Objective Uterine tumors with sex cord-like differentiation were first described in 1945 by Morehead and Bowman.Subsequently, in 1976, Clement and Scully classified these neoplasms into two groups depending on their clinical and histopathological features: Group Ⅰ, endometrial stromal tumors with sex cord-like elements (ESTSCLEs);and Group Ⅱ, uterine tumors resembling ovarian sex cord tumors (UTROSCTs).ESTSCLEs are predominantly made up of endometrial stromal cells alongside focal sex cord differentiation, with sex cord-like aggregates comprising <50% of the mass.UTROSCTs are composed mainly or exclusively of a sex cord-like component.The current World Health Organization classification defines UTROSCTs as miscellaneous tumors, whereas ESTSCLEs are characterized as similar to low-grade endometrial stromal sarcoma.UTROSCTs have been considered rare neoplasms of uncertain malignancy with polyphenotypic immunohistochemical expression,although they typically exhibit benign behavior.In most instances, UTROSCTs are discovered only after hysterectomy or polypectomy.Furthermore, current literature focuses mainly on the pathologic features and diagnosis of UTROSCTs, and there is scant information available on the clinical characteristics and outcomes of UTROSCTs.In this study, we compare the clinicopathological features, diagnosis, treatment, and prognosis of two types of uterine sex cord-like tumors.Methods The clinicopathological features of four uterine tumors resembling ovarian sex cord tumors (UTROSCTs) and two endometrial stromal tumors with sex cord-like elements (ESTSCLEs) were analyzed retrospectively.Results All patients were premenopausal women.The most common clinical presentation was vaginal bleeding (four cases).Total hysterectomy with or without bilateral adnexectomy was the most common treatment pattern (five cases).A patient with UTROSCTs, presenting with recurrence 10 months after transvaginal submucous myomectomy, underwent a total hysterectomy (case 2).All tumors were polypoid or intramural masses, usually located in the uterine fundus or submucosa.The majority of UTROSCTs were positive for cytokeratin (4/4 cases), one was positive for Wilms tumor protein, and of two cases with smooth muscle actin immunoreactivity, two were positive for desmin.UTROSCTs were positive for two or more sex cord markers, whereas sex cord markers were less frequently detected in ESTSCLEs.CD10 was variably positive in two UTROSCT patients and strongly positive in all ESTSCLE patients.Three UTROSCTs and one ESTSCLE were positive for both estrogen and progesterone receptors.All patients with UTROSCTs were alive without evidence of recurrence.One patient with ESTSCLEs underwent postoperative chemotherapy after total vaginal hysterectomy but developed recurrence at the vaginal stump (case 5).The other patient with ESTSCLEs was lost to follow-up.Conclusion UTROSCTs are rare neoplasms, which have a distinct clinical outcome and pathological features when compared with ESTSCLEs.However, there is little data available to guide clinical management.UTROSCTs are tumors with low malignant potential that have been shown to recur in rare cases;however, to our knowledge no deaths have been reported in relation to UTROSCTs.An infiltrative border, vascular invasion, frequent mitotic figures, serosal rupture, stromal predominance,and cytologic atypia are associated with UTROSCT recurrence.Further studies are needed to understand the malignant potential of UTROSCTs.