Uterine tumor resembling ovarian sex-cord tumor (UTROSCT) is a rare distinct cancer included in the current World Health Organization classification of endometrial stromal tumors. A battery of immunohistochemical markers are necessary for accurate diagnosis. Although few case reports and case series have been documented and therefore providing robust prognostic information several authors agree to consider UTROSCT as a low malignant potential tumour. In literature only five cases of conservative management were reported. We reported our experience and a review of conservative cases of literature. In this article we describe a series of 10 cases of UTROSCT and their clinical and pathologic feature. We report two cases of conservative surgical approach obtaining a successfully pregnancies. All of the patients are still alive. In conclusion, fertility sparing surgery should be offered to patients who wish preserve their fertility however radical surgery must be considerate after childbirth. Close follow-up is required for all patients due to the lack of prognostic biomarkers and long follow up is needed to evaluate safety of conservative surgery.
