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Questions and Answers
What is the most common type of gestational trophoblastic disease?
What is the most common type of gestational trophoblastic disease?
Which risk factor significantly increases the likelihood of developing gestational trophoblastic neoplasia?
Which risk factor significantly increases the likelihood of developing gestational trophoblastic neoplasia?
Which type of hydatidiform mole is usually euploid and paternal in origin?
Which type of hydatidiform mole is usually euploid and paternal in origin?
What is a common clinical sign of hydatidiform mole?
What is a common clinical sign of hydatidiform mole?
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What is the chromosomal composition typically associated with a complete hydatidiform mole?
What is the chromosomal composition typically associated with a complete hydatidiform mole?
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Which dietary factors are associated with an increased risk of hydatidiform moles?
Which dietary factors are associated with an increased risk of hydatidiform moles?
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What condition is characterized by the rapid enlargement of the uterus with an absence of an intact fetus?
What condition is characterized by the rapid enlargement of the uterus with an absence of an intact fetus?
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What is a potential indication of 'phantom' hCG levels in a patient?
What is a potential indication of 'phantom' hCG levels in a patient?
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What best describes the 'hook effect' in hCG testing?
What best describes the 'hook effect' in hCG testing?
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Which of the following is NOT a criterion for diagnosing malignant gestational trophoblastic neoplasia?
Which of the following is NOT a criterion for diagnosing malignant gestational trophoblastic neoplasia?
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In the context of malignant gestational trophoblastic neoplasia, what is the recommended action if imaging shows no evidence of metastatic disease but hCG remains elevated?
In the context of malignant gestational trophoblastic neoplasia, what is the recommended action if imaging shows no evidence of metastatic disease but hCG remains elevated?
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Which classic microscopic finding is NOT associated with moles?
Which classic microscopic finding is NOT associated with moles?
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What constitutes the most common malignant lesion in gestational trophoblastic neoplasia?
What constitutes the most common malignant lesion in gestational trophoblastic neoplasia?
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What percentage of patients with hydatidiform moles develop invasive moles?
What percentage of patients with hydatidiform moles develop invasive moles?
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Which finding is characteristic of choriocarcinoma?
Which finding is characteristic of choriocarcinoma?
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What is the primary source of placental-site trophoblastic tumors (PSTT)?
What is the primary source of placental-site trophoblastic tumors (PSTT)?
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What complication is associated with invasive moles?
What complication is associated with invasive moles?
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Which gestational event is most commonly associated with choriocarcinoma?
Which gestational event is most commonly associated with choriocarcinoma?
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Which statement about PSTT is false?
Which statement about PSTT is false?
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What is the incidence rate of choriocarcinoma in the United States?
What is the incidence rate of choriocarcinoma in the United States?
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Which of the following is NOT a clinical presentation of choriocarcinoma?
Which of the following is NOT a clinical presentation of choriocarcinoma?
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What symptom is observed in over 90% of patients with molar pregnancies?
What symptom is observed in over 90% of patients with molar pregnancies?
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Which ultrasound finding is characteristic of a molar pregnancy?
Which ultrasound finding is characteristic of a molar pregnancy?
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What is a common laboratory finding in gestational trophoblastic neoplasms?
What is a common laboratory finding in gestational trophoblastic neoplasms?
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What indicates a potential ongoing disease process after a molar pregnancy evacuation?
What indicates a potential ongoing disease process after a molar pregnancy evacuation?
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Which complication can arise from the deportation of trophoblastic tissue to the lung?
Which complication can arise from the deportation of trophoblastic tissue to the lung?
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Which of the following is a pathognomonic sign for a molar pregnancy?
Which of the following is a pathognomonic sign for a molar pregnancy?
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What is a possible finding in the diagnosis of a partial mole during an ultrasonogram?
What is a possible finding in the diagnosis of a partial mole during an ultrasonogram?
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Which symptom can occur in 10% of patients with a molar pregnancy due to hCG stimulation?
