Abstract

Simple SummaryUterine sarcomas can affect patients of reproductive age. In this setting, the chance of a fertility-sparing treatment would allow women to become pregnant. In the literature, only a few experiences of fertility-sparing treatment of uterine sarcomas have been reported; however, the oncological safety and reproductive outcomes remain unclear. The aim of this systematic review is to report and summarize all the published evidence about the fertility-sparing approach in these rare and heterogenous tumors, and to help physicians in making clinical decisions.Uterine sarcomas are rare cancers, sometimes diagnosed in women of childbearing age. Hysterectomy is the standard treatment in early stages. The option of lesion removal to save fertility is described in the literature, but it is still considered experimental. The objective of this systematic review is to report on the available evidence on the reproductive and oncological outcomes of fertility-sparing treatment in women with uterine sarcomas. PubMed, Scopus and Cochrane Central Register of Controlled Trials were searched between 1 January 2011 and 21 June 2021 for publications in English about women with uterine sarcoma treated with a fertility-sparing intervention. Thirty-seven studies were included for a total of 210 patients: 63 low-grade endometrial stromal sarcomas, 35 embryonal rhabdomyosarcomas of the cervix, 19 adenosarcomas, 7 leiomyosarcomas and 2 uterine tumors resembling an ovarian sex cord. Conservative treatment ensured pregnancy in 32% of cases. In terms of oncological outcomes, relapse was related to histology and the worst prognosis was reported for leiomyosarcoma, followed by low-grade endometrial stromal sarcoma, which relapsed in 71% and 54% of cases, respectively. The highest death rate was associated with leiomyosarcoma (57.1%). This study demonstrated that fertility-sparing treatments may be employed in selected cases of early stage uterine sarcoma.

Highlights

  • Uterine sarcomas represent an extremely malignant and rare group of heterogeneous tumors which grow from the uterine body and account for about 3% of all cancers in this organ [1]

  • Adnexa are systematically removed in postmenopausal women, whereas it is recommended to remove them before menopause in case of sarcoma histotypes more susceptible to hormones, such as low-grade stromal sarcomas and adenosarcomas

  • The included population was composed of women with a histological diagnosis of uterine sarcoma who wanted to preserve the reproductive function and were treated with a fertility-sparing intervention, in Cancers 2021, 13, 5808 which the uterus and at least one ovary and fallopian tube were preserved

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Summary

Introduction

Uterine sarcomas represent an extremely malignant and rare group of heterogeneous tumors which grow from the uterine body and account for about 3% of all cancers in this organ [1]. The rarity and histopathologic heterogeneity of uterine sarcomas make the classification of risk factors, the definition of prognosis and the best treatment strategy very difficult. These are conditions that occur more frequently during menopause (60%), even though a minority of them are women of childbearing age [3]. The high recurrence rate of LMS provides the rationale for postoperative treatment; neither adjuvant cytotoxic chemotherapy nor adjuvant radiotherapy have been shown to reduce the risk of relapse in early stages [5]

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