Low-grade endometrioid ovarian and endometrial tumors are unique clinical entities and their molecular characteristics affect their biology and clinical course. Although low-grade endometrioid ovarian tumors are rare, low-grade endometrioid endometrial carcinomas are common among uterine tumors. These tumors are often diagnosed at an early stage in women of childbearing age
thus, the selection of patients for conservative treatment is crucial. Synchronous tumors are not rare in this sub-group of patients and might represent a challenge for treatment. In the setting of advanced/recurrent disease, both these histologies are poorly represented in large randomized clinical trials
thus, their management is often based on evidence in the field of low-grade serous or high-grade endometrioid histology. The molecular characterization of these tumors has provided further patient stratification with relevant implications for clinical management. Given the paucity of available data, there are several controversies regarding the diagnosis and management of these tumors, from the correct identification of the primary tumor to the surgical approach and medical treatment of the recurrent/advanced disease. This review aims to provide an overview of the main controversial issues on this topic, along with the evidence currently available to guide clinical management, with particular interest in recent and future clinical trials.