A hydatidiform mole is a rare mass or growth that forms inside the uterus at the beginning of a pregnancy.

It is a type of gestational trophoblastic disease (GTD). See also:

Gestational trophoblastic disease Choriocarcinoma (a cancerous form of GTD)

Causes
A hydatidiform mole, or molar pregnancy, results from over-production of the tissue that is supposed to develop into the placenta. The placenta normally feeds a fetus during pregnancy. In this condition, the tissues develop into an abnormal growth, called a mass. There are two types: Partial molar pregnancy Complete molar pregnancy A partial molar pregnancy means there is an abnormal placenta and some fetal development. In a complete molar pregnancy, there is an abnormal placenta but no fetus. Both forms are due to problems during fertilization. The exact cause of fertilization problems are unknown. However, a diet low in protein, animal fat, and vitamin A may play a role.

Symptoms
Abnormal growth of the womb (uterus) Excessive growth in about half of cases Smaller-than-expected growth in about a third of cases Nausea and vomiting that may be severe enough to require a hospital stay Vaginal bleeding in pregnancy during the first 3 months of pregnancy

Symptoms of hyperthyroidism Heat intolerance Loose stools Rapid heart rate Restlessness, nervousness

Trembling hands Unexplained weight loss Symptoms similar to preeclampsia that occur in the 1st trimester or early 2nd trimester
-- this is almost always a sign of a hydatidiform mole, because preeclampsia is extremely rare this early in a normal pregnancy High blood pressure Swelling in feet, ankles, legs

Skin warmer and more moist than usual

Exams and Tests

A pelvic examination may show signs similar to a normal pregnancy, but the size of the womb may be abnormal and the baby's heart sounds are absent. There may be some vaginal bleeding. A pregnancy ultrasound will show an abnormal placenta with or without some development of a baby.

Tests may include:

HCG blood test

Chest x-ray CT or MRI of the abdomen Complete blood count Blood clotting tests Kidney and liver function tests

Treatment
If your doctor suspects a molar pregnancy, a suction curettage (D and C) may be performed. A hysterectomy may be an option for older women who do not wish to become pregnant in the future. After treatment, serum HCG levels will be followed. It is important to avoid pregnancy and to use a reliable contraceptive for 6 - 12 months after treatment for a molar pregnancy. This allows for accurate testing to be sure that the abnormal tissue does not return. Women who get pregnant too soon after a molar pregnancy have a greater risk of having another one.

Outlook (Prognosis)
More than 80% of hydatidiform moles are benign (noncancerous). The outcome after treatment is usually excellent. Close follow-up is essential. After treatment, you should use very effective contraception for at least 6 to 12 months to avoid pregnancy. In some cases, hydatidiform moles may develop into invasive moles. These moles may grow so far into the uterine wall and cause bleeding or other complications. In a few cases, a hydatidiform mole may develop into a choriocarcinoma, a fast-growing cancerous form of gestational trophoblastic disease. See: Choriocarcinoma

Possible Complications
Lung problems may occur after a D and C if the woman's uterus is bigger than 16 weeks gestational size. Other complications related to the surgery to remove a molar pregnancy include: Preeclampsia Thyroid problems Gestational trophoblastic disease encompasses several disease processes that originate in the placenta. These include complete and partial moles, placental site trophoblastic tumors, choriocarcinomas, and invasive moles. Almost all women with malignant gestational trophoblastic disease can be cured with preservation of reproductive function. The following discussion is limited to hydatidiform moles (complete and partial). A complete mole contains no fetal tissue. Ninety percent are 46,XX, and 10% are 46,XY.[1, 2] Complete moles can be divided into 2 types: Androgenetic complete mole Homozygous These account for 80% of complete moles.

