Abstract: Objective：To explore the clinicopathological characteristics, immunophenotype, diagnosis, differential diagnosis, treatment and prognosis of ovarian stromal tumor with minor sex cord elements. Methods：Four cases of ovarian stromal tumor with minor sex cord elements were studied with histopathological and immunohistochemical staining, and their clinical and pathological findings were analyzed. The clinical and pathological data of 9 cases reported in the related literature were reviewed and summarized. Results：Ovarian stromal tumors with minor sex cord elements are frequently seen in middle-aged and elderly women. Patients often experience abdominal discomfort and vaginal bleeding. The tumors occur mostly in one of the ovaries. The maximum diameter of the tumor was larger than 4 cm. Histologically, the tumor was comprised mainly of fibro-thecoma component with few sex cord elements (less than 10%). Sex cord components varied in appearance between fully differentiated granulosa cells and tubular structures resembling immature Sertoli cells. All of the four cases showed positive staining for α-inhibin，CD99, and Calretinin. Four patients were followed up to the present and all of them showed no tumor survival. Nine cases in the literature were followed up to the publishment of  related references, and all exhibited no tumor survival. Conclusions：The diagnosis of ovarian stromal tumor with minor sex cord elements is based on distinctive histopathology and immunohistochemical staining. Its clinical manifestation is similar to other sex cord-stroma tumors. Surgical resection is the main method of treatment, and the prognosis of the tumor is good.

Key words: Ovarian neoplasms, Sex cord-gonadal stromal tumors, Pathology, clinical, Stromal tumor with minor sex cord elements