背景:高危妊娠滋养细胞瘤(GTN)很少见,可以通过多种方法治疗。有限的公布机构数据尚未得到系统审查。
目的:编制全球高危GTN(预后评分≥7)队列,根据疾病特征和主要化疗总结治疗和结果。
方法:MEDLINE,Embase,Scopus,ClinicalTrials.gov,和Cochrane在2021年3月被搜索。
方法:报告≥10例GTN高危患者死亡率的全文。
方法:将二项式比例求和,和随机效应荟萃分析。
结果:从1,137条记录中,我们纳入了35项研究,代表20个国家。在2,276名独特的高危GTN患者中,99.7%接受化疗,35.8%手术,4.9%的辐射。死亡率为10.9%(243/2,236;荟萃分析:10%,95CI7-12%),对原发化疗完全缓解的可能性为79.7%(1,506/1,890;荟萃分析:78%,95CI74-83%)。在24项报告研究中,现代首选化疗(EMA/CO或EMA/EP)与较低的死亡率相关(总体:8.8%vs.9.5%;比较荟萃分析:8.1%与12.4%,OR=0.42,95CI0.20-0.90,14项研究)和更高的完全反应可能性(总体:76.6%vs.72.8%;比较荟萃分析:75.9%与60.7%,OR=2.98,95CI1.06-8.35,14项研究),尽管针对非首选方案的研究报告了具有可比性的结局。超高危疾病的死亡率增加(30%vs.7.5%的高风险;荟萃分析OR=7.44;95CI4.29-12.9)和足月分娩后的疾病(20.8%与磨牙妊娠后7.3%;荟萃分析OR=2.64;95CI1.10-6.31)。复发率估计范围为3-6%。
结论:高危GTN对几种化疗方案有反应,EMA/CO或EMA/EP与结局改善相关。超高风险患者死亡率增加,复发,和产后妊娠疾病。
High-risk gestational trophoblastic neoplasia (GTN) is rare and treated with diverse approaches. Limited published institutional data has yet to be systematically reviewed.
To compile global high-risk GTN (prognostic score ≥7) cohorts to summarise treatments and outcomes by disease characteristics and primary chemotherapy.
MEDLINE, Embase, Scopus, ClinicalTrials.gov and Cochrane were searched through March 2021.
Full-text manuscripts reporting mortality among ≥10 high-risk GTN patients.
Binomial proportions were summed, and random-effects meta-analyses performed.
From 1137 records, we included 35 studies, representing 20 countries. Among 2276 unique high-risk GTN patients, 99.7% received chemotherapy, 35.8% surgery and 4.9% radiation. Mortality was 10.9% (243/2236; meta-analysis: 10%, 95% confidence interval [CI] 7-12%) and likelihood of complete response to primary chemotherapy was 79.7% (1506/1890; meta-analysis: 78%, 95% CI: 74-83%). Across 24 reporting studies, modern preferred chemotherapy (EMA/CO or EMA/EP) was associated with lower mortality (overall: 8.8 versus 9.5%; comparative meta-analysis: 8.1 versus 12.4%, OR 0.42, 95% CI: 0.20-0.90%, 14 studies) and higher likelihood of complete response (overall: 76.6 versus 72.8%; comparative meta-analysis: 75.9 versus 60.7%, OR 2.98, 95% CI: 1.06-8.35%, 14 studies), though studies focused on non-preferred regimens reported comparable outcomes. Mortality was increased for ultra-high-risk disease (30 versus 7.5% high-risk; meta-analysis OR 7.44, 95% CI: 4.29-12.9%) and disease following term delivery (20.8 versus 7.3% following molar pregnancy; meta-analysis OR 2.64, 95% CI: 1.10-6.31%). Relapse rate estimates ranged from 3 to 6%.
High-risk GTN is responsive to several chemotherapy regimens, with EMA/CO or EMA/EP associated with improved outcomes. Mortality is increased in patients with ultra-high-risk, relapsed and post-term pregnancy disease.