Two identical paternal chromosome complements, derived from duplication of the paternal haploid chromosomes. Always female; 46,YY has never been observed. Heterozygous These account for 20% of complete moles. May be male or female. All chromosomes are of parental origin, most likely due to dispermy. Biparental complete mole: Maternal and paternal genes are present but failure of maternal imprinting causes only the paternal genome to be expressed.[3] The biparental complete mole is rare. A recurrent form of biparental mole, which is familial and appears to be inherited as an autosomal recessive trait, has been described. Al-Hussaini describes a series of 5 women with as many as 9 consecutive molar pregnancies.[4, 5] Mutations in NLRP7 at 19q13.4 have been identified as causative in recurrent molar pregnancies.[6, 7, 8] With a partial mole, fetal tissue is often present. Fetal erythrocytes and vessels in the villi are a common finding. The chromosomal complement is 69,XXX or 69,XXY.[9] This results from fertilization of a haploid ovum and duplication of the paternal haploid chromosomes or from dispermy. Tetraploidy may also be encountered. As in a complete mole, hyperplastic trophoblastic tissue and swelling of the chorionic villi occur. United States By studying elective pregnancy terminations, hydatidiform moles were determined to occur in approximately 1 in 1200 pregnancies.[10] International The reported frequency of hydatidiform mole varies greatly. Some of this variability can be explained by differences in methodology (eg, single hospital vs population studies, identification of cases). The reported frequencies range from 1 in 100 pregnancies in Indonesia to 1 in 200 pregnancies in Mexico to 1 in 5000 pregnancies in Paraguay.[11] The study of pathologic material from first- and second-trimester abortions established a frequency of complete and partial hydatidiform moles in Ireland of 1 per 1945 pregnancies and 1 per 695 pregnancies, respectively.[12]

Mortality/Morbidity
A hydatidiform mole is considered malignant if metastases or destructive invasion of the myometrium (ie, invasive mole) occurs, or when the serum hCG levels plateau or rise during the period of follow-up and an intervening pregnancy is excluded. Malignancy (see eMedicine's article Gestational Trophoblastic Neoplasia) is diagnosed in 15-20% of patients with a complete hydatidiform mole and 2-3% of partial moles.[13, 14] Lung metastases are found in 4-5% of patients with a complete hydatidiform mole and rarely in cases of partial hydatidiform moles.[15, 16]

Race
Differences in the frequency of hydatidiform moles between ethnic groups have been reported internationally.[11, 17] In the United States, comparison of frequency of hydatidiform moles in African Americans and Caucasians have yielded conflicting results.[17] If differences exist, whether they are due to genetic differences or environmental factors is not clear.

Sex
Hydatidiform mole is a disease of pregnancy and therefore a disease of women. See Medscape's Pregnancy Resource Center.

Age
Hydatidiform mole is more common at the extremes of reproductive age. Women in their early teenage or perimenopausal years are most at risk.[18, 19, 20, 11, 17]Women older than 35 years have a 2-fold increase in

risk. Women older than 40 years experience a 5- to 10-fold increase in risk compared to younger women. Parity does not affect the risk.

Complete mole: The typical clinical presentation of complete molar pregnancies has changed with the advent of
high-resolution ultrasonography. Most moles are now diagnosed in the first trimester before the onset of the classic signs and symptoms.[21, 22] Vaginal bleeding: The most common classic symptom of a complete mole is vaginal bleeding. Molar tissue separates from the decidua, causing bleeding. The uterus may become distended by large amounts of blood, and dark fluid may leak into the vagina. This symptom occurs in 50% of cases. Hyperemesis: Patients may also report severe nausea and vomiting. This is due to extremely high levels of human chorionic gonadotropin (hCG). Hyperthyroidism: Signs and symptoms of hyperthyroidism can be present due to stimulation of the thyroid gland by the high levels of circulating hCG or by a thyroid stimulating substance (ie, thyrotropin) produced by the trophoblasts.[23] Partial mole: Patients with partial mole do not have the same clinical features as those with complete mole. These patients usually present with signs and symptoms consistent with an incomplete or missed abortion. Vaginal bleeding Absence of fetal heart tones

Complete mole (See images below.) Size inconsistent with gestational age: A uterine enlargement greater than expected for gestational age is a classic sign of a complete mole. Unexpected enlargement is caused by excessive trophoblastic growth and retained blood. However, patients present with size-appropriate enlargement or smaller-than-expected enlargement at a similar frequency. Preeclampsia: Pelvic ultrasonography has resulted in the early diagnosis of most cases of hydatidiform mole and preeclampsia is seen in less than 2% of cases.[22] Theca lutein cysts: These are ovarian cysts greater than 6 cm in diameter and accompanying ovarian enlargement. These cysts are not usually palpated on bimanual examination but are identified by ultrasonography. Patients may report pressure or pelvic pain. Because of the increased ovarian size, torsion is a risk. These cysts develop in response to high levels of beta-hCG. They spontaneously regress after the mole is evacuated, but it may take up to 12 weeks for complete regression. Theca lutein cysts. Complete mole. Complete
mole with an area of clot near cervix consistent with bleeding.

