作　　者： (王婧）; (吴焕文）; (梁智勇）;

机构地区： 中国医学科学院北京协和医院病理科,北京100730

出　　处： 《诊断病理学杂志》 2017年第8期561-570,共10页

摘　　要： 目的探讨卵巢类固醇细胞瘤(SCT)的临床病理特征、免疫表型、诊断与鉴别诊断及治疗与预后。方法对19例卵巢SCT的临床表现、病理学形态、免疫组化等进行观察,并复习相关文献。结果患者年龄18~63岁(平均41岁),常因男性化表现就诊。肿瘤直径0.3~16 cm,包括非特异性类固醇细胞瘤和Leydig细胞瘤两类。前者以透明细胞为主体,类似肾上腺皮质结构;后者以嗜酸性细胞为主,细胞核密集分布区和稀疏区相间分布,可见血管壁纤维素样坏死,胞质内含有Reinke结晶或嗜酸性包涵体。免疫组化:多数病例α-inhibin、calretinin、CD99和melan-A不同程度(+),1例AE1/AE3局灶(+),另有1例Syn局灶(+)。仅有1例肿瘤出现腹腔内复发和转移。患者术后中位随访时间24个月,均呈无瘤生存状态。结论卵巢SCT诊断依赖于特征性临床表现、组织学及免疫组化染色特点。手术治疗总体预后良好,但个别病例可以发生局部复发或腹腔内转移。 Objective To explore the clinicopathological characteristics,immune profile,treatment and prognosis of ovarian steroid cell tumor（ SCT）. Methods 19 cases of ovarian SCT were studied with histological observation and immunohistochemical staining,and analyzed with review of literature. Results The patients,averaged 41 years old,were generally presented with virulism. The tumors measured 3-160 mm in size. Hitologically,they were categorized into SCT-NOS and Leydig cell tumor. Cells with abundant lipid-rich cytoplasm were more commonly seen in SCT-NOS,resembling adrenal zona glomerulosa and zona fasciculate. Leydig cell tumors were mainly composed of eosinophilic cells,with cytoplasmic Reinke crystals or eosinophilic inclusions. Clustering of nuclei alternated with nuclear-free zone,and fibrinoid necrosis of blood vessel wall could be seen. Immunohistochemically,most tumors showed positive staining of α-inhibin,calretinin,CD99 and Melan-A. Focally positive staining of AE1/AE3 and Syn was detected in one case,respectively. Only 1 case showed intraperitoneal metastases and relapsing. All patients were in the state of disease-free survival after surgery and a follow-up averaged 24 months. Conclusion The diagnosis of ovarian SCT is based on distinctive histopathology and immunohistochemical staining profile. The tumor presents good prognosis,albeit a few cases of intraperitoneal metastases and relapsing.

关 键 词： 卵巢 类固醇细胞瘤 临床病理特征 免疫组化 预后