Person:

Goldstein, Donald

Gestational trophoblastic neoplasia (GTN) describes a heterogeneous group of interrelated lesions that arise from abnormal proliferation of placental trophoblasts. GTN lesions are histologically distinct, malignant lesions that include invasive hydatidiform mole, choriocarcinoma, placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT). GTN tumors are generally highly responsive to chemotherapy. Early stage GTN disease is often cured with single-agent chemotherapy. In contrast, advanced stage disease requires multiagent combination chemotherapeutic regimens to achieve a cure. Various adjuvant surgical procedures can be helpful to treat women with GTN. Patients require careful followup after completing treatment and recurrent disease should be aggressively managed. Women with a history of GTN are at increased risk of subsequent GTN, hence future pregnancies require careful monitoring to ensure normal gestational development. This article will review the workup, management and followup of women with all stages of GTN as well as with recurrent disease.

Major advances have been achieved during the past 40 years in the epidemiology, etiology, pathology, endocrinology, immunology, diagnosis, and treatment of molar pregnancy (MP) and gestational trophoblastic neoplasia (GTN). MP is now recognized as composing two distinct entities--complete and partial, with distinct histopathology, genetics, and clinical presentations. Proper management is dependent on a thorough understanding of each type. Early diagnosis and effective treatment of patients with GTN has resulted in 100 percent cure rates in non-metastatic disease and in the majority of patients with metastases. In most instances, resistant disease leading to death results from delayed diagnosis and overwhelming tumor burden. Moreover, in most instances successful treatment can be accomplished with preservation of fertility and normal pregnancy outcome anticipated. A rare variant of choriocarcinoma called placental site trophoblastic tumor (PSTT) has been described, which, although curable by surgery when localized, is usually fatal when disseminated. It is anticipated that during the decade of the nineties the scientific work in progress will lead to earlier diagnosis and improved survival in resistant cases.