子宫苗勒腺肉瘤伴性索样分化临床病理观察

Uterine Müllerian adenosarcoma with sex cord-like differentiation: a case report and review of literature

目的探讨子宫苗勒腺肉瘤伴性索样分化（MASCE）的临床病理学特征、免疫表型、诊断与鉴别诊断。方法对1例MASCE进行HE和免疫组化染色,并复习相关文献。结果患者女性,56岁。绝经后阴道出血。巨检：肿瘤位于宫腔内,7 cm×4 cm×3 cm大小灰白色肿物,突向宫腔,切面灰红、灰白色,肿物基底部与子宫肌层界限尚清。镜检：肿瘤由良性腺体和富于细胞的间叶成分组成。良性腺体为子宫内膜样腺体,呈囊状扩张,部分腺体伴鳞状上皮化生。间质富于细胞,围绕腺体呈袖套状分布。间质成分类似于低级别子宫内膜间质肉瘤,有轻度异型,可见核分裂,核分裂象约2~3个/10HPF。肉瘤样间质内可见条索状、腺泡状排列的细胞,呈多角形,胞质较丰富或空泡状,细胞形态温和。免疫组化：腺上皮和间质细胞ER和PR（＋）,围绕腺体的梭形细胞CD10强（＋）,而远离腺体的梭形细胞CD10（-）。肉瘤样成分内的条索状和腺泡状排列的细胞inhibin-α（＋）,calretinin局灶性（＋）,而CD68、desmin、SMA、S-100蛋白和HMB45（-）,Ki-67增殖指数为10%。结论 MASCE是一种比较少见的子宫上皮-间叶混合性肿瘤,生物学行为为低度恶性,需与子宫内膜间质肉瘤伴性索样分化和子宫性索样肿瘤等鉴别。

Purpose To explore the clinicopathologic features, immuuophenotype, diagnosis and differential diagnosis of uterine Mollerian adenosareoma with sex cord-like differentiation （MASCE）. Methods One case of MASCE was studied with HE and immunohistochemical staining, and the relevant literatures were reviewed. Results A 56-year-old woman presented with post-menopause vaginal bleeding. Grossly, the tumor located in the uterine cavity and projected into the uterine cavity measuring 7 cm×4 cm× 3 cm. Grey-white and grey-red in the cut surface was seen. Microscopically, the tumor was composed of benign glands and cellular mesenchymal elements. The benign glands were endometrioid with cystic dilatation and squamous metaplasia. The stroma] ceils were spindle-shaped and slightly atypical, periglandular stromal cuffing was obvious. The stromal component of the tumor resembled a low-grade endometrial stromal sarcoma. The sarcomatous cells showed mild atypia and had mitotic rates that ranged from 1 to 3 MFs/10 HPFs. The polygonal cells with abundant or clear cytoplasm arranged in cords and aeini resembling ovarian sex cord-like elements were seen within sarcomatous stromal component. Immunohistochemically, the glandular and stromal components were positive for ER and PR. The spindle cells surrounding glands were positive for CD10, but negative for CD10 away from glands. The ovarian sex cord-like elements within the sarcomatous component were positive for inhibin - a, focal expression of calretinin, but negative for CD68, Desmin, SMA, S-100, and HMB45. The proliferating index labeled by Ki-67 was about 10%. Conclusions MASCE is a rare mixed epithelial and mesenchymal tumor of uterine with low-grade malignant biological behaviour. It should be differentiated from low-grade endometrial stromal sarcoma with sex cord-like differentiation and uterine tumor resembling ovarian sex cord tumor.