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妊娠滋养细胞疾病的当前管理

Current management of gestational trophoblastic diseases.

作者信息

Berkowitz Ross S, Goldstein Donald P

机构信息

New England Trophoblastic Disease Center, Division of Gynecologic Oncology, Brigham and Women's Hospital and Dana Farber Cancer Institute, Harvard Medical School, Boston, MA 02115, USA.

出版信息

Gynecol Oncol. 2009 Mar;112(3):654-62. doi: 10.1016/j.ygyno.2008.09.005. Epub 2008 Oct 12.

Abstract

OBJECTIVES

This review was undertaken to describe current understanding of the natural history of molar pregnancy and persistent gestational trophoblastic neoplasia (GTN) as well as recent advances in their management.

MATERIALS AND METHODS

Recent literature related to molar pregnancy and GTN was thoroughly analyzed to provide a comprehensive review of the current knowledge of their pathogenesis and treatment.

RESULTS

Studies in patients with familial recurrent molar pregnancy indicate that dysregulation of parentally imprinted genes is important in the pathogenesis of complete hydatidiform mole (CHM). CHM is now being diagnosed earlier in pregnancy in the first trimester changing the clinical presentation and making the histologic appearance more similar to partial hydatidiform mole (PHM) and hydropic abortion. While the classic presenting symptoms of CHM are less frequent, the risk of developing GTN remains unchanged. Flow cytometry and immunostaining for maternally-expressed genes are helpful in distinguishing early CHM from PHM or hydropic abortion. Patients with molar pregnancy have a low risk of developing persistent GTN after achieving even one non-detectable hCG level (hCG <5 mIU/ml). Patients with persistent low levels of hCG should undergo tests to determine if the hCG is real or phantom. If the hCG is real, then further tests should determine what percentage of the total hCG is hyperglycosylated hCG and free beta subunit to establish a proper diagnosis and institute appropriate management. Patients with non-metastatic GTN have a high remission rate with many different single-agent regimens including methotrexate and actinomycin D. Patients with high-risk metastatic GTN require aggressive combination chemotherapy in conjunction with surgery and radiation therapy to attain remission. After achieving remission, patients can generally expect normal reproduction in the future.

CONCLUSION

Our understanding of the natural history and management of molar pregnancy and GTN has advanced considerably in recent years. While most patients can anticipate a high cure rate, efforts are still necessary to develop effective new second-line therapies for patients with drug-resistant disease.

摘要

目的

进行本综述以描述当前对葡萄胎和持续性妊娠滋养细胞肿瘤(GTN)自然史的认识以及它们在治疗方面的最新进展。

材料与方法

对与葡萄胎和GTN相关的近期文献进行全面分析,以全面综述它们发病机制和治疗的当前知识。

结果

对家族性复发性葡萄胎患者的研究表明,亲本印记基因的失调在完全性葡萄胎(CHM)的发病机制中很重要。现在CHM在妊娠早期即孕早期被更早诊断,这改变了临床表现,使其组织学表现更类似于部分性葡萄胎(PHM)和水肿性流产。虽然CHM的典型表现症状较少见,但发生GTN的风险保持不变。对母源表达基因进行流式细胞术和免疫染色有助于区分早期CHM与PHM或水肿性流产。葡萄胎患者即使达到一个不可检测的人绒毛膜促性腺激素(hCG)水平(hCG<5 mIU/ml)后发生持续性GTN的风险也较低。hCG持续低水平的患者应进行检查以确定hCG是真实的还是假阳性。如果hCG是真实的,那么进一步检查应确定总hCG中高糖基化hCG和游离β亚基的百分比,以建立正确诊断并采取适当治疗。非转移性GTN患者使用包括甲氨蝶呤和放线菌素D在内的许多不同单药方案有较高的缓解率。高危转移性GTN患者需要积极的联合化疗并结合手术和放疗以实现缓解。实现缓解后,患者未来一般可预期正常生育。

结论

近年来,我们对葡萄胎和GTN自然史及治疗的认识有了很大进展。虽然大多数患者可预期高治愈率,但仍需要努力为耐药疾病患者开发有效的新二线治疗方法。

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