Fertility-sparing management of low-grade endometrial stromal sarcoma: A systematic review of oncologic and reproductive outcomes

Abstract

Objective: Low-grade endometrial stromal sarcoma (LG-ESS) is a rare malignancy and the standard of care, precludes future childbearing. Although fertility-sparing treatment (FST) may be considered in carefully selected patients, high-quality evidence regarding its efficacy and safety is limited. This review aims to systematically evaluate the oncologic and reproductive outcomes associated with conservative treatment for LG-ESS. Methods: Pubmed Database, Scopus Database and Embase Database were screened in September 2024 from the first publication about women with LG-ESS treated with a surgical FST. We included the studies containing data about oncologic, and reproductive outcomes. This study adheres to PRISMA guidelines and is registered with PROSPERO (CRD42024605140). The quality of the studies was assessed using the Newcastle-Ottawa scale. Results: 9 studies fulfilled inclusion criteria, and 89 patients were analyzed. Recurrence was observed in 51 out of 89 patients (57.3 %) with a mean recurrence-free interval ranging between 3 and 40.5 months. A mortality rate of 1.1 % was observed, with a mean follow-up duration ranging from 38.5 to 84.5 months. The overall pregnancy rate was 41.5 % and the live birth rate was 78.1 %. The preterm delivery rate was 8 % and 3.9 % of patients required assisted reproduction technology. Conclusions: Considering the limitations of the available evidence, FST in women with LG-ESS carries a relatively high risk of tumor relapse, though it does not increase the risk of death. Fertility outcomes seem to be encouraging. Resection of the malignant uterine lesion combined with adjuvant hormonal treatment may be considered for selected early-stage patients, with close follow-up.