AUTHORS: Courtney Pfeuti, MD and Anthony Sciscione, DO
Molar pregnancy (hydatidiform mole) is a premalignant gestational disorder and is included in the spectrum of disorders characterized as gestational trophoblastic disease. Molar pregnancies are classified as complete or partial based on morphologic and pathologic examination. Both complete and partial molar pregnancies have an abnormal placenta with enlargement and swelling of the chorionic villi and hyperplasia of the villous trophoblastic cells. Most molar pregnancies are complete and are characterized by generalized hydropic villous changes with no fetal tissue. Partial moles are characterized by a mixture of large hydropic villi and normal placental tissue and often have fetal tissue present. Gestational trophoblastic disease (GTD) is composed of a spectrum of neoplastic conditions derived from the placenta. Whereas hydatidiform moles, gestational choriocarcinoma, and placental site trophoblastic tumor (PSTT) are histologic diagnoses, postmolar gestational trophoblastic neoplasia (GTN) is defined by clinical and laboratory criteria. The disease entities included in GTD have a wide variation in behavior, but GTN specifically refers to those with the potential for tissue invasion and metastases.
The risk of malignant sequelae (GTN) for a complete mole is 7% to 30% and for a partial mole is less than 2.5% to 7.5%.
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Table E1 Classic Presenting Signs and Symptoms of Complete and Partial Hydatidiform Moles
Sign or Symptom | Complete Mole (%) (n = 306) | Partial Mole (%) (n = 81) |
---|---|---|
Vaginal bleeding | 97 | 73 |
Excessive uterine size | 51 | 4 |
Theca lutein cysts >6 cm | 50 | 0 |
Preeclampsia | 27 | 3 |
Hyperemesis | 26 | 0 |
Hyperthyroidism | 7 | 0 |
Trophoblastic emboli | 2 | 0 |
From Niederhuber JE: Abeloffs clinical oncology, ed 6, Philadelphia, 2020, Elsevier.
Figure E1 Genetic makeup of normal pregnancy and partial and complete molar pregnancies.
From Magowan BA: Clinical obstetrics & gynecology, ed 4, New York, 2019, Elsevier.
Complete mole, partial mole (Table E2), ectopic pregnancy, abortion (incomplete or spontaneous), normal intrauterine pregnancy
TABLE E2 Features of Partial and Complete Hydatidiform Moles
Feature | Partial Mole | Complete Mole |
---|---|---|
Karyotype | 69,XXX or , XXY | 46,XX or , XY |
Pathology | ||
Fetus | Often present | Absent |
Amnion, fetal RBC | Usually present | Absent |
Villous edema | Variable, focal | Diffuse |
Clinical Presentation | ||
Diagnosis | Missed abortion | Molar gestation |
Uterine size | Small for dates | 50% large for dates |
Theca-lutein cysts | Rare | 25%-30% |
Postmolar GTN | 2.5%-7.5% | 6.8%-20% |
GTN, Gestational trophoblastic neoplasia; RBC, red blood cell.
From Disaia PJ et al: Clinical gynecologic oncology, ed 9, Philadelphia, 2017, Elsevier.
Figure E4 Endovaginal ultrasound of the theca lutein cyst with early molar pregnancy.
Note enlarged anechoic cystic spaces within the ovary (arrows).
From Fielding JR et al: Gynecology imaging, Philadelphia, 2011, Saunders.
These usually contain multiple thin septations and have an appearance similar to iatrogenic ovarian hyperstimulation during ovulation induction. Theca-lutein cysts regress spontaneously after evacuation of the molar pregnancy, but regression often lags behind human chorionic gonadotropin level decline.
Courtesy John Soper, MD. From Disaia PJ et al: Clinical gynecologic oncology, ed 9, Philadelphia, 2017, Elsevier.
Figure E6 Complete molar pregnancy: Classic appearance.
Transabdominal scan shows a vesicular echogenic mass distending the endometrium. The mass is filled with innumerable uniformly distributed cystic spaces that corresponded to hydropic chorionic villi at pathology.
From Rumack CM et al: Diagnostic ultrasound, ed 4, Philadelphia, 2011, Mosby.
Surgical uterine evacuation with dilation and curettage (D&E) is the mainstay of management of a molar pregnancy, either partial or complete. Suction D&C is the preferred method in patients who desire future pregnancies. Ultrasound guidance and avoidance of sharp curettage may decrease the risk of uterine perforation.Pitocin infusion at a rate of 20 units/L can be considered after cervical dilation and can be continued for several hours postprocedure if bleeding persists. Hysterectomy should be considered in women older than 40 yr. Tables E3 and E4 summarize the management of hydatidiform moles.
