Objective To clarify the clinicopathological features of ovarian clear cell carcinoma derived from endometriotic cyst (EC-OCCC).Methods Totally 54 cases of EC-OCCC were recruited in the current retrospective study.The relation between ages, clinical symptoms and signs, surgical and pathological stages, serum CA125, findings of ultrasound, treatments and the sites of tumors, macro-and micro-features and expression of immunostainings were analyzed.Results (1) Clinical features: the ages of patients were (50±6) years old (range 31-62 years old).Pelvic mass was the major complaint of 50 patients (93％, 50/54).Forty-five cases belonged to International federation of Gynecology and Obstetrics (FIGO) stage Ⅰ, 4 cases were stage Ⅱ and another 5 cases were stage Ⅲ.Serum CA125 was elevated in 21 cases (54％, 21/39) before therapy.Doppler ultrasound showed 46 cases (85％, 46/54) had solid masses in pelvis.(2) Pathological findings: 52 cases (96％, 52/54) had their tumor unilaterally, and 2 cases (4％, 2/54) occurred bilaterally.The maximal diameters of endometriotic cyst (EC) ranged from 1.5 to 23.0 cm and maximal diameters of ovarian clear cell carcinoma (OCCC) components were from 0.5 to 12.0 cm.Fifty-one cases (94％, 51/54) had their tumor within EC, which showed focally irregular protrudings, grey-white papillae or solid nodules attached to the cyst wall.Three cases (6％, 3/54) appeared as irregular thickened wall of the cysts, ranged from 1.5 to 6.0 cm in the maximal length, with the microscopic features of EC and OCCC and the transitional areas between the 2 morphologies.All cases expressed cytokeratin (CK) 7 and pan-CK AE1/AE3, 17 cases (33％, 17/51) expressed ER and 5 cases (10％, 5/51) expressed PR.TP53 showed mutational phenotype in 19 cases (36％, 19/53).Sixteen cases (30％, 16/54) combined with uterine adenomyosis and 25 cases (46％, 25/54) with endometriosis at other sites.(3) Survival survey: during the period of 39.1 months follow-up, 3 cases relapsed and 2 cases died.(4) There was a significant difference of serum CA125 between patients of early-and advanced-stages (P=0.049).There were no differences identified in ages, diameters of EC and OCCC, the expression level of ER, PR and TP53, the co-existence of adenomyosis and endometrosis, as well as ultrasonic findings (P＞0.05).Conclusion EC-OCCC could be recognized in early stage by symptoms and ultrasound due to accompanied endometriotic cysts, resulting in relatively good prognosis.%目的 探讨起源于卵巢子宫内膜样囊肿的卵巢透明细胞癌(EC-OCCC)的临床病理特征.方法 回顾性分析2007年7月至2014年7月在复旦大学附属妇产科医院就诊的54例EC-OCCC患者的临床病理资料,包括患者年龄、临床症状和体征、手术病理分期、血清CA125水平、彩超检查结果、治疗方法,以及肿瘤的发生部位、大体表现、镜下特点、免疫组化法检测结果.随访患者的生存情况,并对早期(Ⅰ期)、晚期(Ⅱ～Ⅲ期)EC-OCCC患者的临床病理指标进行比较.结果 (1)临床特点:54例EC-OCCC患者的年龄为(50±6)岁(31 ～ 62岁),其中50例(93％,50/54)因体检或其他妇科疾病检查时发现盆腔肿物.手术病理分期:Ⅰ期45例,Ⅱ期4例,Ⅲ期5例;39例术前行血清CA125水平检测的患者中,21例(54％,21/39)血清CA125水平有不同程度升高;46例(85％,46/54)术前彩超检查提示盆腔肿块中含实质性结构.(2)病理特点:54例EC-OCCC患者中,52例(96％,52/54)肿瘤发生于单侧卵巢,2例(4％,2/54)发生于双侧卵巢;卵巢子宫内膜样囊肿(EC)最大径为1.5 ～ 23.0 cm,卵巢透明细胞癌(OCCC)直径为0.5～ 12.0 cm.OCCC在EC的囊腔内者51例(94％,51/54),大体表现为EC的囊壁表面有不规则隆起、实性灰白色结节及不规则状团块;OCCC在EC的囊壁内者3例(6％,3/54),大体表现为EC的囊壁异常增厚,囊壁厚度为1.5 ～ 6.0 cm.显微镜下可见典型的卵巢EC及OCCC成分,EC被覆的子宫内膜样腺上皮与OCCC相移行.54例EC-OCCC患者中,53例行免疫组化法检测,53例(100％,53/53)细胞角蛋白(CK)7及广谱CK AE1/AE3均阳性,19例(36％,19/53)TP53基因为突变表型;51例行ER、PR检测,其中17例(33％,17/51)ER阳性,5例(10％,5/51)PR阳性;合并子宫腺肌病者16例(30％,16/54),合并子宫内膜异位症者25例(46％,25/54).(3)生存情况:术后平均随访39.1个月,3例复发,2例死亡.(4)早期、晚期EC-OCCC患者间,除术前血清CA125水平比较,差异有统计学意义(P=0.049)外,两者间年龄、EC直径、OCCC直径以及ER、PR和TP53表达、合并子宫腺肌病和子宫内膜异位症、彩超检查结果比较,差异均无统计学意义(P＞0.05).结论 EC-OCCC因有子宫内膜异位症相关症状及存在彩超检查容易发现的盆腔肿块,易被早期诊断,故而患者预后较好.
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