Background

In 1895, the association of this disease with pregnancy was developed. Healthy trophoblastic tissue invades the endometrium, resulting in a thick uterine vasculature and a close connection between the mother and the fetus, known as the placenta.

The regulating mechanisms become defective in gestational trophoblastic disorder, resulting in vascular, highly invasive, and metastatic tumors.

It comprises benign and malignant entities such as hydatidiform mole (partial and complete), invasive mole, choriocarcinoma, placental site trophoblastic tumor, and epithelioid trophoblastic tumor.

Epidemiology

Asia has a higher prevalence than European or North American populations. Similarly, there are significant regional variations in the prevalence of choriocarcinoma, which ranges from 202 per 100000 pregnancies in China to 2 per 1,00,000 pregnancies in the United States.

The probability of a further partial or complete mole increases to 2% after a molar pregnancy. After two molar pregnancies, the likelihood of developing the third molar pregnancy is 15-20% and changing partners does not reduce the risk.

Anatomy

Pathophysiology

The placenta is the primary source of gestational trophoblastic neoplasia in all forms. Complete moles in the first trimester display an abnormal sprouting villous structure with karyorrhectic stromal debris, trophoblast proliferation, and twisted villous blood vessels.

It can be challenging to morphologically distinguish a non-molar miscarriage from a partial hydatidiform mole because villous dysmorphism can appear without the partial mole’s distinctive trophoblast proliferation. Molar pregnancy may coexist with a healthy pregnancy in rare instances.

Despite the significant chance of spontaneous abortion, 40 to 60% of cases result in live births. In concurrent molar and normal pregnancy, as opposed to a single molar pregnancy, the risk of gestational trophoblastic neoplasm increases from 15 to 46%, respectively. Pregnancy can progress if there are no abnormalities and normal biological and imaging findings.

Etiology

The first cells to divide from a fertilized ovum are trophoblasts; they provide the embryo with nutrition and eventually develop into the fetal section of the placenta. Intermediate trophoblasts, syncytiotrophoblasts, and cytotrophoblasts make up the placental trophoblasts. Molar pregnancies and gestational trophoblastic neoplasia derive from placental trophoblasts.

A complete hydatidiform mole typically develops after one sperm fertilizes an ovum devoid of maternal chromosomes and then repeats its DNA, resulting in a 46XX androgenetic karyotype in which all chromosomes are paternally generated. 10% of fully developed moles are 46XY, the consequence of fertilization by two sperm.

Although mitochondrial DNA is still derived from the mother, nuclear DNA is exclusively paternal. The risk of gestational trophoblastic neoplasia may also be related to hormonal variables. Women who have menarche beyond 12, have a mild menstrual flow, and previously consumed oral contraceptives are more likely to develop the condition.

Genetics

Prognostic Factors

Many metastases are present in most high-risk patients with gestational trophoblastic neoplasia months or years after the original causative pregnancy. The location of the disease affects the signs and symptoms.

Patients with brain metastasis may exhibit convulsions, hemiparesis, or headaches. Unfortunately, the diagnosis can be overlooked because menstrual irregularities are not always present.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Future Trends

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References

https://www.ncbi.nlm.nih.gov/books/NBK562225/