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摘要: 上皮样滋养细胞肿瘤(epithelioid trophoblastic tumor, ETT)是一类少见的恶性滋养细胞肿瘤, 来源于绒毛膜型中间型滋养细胞。ETT的临床表现缺乏特异性, 给诊断带来很大难度, 确诊主要依靠手术病理诊断。虽然ETT生长缓慢, 但相比胎盘部位滋养细胞肿瘤, 其恶性程度明显升高, 某些ETT病例表现出侵袭性及致命的临床结局, 一旦出现转移或复发, 治疗效果通常较差。手术对于疾病的治疗有重要作用, 术后辅助化疗仍无规范可寻。不良预后因素包括:子宫多发病灶、出现子宫外病灶、距离前次妊娠间隔时间>4年以及高Ki-67指数。目前对于ETT的认识非常有限, 需要收集更多病例或进行前瞻性临床试验以指导对该疾病的诊断和治疗。Abstract: Epithelioid trophoblastic tumor(ETT) is a rare type of gestational trophoblastic neoplasm(GTN), which originates from intermediate trophoblastic cells of chorionic-type. Its presentation is complex and diverse, making the diagnosis challenging. The diagnosis depends mainly on pathological diagnosis. Generally, ETT is a slow-growing malignant tumor, though some cases may have aggressive and fatal clinical courses involving multiple metastases, and demonstrate a greater malignant behavior compared to the placental site trophoblastic tumor(PSTT). Surgical resection remains the cornerstone of treatment. There is no standardized postoperative adjuvant chemotherapy regimen. Multifocal disease within the uterus, the presence of extra-uterine disease, an interval greater than 4 years from antecedent pregnancy, and high Ki-67 index may be markers of poor prognosis. At present, the behavior and clinical course of ETT are yet not fully understood. More cases and data of ETT should be collected to help guide diagnosis and management.
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Key words:
- epithelioid trophoblastic tumor /
- diagnosis /
- treatment /
- prognosis
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