Department of Obstetrics and Gynecology, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, 710061, Shaanxi Province, China.
Arch Gynecol Obstet. 2019 Sep;300(3):725-735. doi: 10.1007/s00404-019-05239-0. Epub 2019 Jul 16.
Epithelioid trophoblastic tumor (ETT) derived from intermediate trophoblasts is one type of gestational trophoblastic neoplasia (GTN), and it accounts for less than 2% of all gestational trophoblastic diseases (GTD). Extrauterine ETT is extremely rare, and there is currently no consistent strategy for its treatment and management. Therefore, the aim of the study is to analyze and summarize the clinicopathologic features of extrauterine ETT with or without metastasis.
The Web of Knowledge, Google Scholar, EMbase, congress of library, and PubMed were searched for extrauterine ETT without primary uterine lesions. All available data were extracted from published case reports or serial case reports, and then, the clinical and pathological characteristics were analyzed.
Twenty-two clinical studies consisting of 27 patients diagnosed with extrauterine ETT, according to the given inclusion and exclusion criteria, were included in the study. A total of 27 cases of extrauterine ETT were identified. Of these cases, four (14.81%) were located in the lungs, three (11.11%) in the ovaries, two (7.41%) in the vagina, and eight (29.63%) patients had other primary lesions. The patients originated from different continents, with 59% located in Asia and 26% in North America. Among 23 patients, the antecedent pregnancy prior to the diagnosis was full-term in 12 cases, abortion in 6 cases, hydatidiform mole in 3 cases, and invasive mole in 1 case. From the available antecedent information on pregnancy, the median interval from pregnancy to diagnosis of extrauterine ETT was 4 years. Additionally, the median gravidity and para of the patients was three times and two times, respectively. The median hCG titer was 14,374 mIU/mL in 5 patients, and the mean β-HCG titer was 3,724,805 mIU/mL in 14 patients. For all patients, the disease was confined to extrauterine ETT at diagnosis. From the available information, 20 cases were successfully treated by extraction of local lesions, and 12 cases received chemotherapy. Diagnosis was confirmed by histological tests. The Ki-67 staining ranged from 8.7 to 80%, and tumors were positive for hCG, PLAP, EMA, and p63.
In this study, we observed that abnormal levels of serum hCG titers and the local presentation of lesions with varying intervals after antecedent term pregnancy were the most common presenting features of extrauterine ETT. In addition, we found that the extraction of extrauterine lesions was needed for the treatment of extrauterine ETT. Of course, the follow-up was also important.
上皮样滋养细胞肿瘤（ETT）来源于中间滋养细胞，是一种妊娠滋养细胞肿瘤（GTN），占所有妊娠滋养细胞疾病（GTD）的不到 2%。子宫外 ETT 极为罕见，目前尚无一致的治疗和管理策略。因此，本研究旨在分析和总结有或无转移的子宫外 ETT 的临床病理特征。
在 Web of Knowledge、Google Scholar、EMbase、图书馆会议和 PubMed 上搜索无原发性子宫病变的子宫外 ETT。从已发表的病例报告或系列病例报告中提取所有可用数据，然后分析临床和病理特征。
根据纳入和排除标准，共有 22 项临床研究包括 27 例诊断为子宫外 ETT 的患者。共发现 27 例子宫外 ETT 病例。其中，4 例（14.81%）位于肺部，3 例（11.11%）位于卵巢，2 例（7.41%）位于阴道，8 例（29.63%）患者有其他原发性病变。患者来自不同的大洲，亚洲占 59%，北美占 26%。在 23 名患者中，诊断前的前次妊娠有 12 例足月产，6 例流产，3 例葡萄胎，1 例侵袭性葡萄胎。根据可获得的妊娠前期信息，从妊娠到诊断子宫外 ETT 的中位间隔时间为 4 年。此外，患者的中位孕次和产次分别为 3 次和 2 次。5 例患者的 hCG 滴度中位数为 14,374 mIU/ml，14 例患者的平均β-HCG 滴度为 3,724,805 mIU/ml。所有患者的疾病均局限于子宫外 ETT 诊断时。根据可获得的信息，20 例患者通过提取局部病变成功治疗，12 例患者接受化疗。诊断通过组织学检查确认。Ki-67 染色范围为 8.7%至 80%，肿瘤对 hCG、PLAP、EMA 和 p63 呈阳性。
在本研究中，我们观察到异常水平的血清 hCG 滴度和前次足月妊娠后不同时间间隔的局部病变表现是子宫外 ETT 最常见的表现特征。此外，我们发现需要提取子宫外病变来治疗子宫外 ETT。当然，随访也很重要。
