类似卵巢性索肿瘤的子宫肿瘤6例临床病理分析

目的 探讨类似卵巢性索肿瘤的子宫肿瘤(uterine tumor resembling ovarian sex cord tumor,UTROSCT)的临床病理学特征、分子遗传学改变及其鉴别诊断.方法 回顾性分析6例UTROSCT的临床病理学特征、免疫表型等.采用FISH检测JAZF1基因及NCOA2基因易位情况,并复...

Uterine tumor resembling ovarian sex cord tumor:a clinicopathologic analysis of 6 cases

Purpose To investigate the clinicopathologic and genetic features,pathologic diagnosis and differential diagnosis of uterine tumor resembling ovarian sex cord tumor(UTROSCT).Methods Six cases of UTROSCT were collected.The clinicopathologic features were summarized,and the JAZF1 and NCOA2 gene analyses were performed by FISH.Results All the 6 patients were middle-aged and elderly women,aged from 43 to 58 years(mean 51.3 years).The tumors measured in maximum diameter from 2 cm to 11 cm and showed gray-white to yellow and tan on cut surface.Microscopically,themorphology of the tumor included mutually anastomosed strips,trabeculae,tubules,reticular and solid structures.The tumor stroma was sparse with hyaline degeneration.There were a varying number of arteriovenous vessels,and no obvious spiral arterioles in the stroma.The neoplastic cells showed obvious nucleolus and vacuolar chromatin.Nuclear grooves could be seen occasionally.Mitotic activity was generally low.By immunohistochemistry,these tumors have a polyphenotypic immunoprofile.All tumors showed strong and diffuse reactive for at least two markers of sex cord differentiation(CD99,CD56,Calretinin,WT-1,α-inhibin)and showed variable positivity for at least one smooth muscle marker(desmin,H-Caldesmon,SMA).Five cases expressed vimentin,four expressed CKpan and three expressed CD10.S-100 and HMB-45 were negative in all six tumors.Ki-67 proliferation index was about 1%-30%.There were no JAZF1 and NCOA2 gene associated translocation in 4 cases.Follow-up data was available in 5 cases(range 5 to 61 months)were all free of disease.Conclusion UTROSCT is a rare uterine tumor with multiple immunophenotypes.The diagnosis mainly depends on the histological features and immunohistochemical findings.Surgical resection is the main treatment,and usually has a good prognosis.