类似卵巢性索肿瘤的子宫肿瘤4例及其临床病理特征OACSTPCD

Objective:Uterine tumor resembling ovarian sex cord tumor(UTROSCT)is a rare tumor that occurs in the uterine corpus.This study aims to investigate the clinicopathological features,diagnosis,and differential diagnosis of UTROSCT. Methods:The clinicopathological data,immunohistochemistry,and fluorescence in situ hybridization of 4 cases diagnosed with UTROSCT in the Third Affiliated Hospital of Zhengzhou University from January 2020 to June 2023 were retrospectively analyzed to summarize the characteristics of the disease. Results:The patients'ages ranged from 44 to 59 years(median age 46.5),3 patients presented with abnormal menstruation,and 1 was discovered during a routine examination.Tumor diameters ranged from 3.0 to 5.0 cm(median 4.1 cm),with 3 cases located submucosally and 1 within the intermuscular wall.Color Doppler ultrasonography showed heterogeneous hypoechoic patterns in all cases.Two cases underwent hysteroscopic electrosurgery for submucosal leiomyoma,1 case underwent laparoscopic total uterus with bilateral salpingectomy,and 1 case underwent laparoscopic total uterus with double adnexa resection.All 4 patients were followed up for 2 to 42 months(median 33.5 months),with no recurrence or metastasis observed.Histologically,the tumor cells were arranged in sheets,nests,trabeculae,and cords.Three tumors consisted of epithelioid cells of medium size with moderate or scant cytoplasm,some of which were clear.One tumor consisted of epithelioid and foamy cells,where the foamy cells were uniform in size with abundant cytoplasm.The nuclei of both cell types were round or oval,with fine chromatin and visible nucleoli.All 4 tumors showed mild with no evident necrosis,intravascular tumor thrombus,or pathological mitosis.Immunohistochemistry showed tumor cells expressing vimentin(4/4),cytokeratins(CK)(3/4),estrogen receptor(ER)(4/4),progesterone receptor(PR)(4/4),cluster of differentiation 10(CD10)(3/4),and at least 2 gonadotropic markers:calretinin(3/4),steroidogenic factor-1(SF-1)(2/4),cluster of differentiation 99(CD99)(4/4),cluster of differentiation 56(CD56)(4/4),α-inhibin(2/4),and Wilms'tumor gene 1(WT-1)(3/4).All tumors expressed the myogenic markers smooth muscle actin(SMA)(4/4)and desmin(4/4),but not epithelial membrane antigen(EMA)(0/4).The proliferation index Ki-67 ranged from 5%to 10%.Fluorescence in situ hybridization assay showed NCOA3 gene breakage in cases 3 and 4(2/4),with no breakage in cases 1 and 2,and no JAZF1 gene breakage was observed in case 2(0/1). Conclusion:UTROSCT is a very rare uterine tumor predominantly found in middle-aged women,possessing low malignant potential.Its presentation on color Doppler ultrasonography as a heterogeneous hypoechoic mass,combined with histopathological,immunohistochemical,and molecular changes,aids in its diagnosis and differential diagnosis.