Ovarian clear cell adenocarcinoma (OCCA) is uncommon histotype in epithelial ovarian cancer,which is characterised by its association with endometriosis,and frequent mutations of ARID 1A and PIK3CA. Patients in early stage amount for high proportion,and clinical manifestation of OCCA has no specifity. Morphology of cells in ascites,tumor marker and iconography are helpful to diagnosis before surgery. The treatment of OCCA mainly includes comprehensive staging surgery and combined chemotherapy such as paclitaxel,platinum,irinotecan.mitomycinand etoposide etc. Clinical stage,size of residual tumor,state of cytology of ascites could affect the prognosis of the patients.%卵巢透明细胞癌是一种较少见的特殊类型的上皮性卵巢癌,与子宫内膜异位症和AT丰富结合域1A 基因(ARID1A)、磷酸肌醇3激酶催化亚基α异构体基因(PI3KCA)的突变有关.临床早期所占比例较高,临床表现无特异性.腹水细胞学形态、肿瘤标记物及影像学有助于术前诊断.治疗主要采用全面的分期手术和术后辅以紫杉醇、铂类、依立替康、丝裂霉素、足叶乙甙等联合化疗.临床分期、残余瘤大小、腹腔细胞学状态等因素影响患者的预后.
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