Prognostic factors and outcomes in endometrial stromal sarcoma with the 2009 FIGO staging system: A multicenter review of 114 cases
Introduction
Endometrial stromal sarcomas are rare, pure mesenchymal tumors, comprising about 0.2% of all uterine malignancies and 10–25% of all uterine sarcomas [1]. Endometrial stromal sarcomas were formerly divided into low-grade and high-grade tumors with the degree of differentiation determined by a threshold of 10 mitoses per 10 HPF [2]. However, high-grade tumors have distinct histology and clinical properties compared with low-grade tumors. Currently, the term endometrial stromal sarcoma (ESS) refers only to low-grade ESS according to the 2003 World Health Organization (WHO) classification.
ESSs are generally characterized by indolent behavior and a favorable prognosis. The 5-year overall survival (OS) rates of ESS patients is reported to be 80–100% [3]. In addition, in most patients, the disease is diagnosed at an early stage; 65–86% of ESSs were confined to the uterus at diagnosis [4], [5], [6]. However, late recurrence is common even in patients with early stage disease, with more than one-third of patients developing recurrent disease [3], [5], [6]. Surgery including total abdominal hysterectomy and bilateral salpingo-oophorectomy (BSO) is considered the standard initial therapy [1], [4], [7]. However, the merits of BSO in young premenopausal patients remain controversial. The role of lymphadenectomy and the prognostic significance of lymph node metastasis have not been clearly defined [3], [8], [9]. In adjuvant treatment, radiotherapy, chemotherapy, and hormonal therapy are the current options. However, the optimal adjuvant treatment remains unclear [7].
Due to the rarity of these tumors and the heterogeneity including high-grade tumors or various histologic subtypes of uterine sarcoma of published studies, existing data for ESS are limited to evaluations of the natural history and optimal management of ESS. Furthermore, there is no obvious consensus regarding prognostic factors for ESS.
Since 1988, uterine sarcomas including ESS had been staged by the same staging system as uterine corpus carcinoma. In 2009, the International Federation of Gynecologists and Obstetricians (FIGO) introduced a distinct staging system for leiomyosarcoma and ESS, and some studies have validated the new staging system for these tumors [10], [11].
Therefore, the aim of this multicenter study was to enhance understanding of ESS and to assess the prognostic factors associated with overall survival (OS), recurrence-free survival (RFS), and survival after recurrence in ESS by applying the 2009 FIGO staging system.
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Study population
This multicenter retrospective study included patients with ESS (formerly low-grade endometrial stromal sarcoma) who were treated at the Samsung Medical Center (SMC, Seoul, Republic of Korea) and Asan Medical Center (AMC, Seoul, Republic of Korea). From January 1990 to January 2012, data of patients in whom ESS was diagnosed were collected from the electronic medical records under institutional review board approval at both institutions. We only included patients with low-grade tumors who had
Results
During this study period, a total of 117 patients with ESS were identified. Three patients were excluded because of concomitant gastric cancer (one patient) and correction of the histologic type to undifferentiated uterine sarcoma (two patients) by review of the pathology slides. The remaining 114 patients were included in the study.
Baseline characteristics of these patients are presented in Table 1. The median age at diagnosis of patients was 45 years (range: 18–74). Of the 114 patients, 88
Discussion
This study is one of the largest cohorts of homogenous ESS describing the prognostic factors with a comprehensive review of the clinical course of the disease according to the 2009 FIGO staging system. In this study we found that BSO was independently associated with good recurrence-free survival in patients with ESS. Particularly, in the premenopausal patients, BSO was significantly associated with longer RFS. In addition, patients who underwent cytoreductive resection of recurrent ESS
Conflict of interest statement
The authors declare that there are no conflicts of interest.
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2021, Gynecologic OncologyCitation Excerpt :While some studies reported lymphadenectomy as an independent predictor of improved survival [11,12], others failed to show any impact of lymphadenectomy on disease outcomes [3,21,22]. LN metastasis in LGESS has been reported to range from 6.6% to 16.6% in previous studies [3,10,19], which was 14.8% in our study. Similar to studies of endometrial carcinoma [23,24], we found that more than a half of patients (75%) with pelvic LN metastasis had positive paraaortic LNs.
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Contributed equally to this work.