Which symptom can occur in 10% of patients with a molar pregnancy due to hCG stimulation?
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What is the primary tool for diagnosing and monitoring gestational trophoblastic disease?
What is the primary tool for diagnosing and monitoring gestational trophoblastic disease?
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Which differential diagnosis may show an intrauterine mass during imaging?
Which differential diagnosis may show an intrauterine mass during imaging?
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What is the recommended method for the evacuation of a hydatidiform mole?
What is the recommended method for the evacuation of a hydatidiform mole?
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What follow-up monitoring is essential after the evacuation of a molar pregnancy?
What follow-up monitoring is essential after the evacuation of a molar pregnancy?
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Patients with which type of hydatidiform mole are at higher risk for malignant sequelae?
Patients with which type of hydatidiform mole are at higher risk for malignant sequelae?
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What percentage of patients may experience spontaneous regression of hCG values to normal after a hydatidiform mole evacuation?
What percentage of patients may experience spontaneous regression of hCG values to normal after a hydatidiform mole evacuation?
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What is the significance of administering Rh immune globulin after the evacuation of a hydatidiform mole?
What is the significance of administering Rh immune globulin after the evacuation of a hydatidiform mole?
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What should be done if hCG levels plateau or increase after 8 weeks of evacuation?
What should be done if hCG levels plateau or increase after 8 weeks of evacuation?
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In patients with high-risk features for malignant gestational trophoblastic disease, what is critical to determine?
In patients with high-risk features for malignant gestational trophoblastic disease, what is critical to determine?
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What factors increase the risk for malignant gestational trophoblastic disease?
What factors increase the risk for malignant gestational trophoblastic disease?
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What is the standard timeframe for the normalization of hCG levels after evacuation of a hydatidiform mole?
What is the standard timeframe for the normalization of hCG levels after evacuation of a hydatidiform mole?
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In what circumstance should prophylactic chemotherapy typically be considered after a hydatidiform mole?
In what circumstance should prophylactic chemotherapy typically be considered after a hydatidiform mole?
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Study Notes
Gestational Trophoblastic Diseases
- These are neoplastic conditions arising from trophoblastic cells, abnormal placental tissue
- Hydatidiform moles (complete and partial) are a common form, benign in nature
- Gestational trophoblastic neoplasia (GTN) can be invasive (moles, choriocarcinomas, PSTTs)
Hydatidiform Molar Pregnancy
- Two forms: Complete and Partial
- Complete moles: Euploid, paternal in origin, sex chromatin positive (46,XX or 46,XY). Develop when an empty ovum duplicates its chromosomes or fertilized by two haploid sperm
- Partial moles: Triploid (69,XXY, 69,XXX, or 69,XYY). The ovum with an active nucleus is fertilized by a duplicated sperm or 2 haploid ones
Diagnosis
- Uterine bleeding in first trimester
- Absence of fetal heart tones and fetal structures
- Rapid uterine enlargement, larger than dates expected
- Elevated human chorionic gonadotropin (hCG) titers
Risk Factors
- Women younger than 20 and older than 40
- Nulliparous women
- Low socioeconomic status
- Diets deficient in protein, folic acid, and beta-carotene
- Blood group A women impregnated by group O
Invasive Mole
- Reported in 10-15% of patients with hydatidiform mole
- Locally invasive, invades myometrium and adjacent structures
- Potentially ruptures uterus, causing hemoperitoneum
- Able to spontaneously regress
Choriocarcinoma