TABLE E3 Management of Hydatidiform Mole
Evacuation: Suction D&E (or hysterectomy in selected patients) | |||
Postevacuation quantitative hCG level and chest radiography | |||
Monitor quantitative hCG levels every 1-2 wk until three normal values or criteria for GTN | |||
After hCG level is normal for three values, then monitor hCG levels every 1-3 mo for 6 mo | |||
Initiate chemotherapy for GTN using indications listed in: |
D&E, Suction dilation and evacuation; GTN, gestational trophoblastic neoplasia; hCG, human chorionic gonadotropin.
From Disaia PJ et al: Clinical gynecologic oncology, ed 9, Philadelphia, 2017, Elsevier.
TABLE E4 Diagnosis and Evaluation of Gestational Trophoblastic Neoplasia
Diagnosis of GTN | |||
After molar evacuation: Four values or more of plateaued hCG (±10%) over at least 3 wk: Days 1, 7, 14, and 21 | |||
After molar evacuation: A rise of hCG of 10% or greater for three values or more over at least 2 wk: Days 1, 7, and 14 | |||
After molar evacuation: Persistence of hCG beyond 6 mo | |||
The histologic diagnosis of choriocarcinoma, invasive mole, PSTT, or epithelioid trophoblastic disease | |||
Metastatic disease without established primary site with elevated hCG (pregnancy has been excluded) | |||
Evaluation of GTN | |||
Complete physical and pelvic examination; baseline hematologic, renal, and hepatic functions | |||
Baseline quantitative hCG level | |||
Chest radiograph or CT scan of chest | |||
Brain MRI | |||
CT or MRI scan of abdomen and pelvis |
CT, Computed tomography; GTN, gestational trophoblastic neoplasia; hCG, human chorionic gonadotropin; MRI, magnetic resonance imaging; PSTT, placental site trophoblastic tumor.
From Disaia PJ et al: Clinical gynecologic oncology, ed 9, Philadelphia, 2017, Elsevier.
If pathology results are consistent with complete or partial mole, patients must be followed to evaluate for trophoblastic neoplasia. 7% to 30% of complete moles and 2.5% to 7.55% of partial moles can develop into trophoblastic neoplasia. Quantitative beta hCG should be followed weekly until three consecutive results show normal levels. After that, check quantitative beta hCG every month for a total of 6 mo. Patients should remain on reliable contraception during this time to prevent confusion from a rising beta hCG in the case of a new pregnancy.
Specific criteria by beta hCG have been established by International Federation of Gynecology and Obstetrics (FIGO) for diagnosis of postmolar gestational trophoblastic disease (see Tables E5, E6, and E7). Patients with pre-evacuation hCG greater than 100,000 mIU/ml, excessive uterine enlargement, theca lutein cysts greater than 6 cm, or older than 40 yr are at increased risk of postmolar gestational trophoblastic neoplasia. Patients with a complete or partial mole have a 1% to 2% incidence of second mole in subsequent pregnancies.
TABLE E5 The 2002 Criteria for the Diagnosis of Posthydatidiform Mole Trophoblastic Neoplasia
hCG-level plateau of four values ±10% recorded over a 3-wk duration (days 1, 7, 14, and 21) | |||
An hCG-level increase of more than 10% of three values recorded over a 2-wk duration (days 1, 7, and 14) | |||
Persistence of detectable hCG for more than 6 mo after molar evacuation |
hCG, Human chorionic gonadotropin.
TABLE E6 International Federation of Gynecology and Obstetrics Staging of Gestational Trophoblastic Neoplasia
Stage I | Disease confined to the uterus | ||
Stage II | GTN extends outside the uterus, but is limited to the genital structures (adnexa, vagina, broad ligament) | ||
Stage III | GTN extends to the lungs, with or without genital tract involvement | ||
Stage IV | All other metastatic sites |
GTN, Gestational trophoblastic neoplasia.
From Kohorn EI: The new FIGO 2000 staging and risk factor scoring system for gestational trophoblastic disease: description and critical assessment, Int J Gynecol Cancer 11:73-77, 2001.
TABLE E7 The World Health Organization (WHO) Prognostic Scoring System is Used for the Medical Management of Patients With Partial, Complete Moles, and Choriocarcinomas
FIGO Scoring | 0 | 1 | 2 | 4 |
---|---|---|---|---|
Age | <40 | ≥40 | - | - |
Antecedent pregnancy | Mole | Abortion | Term | - |
Interval months from index pregnancy | <4 | 4-<7 | 7-<13 | ≥13 |
Pretreatment serum hCG (IU/L) | <103 | 103-<104 | 104-<105 | ≥105 |
Large tumor size (including uterus) cm | <3 | 3-<5 | ≥5 | - |
Site of metastases | Lung | Spleen, kidney | Gastrointestinal | Liver, brain |
Previous failed chemotherapy | - | - | Single drug | Two or more drugs |
FIGO, International Federation of Gynecology and Obstetrics; hCG, human chorionic gonadotropin.
Spontaneous Abortion (Related Key Topic)
Vaginal Bleeding During Pregnancy (Related Key Topic)