Sebire N J, Lindsay Iain
Trophoblastic Disease Unit, Charing Cross Hospital, London, and Department of Paediatric Pathology, Great Ormond Street Hospital, London WC1N 3JH, United Kingdom.
Fetal Pediatr Pathol. 2010;29(1):30-44. doi: 10.3109/15513810903266120.
Gestational trophoblastic neoplasia (GTN) encompasses several entities including complete (CHM) and partial (PHM) hydatidiform mole (HM) and the malignant gestational trophoblastic tumors (GTTs), choriocarcinoma (CC), and placental-site trophoblastic tumor (PSTT), including epithelioid trophoblastic tumor (ETT). To detect pGTN, postmolar surveillance by measurement of maternal human chorionic gonoadotropin (hCG) levels should be performed. With such a protocol, many cases of pGTN are identified early at a presymptomatic stage based on plateuing or rising hCG concentrations and subsequently treated successfully with chemotherapy. In such cases, histopathological confirmation of the precise nature of the pGTN usually is not available. However, GTT also may present clinically with primary or metastatic disease, either following and unrecognized HM or developing from a nonmolar gestation. Due to their distinctive clinical and histological features, malignant GTTs are generally clearly subdivided into CC and PSTT (including ETT). CC essentially represents malignant trophoblastic tumors with differentiation toward villous trophoblast, with extensive hematogenous spread and high hCG levels, which are highly chemoresponsive. However, PSTTs, represent malignant differentiation toward implantation-site type trophoblast, with lower hCG levels and less response to chemotherapy. Current issues regarding the clinical and histological features of CC and PSTT/ETT are discussed.
妊娠滋养细胞疾病(GTN)包括多种类型,如完全性(CHM)和部分性(PHM)葡萄胎(HM)以及恶性妊娠滋养细胞肿瘤(GTTs),包括绒毛膜癌(CC)和胎盘部位滋养细胞肿瘤(PSTT),其中PSTT又包括上皮样滋养细胞肿瘤(ETT)。为了检测持续性GTN(pGTN),应通过测定母体血清人绒毛膜促性腺激素(hCG)水平进行葡萄胎后监测。按照这样的方案,许多pGTN病例在无症状阶段就基于hCG浓度的平台期或上升而被早期识别,随后通过化疗成功治疗。在这些病例中,通常无法获得pGTN确切性质的组织病理学确认。然而,GTT也可能在临床上表现为原发性或转移性疾病,既可能发生在未被识别的HM之后,也可能从非葡萄胎妊娠发展而来。由于其独特的临床和组织学特征,恶性GTT通常被明确分为CC和PSTT(包括ETT)。CC本质上代表向绒毛滋养层分化的恶性滋养细胞肿瘤,具有广泛的血行转移和高hCG水平,对化疗高度敏感。然而,PSTT代表向种植部位型滋养层的恶性分化,hCG水平较低,对化疗反应较小。本文讨论了关于CC和PSTT/ETT临床和组织学特征的当前问题。