Gestational trophoblastic neoplasia overview

Template:Choriocarcinoma Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Monalisa Dmello, M.B,B.S., M.D. [2]

Overview

In the United States, endometrial cancer is the fourth most common type of cancer among women. Development of endometrial cancer is the result of multiple genetic mutations. Genes involved in the pathogenesis of endometrial cancer include TP53, KRAS, and PTEN. Approximately 8–30% of patients with atypical endometrial hyperplasia may progress to develop endometrial cancer. The pathophysiology of endometrial cancer depends on the 7 histological subtype: endometrioid, uterine papillary serous, mucinous, clear cell, squamous cell, mixed and undifferentiated. Common risk factors in the development of endometrial cancer are estrogen exposure, tamoxifen, obesity, diabetes, high blood pressure and genetic disorders. The hallmark of endometrial cancer is abnormal vaginal bleeding. A positive history of bleeding between normal periods in premenopausal women and vaginal bleeding and/or spotting in postmenopausal women is suggestive of endometrial cancer. Pelvic MRI and endometrial biopsy may be diagnostic of endometrial cancer. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. The optimal therapy for endometrial cancer depends on the stage at diagnosis.

Classification

Gestational trophoblastic neoplasia may be classified according to histology into four subtypes: invasive mole, choriocarcinoma, placental-site trophoblastictumor, and epithelioid trophoblastic tumor.

Pathophysiology

Gestational trophoblastic neoplasia arises from the trophoblastic tissue, which provide nutrients to the embryo and develop into a large part of the placenta. On gross pathology, dark, shaggy, focally hemorrhagic & friable/necrotic-appearing, and invasive border are characteristic findings of gestational trophoblastic neoplasia. The pathophysiology of gestational trophoblastic neoplasia depends on the histological subtype.

Differential Diagnosis

Choriocarcinoma must be differentiated from non neoplastic diseases, neoplastic diseases, and other causes of bleeding during pregnancy.

Epidemiology and Demographics

The incidence of choriocarcinoma is approximately 110-120 per 100,000 pregnancies.

Risk Factors

Common risk factors in the development of choriocarcinoma are child-bearing age, previous hydatidiform mole, and family history of gestational trophoblastic disease.

Natural History, Complications and Prognosis

Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.

Diagnosis

Staging

According to the Féderation Internationale de Gynécologie et d’Obstétrique (FIGO) cancer staging system, there are 4 stages of choriocarcinoma.

History and Symptoms

Symptoms of choriocarcinoma include vaginal bleeding, passing of tissue resembling a “bunch of grapes” from the vagina, and the abdomen may grow at a much faster rate than with a normal pregnancy.

Physical Examination

Common physical examination findings of choriocarcinoma include abdominal distention, pelvic/adnexal mass, and blood in vaginal discharge.

Laboratory Findings

Elevated serum human chorionic gonadotropin is diagnostic of choriocarcinoma.

Chest Xray

Chest radiography (CXR) may be helpful in the diagnosis of pulmonary metastasis of endometrial cancer. The characteristic findings of pulmonary metastasis are peripheral, rounded nodules of variable size scattered throughout both lungs.

CT

CT scan may be performed to detect metastases of choriocarcinoma to lung, brain, and liver.

MRI

Pelvic MRI may be diagnostic of endometrial cancer. The MRI findings of endometrial cancer vary according to the stage of the disease and may include presence of localized tumor, invasion to surrounding structures, large pelvic nodes in nodal involvement, and tumors of distant metastasis.

Ultrasound

On transvaginal ultrasound, endometrial cancer is characterized by thickening of the endometrium and disruption of a subendometrial halo.

Other Diagnostic Studies

Endometrial biopsy may be diagnostic of endometrial cancer.

Treatment

Medical therapy

The optimal therapy for endometrial cancer depends on the stage at diagnosis. A combination of chemotherapy and radiation therapy is indicated in stages IIIB- IV.

Surgery

The feasibility of surgery depends on the stage of endometrial cancer at diagnosis. Surgery is the mainstay of treatment for endometrial cancer stages I-III.

Primary Prevention

Effective measures for the primary prevention of endometrial cancer include administration of combination oral contraceptives.

Secondary Prevention

The risk of recurrence of endometrial cancer is highest within 2 years. Life-time follow-up is needed, especially within the first 2 years following diagnosis and successful treatment. Routine follow-up visists occur at 3-4 months interval during the first 2 years, at 6 months interval during the next 3 years, and yearly thereafter.

References

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