女性中肾管残件肿瘤临床病理观察

Female Wolffian adnexal tumor:a case report and review of the literature

目的探讨女性中肾管残件肿瘤的临床病理学特征、诊断和鉴别诊断,以提高对该肿瘤的认识。方法对1例中肾管残件肿瘤进行常规病理检查、HE、组织化学及免疫组织化学染色(EnVision法),显微镜下观察,并结合文献进行讨论。结果患者女,40岁,体检发现盆腔包块一周。巨检:肿瘤位于右侧输卵管系膜,大小为7.0cm×5.0cm×5.0cm,包膜完整,切面淡黄色,局部呈囊性,囊内少量淡黄色清亮液体。显微镜下肿瘤细胞呈4种不同方式排列:紧密排列的小管状、弥漫实性、筛状或多囊性,细胞形态较温和,核分裂象罕见。免疫组织化学显示肿瘤细胞vimentin、AE1、α-in-hibin、CK19和CD99弥漫阳性,CK和CK7部分阳性,Ki-67核阳性指数<5%,CK5/6、CK20、calretinin、CD10、EMA、PR和ER均阴性。结论女性中肾管残件肿瘤是一种非常罕见的肿瘤,临床症状缺乏特异性,极易误诊。诊断主要依靠组织病理学及免疫组织化学,并应与支持-间质细胞肿瘤、子宫内膜样腺癌、粒层细胞瘤和腺瘤样瘤等其他妇科肿瘤进行鉴别诊断。

Objective To investigate the clinicopathological features and differential diagnosis of female Wolffian adnexal tumor.Methods Routine pathologic examination,H-E,histochemical,immunohistochemical stains and microscopy were utilized to analyze a case of Wolffian adnexal tumor with review of the relevant literature.Results A 40-year-old female was admitted to the hospital with physical examination showing a pelvic mass for one week.Grossly,the tumor was located in the right mesosalpinx,which was 7.0 cm×5.0 cm×5.0 cm in size and well encapsulated.The cut surface of the tumor was pale-yellow with focal cysts full of primrose liquid.Microscopically,the tumor cells were arranged in four patterns:closely packed tubules,solid,sieve-like and multicyst.The nuclei were cytologically bland and the mitoses were rare.Immunohistochemically,the tumor cells were diffusely positive for vimentin,AE1,α-inhibin,CK19 and CD99,partially positive for CK and CK7,Ki-67 labeling index <5%,but negative for CK5/6,CK20,calretinin,CD10,EMA,PR and ER.Conclusions Female Wolffian adnexal tumor is rare and easily misdiagnosed as other tumors owing to lack of characteristic symptoms.Definite diagnosis of this tumor mostly bases on pathological examination and immunohistochemical analysis.In addition,the differential diagnosis of this tumor includes Sertoli-Leydig cell tumors,endometrioid adenocarcinoma,granulose cell tumors and adenomatoid tumors.