Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy.
Division of Pathology and Laboratory Medicine, European Institute of Oncology, Milan, Italy.
Gynecol Oncol. 2019 Jun;153(3):684-693. doi: 10.1016/j.ygyno.2019.03.011. Epub 2019 Apr 30.
Placental site trophoblastic tumor [PSTT] and epithelioid trophoblastic tumor [ETT] are the rarest gestational trophoblastic neoplasias, developing from intermediate trophoblast of the implantation site and chorion leave, respectively. PSTT and ETT share some clinical-pathological features, such as slow growth rates, early stage at presentation, relatively low βhCG levels and poor response to chemotherapy. The mortality rate ranges from 6.5% to 27% for PSTT and from 10% to 24.2% for ETT. Advanced stage, long interval between antecedent pregnancy and diagnosis, and presence of clear cells are the independent prognostic variables for PSTT, and they may be similar for ETT. Hysterectomy can represent the only therapy for early disease, whereas adjuvant chemotherapy should be reserved to patients with poor risk factors, such as an interval from the antecedent pregnancy >4 years, deep myometrial invasion or serosal involvement. Few cases of fertility-sparing treatment in young women have been reported. An individualized multidisciplinary approach, including chemotherapy and debulking surgery with abdominal and/or extra-abdominal procedures, is warranted for advanced disease. EP/EMA and TP/TE are the preferred regimens in this setting. Immunohistochemistry has sometimes shown expression of EGFR, VEGF, MAPK, PDGF-R and PD-L1, and therefore investigational studies on biological agents targeting these molecules are strongly warranted for chemotherapy resistant-disease.
胎盘部位滋养细胞肿瘤[PSTT]和上皮样滋养细胞肿瘤[ETT]是最罕见的妊娠滋养细胞肿瘤，分别由着床部位的中间滋养细胞和绒毛膜蜕膜发展而来。PSTT 和 ETT 具有一些临床病理特征，如生长缓慢、发病时处于早期、βhCG 水平相对较低以及对化疗反应不佳。PSTT 的死亡率范围为 6.5%至 27%，ETT 的死亡率范围为 10%至 24.2%。晚期、前次妊娠与诊断之间的间隔时间长以及存在透明细胞是 PSTT 的独立预后变量，这些变量可能与 ETT 相似。子宫切除术可作为早期疾病的唯一治疗方法，而对于具有不良预后因素的患者（如前次妊娠与诊断之间的间隔时间>4 年、深肌层浸润或浆膜受累），应保留辅助化疗。有报道称，少数年轻女性采用了保留生育能力的治疗方法。对于晚期疾病，需要采用个体化的多学科方法，包括化疗和减瘤手术，联合腹部和/或腹外手术。在这种情况下，EP/EMA 和 TP/TE 是首选方案。免疫组织化学有时显示 EGFR、VEGF、MAPK、PDGF-R 和 PD-L1 的表达，因此强烈需要针对这些分子的生物制剂进行化疗耐药疾病的研究。
