• Florin Gorun, Larisa Tomescu, Andrei Motoc, Cosmin Citu, Ioan Sas, Denis Mihai Serban, Marius Forga, Ioana Mihaela Citu, and Oana Maria Gorun.
• Department of Obstetrics and Gynecology, "Victor Babes" University of Medicine and Pharmacy Timisoara, Timisoara, Romania.
• Medicine (Baltimore). 2022 Jul 29; 101 (30): e29934e29934.

BackgroundThis study aimed to systematically review the existing literature on epithelioid trophoblastic tumors (ETTs), the rarest type of gestational trophoblastic neoplasia.MethodsA systematic review according to PRISMA guidelines was performed, using ScienceDirect, Web of Science, and Scopus databases. The only filter used was the English language. Eligibility/inclusion criteria: retrospective observational studies (case reports, case series) including full case description of epithelioid trophoblastic tumor lesions.ResultsSeventy studies were assessed for synthesis, including 147 cases. 66.7% of patients with ETT presented with irregular vaginal bleeding. Pretreatment β-hCG levels ranged up to 1000 mIU/mL in 58.5% patients. Of most patients, 42.2% had stage I disease, 10.9% stage II, 25.2% stage III, and 21.8% of patients had stage IV. The most common sites of metastatic disease were the lungs, followed by the liver and brain. After treatment, complete remission was achieved in 75.5% of patients, partial remission in 10.2% of patients, and 14.3% of patients died. On univariate and multivariate analyses, stage IV disease was an independent prognostic factor for overall and disease-free survival.ConclusionsHysterectomy and metastatic lesion resection are essential for controlling ETT. Investigational studies on molecules like EGFR, VEGF, PD-1, CD105, and LPCAT1 are potential therapeutic targets for metastatic ETT.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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