- Occurs in 2-5% of GTN cases
- Incidence in US is 1 in 40,000 pregnancies
- Can accompany or follow any pregnancy type
- In half of cases, the antecedent event is a hydatidiform mole, another 25% follow a term pregnancy, and 25% occur after abortion
- Pure epithelial tumor composed of syncytiotrophoblast and cytotrophoblast cells
- Usually presents with late postpartum vaginal bleeding
- Enlarged uterus, ovaries, vaginal lesions seen in physical exam
- Histologic evaluation shows sheets/foci of trophoblasts on a background of hemorrhage and necrosis, no villi
Placental-site Trophoblastic Tumors (PSTTs)
- Derived from intermediate trophoblastic cells
- Minimal hCG amounts produced compared to other diseases
- Secretes placental lactogen whose levels can be used to monitor treatment response
- Generally confined to uterus, but local invasion into myometrium, lymphatics, and vasculature possible
- Minimal or absent syncytiotrophoblastic tissue
Symptoms
- Abnormal uterine bleeding (90%)
- Nausea and vomiting (10%)
- Uterine size greater than gestational age
- Multiple theca lutein cysts in 15-30% of molar pregnancies
- Preeclampsia in the first or early second trimester
Laboratory Findings
- Hyperthyroidism (10%) from hCG stimulation of thyrotropin receptors
- hCG monitoring critical for diagnosis, treatment, and follow-up
- hCG values typically decline to nondetectable levels within 14 weeks post-evacuation of molar pregnancy
Ultrasonographic Findings
- Multiple hypoechoic areas (snowstorm pattern) corresponding to hydropic villi
- Absence of fetal sac or fetus
- Theca lutein cysts
- Focal areas of trophoblastic changes in partial moles
Differential Diagnosis
- Normal pregnancy
- Abortion
- Ectopic pregnancy
- Ultrasonography and hCG levels help differentiate
Complications
- Pulmonary insufficiency from trophoblastic tissue deportation to lungs
- Pulmonary emboli within 4-6 hours post molar evacuation
- Spontaneous regression of ectopic trophoblastic tissues
Treatment
- Evacuation, method varies but most common is suction curettage under general anesthesia – Rh-negative patients should receive Rh immune globulin post-evacuation
- Monitoring of serial hCG levels after treatment
- Chemotherapy (single or combination) indicated if hCG levels don't return to normal or plateau/increase post-evacuation.
Prophylactic Chemotherapy
- Controversy, and methotrexate or dactinomycin are used after confirming complete molar pregnancy
- Surveillance after the expulsion of the mole is critical, particularly for malignant disease
- Three-quarters of patients with non-metastatic trophoblastic disease and half of patients with metastatic trophoblastic disease will develop tumors after mole, other half a few weeks after term pregnancy, abortion or ectopic pregnancy
Low-Risk Patients
- Metastases confined to lungs or pelvis
- hCG levels below 40,000 mIU/mL at treatment onset
- Treatment is started within 4 months of disease onset
High-Risk Patients
- hCG levels >40,000 mIU/mL
- Disease diagnosis > 4 months after molar pregnancy
- Brain or liver metastases
- Prior failed chemotherapy
- Onset after term gestation
- Poor response (<40%) to single-agent therapy
Central Nervous System Involvement
- Brain metastases frequently occur with choriocarcinoma
- Whole-brain or whole-liver irradiation concomitantly with combination chemotherapy is important if CNS is involved due to risk of hemorrhagic lesions
Placental-Site Trophoblastic Tumor (PSTT)
- Resistant to chemotherapy, hysterectomy is often preferred
- Partial uterine resection possible for those wishing to retain fertility
- EMA-EP is preferred over EMACO in cases of metastatic disease
Non-metastatic Malignant GTN
- Trophoblastic disease confined primarily to the uterus.
- Diagnosis during post molar surveillance period.
- Single agent chemotherapy or combination chemotherapy plus hysterectomy are treatment options, depending on the preservation of fertility
Metastatic Gestational Trophoblastic Disease
- Treatment options include single-agent or combination chemotherapy
- WHO scoring system determines low/high risk groups for treatment
- Factors like age, antecedent pregnancy type, interval from pregnancy to chemotherapy, pretreatment hCG levels, tumor size, number/site of metastases, and prior chemotherapy are crucial for prognostication
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