divided into two groups: high-grade (HG) and low-grade (LG) [2]. HG-ESS is currently defined as an undifferentiated endometrial sarcoma (UES), characterized by more than 10 mitoses per 10 high-power fields (HPFs). Additionally, this sarcoma is more aggressive and has a poorer prognosis. In contrast, LG-ESS has fewer than 10 mitoses per 10 HPFs, and the cell nuclei are not atypical or pleomorphic [2]. LG-ESS is relatively more common and tends to occur before menopause. LG-ESS usually exhibits a more indolent clinical course, but has high relapse potential [3]. These two distinct entities should be treated differently.
For LG-ESS, hysterectomy is the cornerstone of treatment. However, the role of a bilateral salpingo-oophorectomy (BSO), as well as lymphadenectomy for complete surgical staging, is debated. Adjuvant treatment, including hormonal treatment, chemotherapy and radiotherapy, has also not been established. The absence of consensus on the optimal management of this disease is due to its rarity and the heterogeneity of previously published series, the majority of which included HG tumors and other histologic subtypes of uterine sarcoma [4]. In the present study, we focused exclusively on LG-ESS and compiled 153 cases that were treated at our center, representing one of the largest series that has been published. Our experience in managing this uncommon condition and a review of related literature will also be discussed.
Section snippets
Patients and methods
The medical records of women with LG-ESS who were diagnosed and treated at Peking Union Medical College Hospital (PUMCH) were collected. All consecutive patients who underwent surgery and had complete pathology and operation reports were included in the study, whereas patients who were lost to follow-up immediately after surgery were excluded. Clinical data, including clinicopathological variables, treatment, and follow-up information, were then evaluated.
In our series, hysterectomy was the
Statistical analysis
All statistical analyses were performed using SAS® Version 9.2 (SAS Institute, Cary, NC). All tests were 2 sided, and P < 0.05 was considered statistically significant. The Kaplan–Meier method was used to analyze the relapse and survival rates. A log rank test was used to compare the different survival curves. A Cox proportional hazards model was used to assess all parameters that were found to be significant in univariate analysis.
Demographic characteristics and clinical presentation
From July 1979 to May 2013, 196 consecutive women with ESS were treated at PUMCH. In total, 32 cases were diagnosed as UES and were excluded from this study. A total of 11 patients (6.7%) were lost to follow-up immediately after surgery, 7 of whom preferred to continue treatment at hospitals near their residences due to their economic conditions. For the other 4 patients, relevant data were not available in their records. All of these patients were excluded from the analysis. Therefore, 153
Discussion
Although LG-ESS is relatively indolent, late recurrence and distant metastases may occur [3], [6], [7], [8], [9], [10]. The recurrent risk may be as high as 50% [6], [7]. In our study, the 10-year OS rate was 95.8%, which is relatively satisfactory, whereas the 10-year RFS rate was as low as 49.6%. The mean relapse interval was approximately 4.1 years; however, relapse may occur as late as 12.1 years after the primary treatment. Given the disease course, a management strategy that controls the
Conclusions
The present study demonstrated that for LG-ESS patients, hysterectomy with BSO and complete resection of the macroscopic lesion should be treated as mainstay initial and salvage treatments. Ovary-sparing procedures do not compromise survival but increase the risk of relapse; thus long-term follow-up should be mandatory for young patients undergoing this procedure. Myomectomy should only be considered for young patients with a strong desire for future fertility, with fully informed consent;
Conflict of interest statement
The authors have no conflict of interest to declare.
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Source of the study: Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College (PUMCH).
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Grant support: National Science and Technology Infrastructure Program “The National Key Technologies R&D Program of China” (Grant 2008BAI57B02).