Uterine sarcomas. A critical review of the literature

This review aims to provide a comprehensive description of surgical approaches for the management of uterine sarcomas. Uterine sarcomas are rare uterine neoplasms. Frequently, diagnosis is made after hysterectomy or myomectomy scheduled for presumed benign leiomyomas. The gold standard for surgical treatment of uterine sarcomas is hysterectomy with bilateral salpingo-oophorectomy. It is possible to adopt a fertility-sparing approach for those patients who wish to maintain their fertility. The role of pelvic lymphadenectomy is controversial; in fact, removal of lymph nodes is only recommended in the case of radiological suspicion of nodal involvement. Use of a morcellator is associated with increased risk of total recurrence, intra-abdominal recurrence and death. Advanced disease management should be customized based on the patient's performance status given the uncertain role of adjuvant chemotherapy. Treatment of advanced or recurrent disease remains a subject of debate, but surgery is the best approach in terms of morbidity and mortality. There are few options for management of these uterine tumours, and further studies are needed to clarify the diagnostic and therapeutic pathways of patients with a first diagnosis of uterine sarcoma and patients with relapse of uterine sarcoma. No specific evidence supports the adoption of adjuvant therapy in uterine-confined disease, and molecular/genomic profiling may be useful to identify patients at risk of recurrence.