Abstract
BackgroundThe prognosis of recurrent low-grade endometrial stromal sarcoma (LGESS) is little known. This study was to investigate the survival outcomes of a cohort of patients with recurrent LGESS.MethodsPatients with primary LGESS diagnosed and treated for first recurrence confirmed by histology in the study center from February 2012 to June 2019 were retrospectively included. The progression-free interval (PFI) after the last treatment for first recurrence and overall survival (OS) since the diagnosis of first recurrence, which were followed up to June 1, 2020, were compared between groups of various therapy modalities.ResultsFifty-six patients were included, and 43 patients (76.8%) had definite follow-up outcomes. The 5-year PFI and OS rates were 30.0% (95% confidence interval [95% CI] 29.2–30.8) and 75.0% (68.0–82.0), respectively. In univariate analysis, only fertility-sparing treatment, ovarian preservation and surgical treatment had a significant impact on the PFI (hazard ratio [HR] 4.5, 3.1, and 0.2; 95% CI 1.5–13.1, 1.3–7.3, and 0.1–0.7; and p = 0.006, 0.009 and 0.006, respectively), but no factor was found to be associated with increased mortality risk. After adjusted with hormone treatment or chemotherapy, surgical treatment had significant effectiveness on OS (HR 0.3 and 0.3, 95% CI 0.1–1.0 and 0.1–1.0, p = 0.045 and 0.049, respectively). None of the patients with fertility-sparing treatment had successful conception, and all experienced repeated relapse.ConclusionFor patients with recurrent LGESS, fertility-sparing treatment or ovarian preservation should not be provided. Surgery is the treatment of choice, and hormone treatment and/or chemotherapy was effective for the survival benefits of surgical treatment.
Highlights
The prognosis of recurrent low-grade endometrial stromal sarcoma (LGESS) is little known
We discovered that estrogen and progesterone receptor expression or mitotic activities had no significant impact on the survival outcomes of recurrent LGESS patients
Fertility-sparing surgery or ovarian preservation treatment resulted in an inferior progression-free interval (PFI) without successful conception, and should not be offered to patients with recurrent LGESS
Summary
The prognosis of recurrent low-grade endometrial stromal sarcoma (LGESS) is little known. Dai et al Orphanet J Rare Dis (2021) 16:160 for measurable residual or recurrent low-grade ESS has a high response rate [7]. Because of their efficacy and minimal adverse effects, letrozole [8, 9] and medroxyprogesterone [10, 11] have been used to treat patients with recurrent or residual LGESS that is difficult to resect surgically. Patients seem to benefit from surgical removal of metastatic lesions, especially pulmonary lesions, followed by progestin therapy [12] These reports had limited sample sizes of patients with recurrent LGESS and restricted effective decision-making with recurrent patients with LGESS. Since fertility-sparing and ovarian preservation surgeries have been shown to be associated with an increased risk of recurrence in LGESS patients [13, 14], we paid special attention to the impact of these issues on the PFI after recurrence
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