Partial mole Uterine enlargement and preeclampsia is reported in only 5% of patients.[24] Theca lutein cysts, hyperemesis, and hyperthyroidism are extremely rare. Twinning (See image below.) Twinning with a complete mole and a fetus with a normal placenta has been reported (see image below). Cases of healthy infants in these circumstances have been reported.[25, 9] Women with coexistent molar and normal gestations are at higher risk for developing persistent disease and metastasis[26] . Termination of pregnancy is a recommended option. The pregnancy may be continued as long as the maternal status is stable, without hemorrhage, thyrotoxicosis, or severe hypertension. The patient should be informed of the risk of severe maternal morbidity from these complications.[27]

Prenatal genetic diagnosis by chorionic villus sampling or amniocentesis is recommended to evaluate the
karyotype of the fetus. Twin gestation. Complete mole and normal twin.

A diet deficient in animal fat and carotene may be a risk factor.[17,

18]

Hydatidiform Mole

Definition
A hydatidiform mole is a relatively rare condition in which tissue around a fertilized egg that normally would have developed into the placenta instead develops as an abnormal cluster of cells. (This is also called a molar pregnancy.) This grapelike mass forms inside of the uterus after fertilization instead of a normal embryo. A hydatidiform mole triggers a positive pregnancy test and in some cases can become cancerous.

Description
A hydatidiform mole ("hydatid" means "drop of water" and "mole" means "spot") occurs in about 1 out of every 1,500 (1/1,500) pregnancies in the United States. In some parts of Asia, however, the incidence may be as high as 1 in 200 (1/200). Molar pregnancies are most likely to occur in younger and older women (especially over age 45) than in those between ages 20-40. About 1-2% of the time a woman who has had a molar pregnancy will have a second one. A molar pregnancy occurs when cells of the chorionic villi (tiny projections that attach the placenta to the lining of the uterus) don't develop correctly. Instead, they turn into watery clusters that can't support a growing baby. A partial molar pregnancy includes an abnormal embryo (a fertilized egg that has begun to grow) that does not survive. In a compete molar pregnancy there is a small cluster of clear blisters or pouches that don't contain an embryo. If not removed, about 15% of moles can become cancerous. They burrow into the wall of the uterus and cause serious bleeding. Another 5% will develop into fast-growing cancers called choriocarcinomas. Some of these tumors spread very quickly outside the uterus in other parts of the body. Fortunately,cancer developing from these moles is rare and highly curable.

Causes and symptoms

The cause of hydatidiform mole is unclear; some experts believe it is caused by problems with the chromosomes (the structures inside cells that contain genetic information) in either the egg or sperm, or both. It may be associated with poor nutrition, or a problem with the ovaries or the uterus. A mole sometimes can develop from placental tissue that is left behind in the uterus after a miscarriage or childbirth. Women with a hydatidiform mole will have a positive pregnancy test and often believe they have a normal pregnancy for the first three or four months. However, in these cases the uterus will grow abnormally fast. By the end of the third month, if not earlier, the woman will experience vaginal bleeding ranging from scant spotting to excessive bleeding. She may have hyperthyroidism (overproduction of thyroid hormones causing symptoms such as weight loss, increased appetite, and intolerance to heat). Sometimes, the grapelike cluster of cells itself will be shed with the blood during this time. Other symptoms may include severe nausea and vomiting and high blood pressure. As the pregnancy progresses, the fetus will not move and there will be no fetal heartbeat.

Diagnosis
The physician may not suspect a molar pregnancy until after the third month or later, when the absence of a fetal heartbeat together with bleeding and severe nausea and vomiting indicates something is amiss. First, the physician will examine the woman's abdomen, feeling for any strange lumps or abnormalities in the uterus. A tubal pregnancy, which can be life threatening if not treated, will be ruled out. Then the physician will check the levels of human chorionic gonadotropin (hCG), a hormone that is normally produced by a placenta or a mole. Abnormally high levels of hCG together with the symptoms of vaginal bleeding, lack of fetal heartbeat, and an unusually large uterus all indicate a molar pregnancy. An ultrasound of the uterus to make sure there is no living fetus will confirm the diagnosis.

Treatment
It is extremely important to make sure that all of the mole is removed from the uterus, since it is possible that the tissue is potentially cancerous. Often, the tissue is naturally expelled by the fourth month of pregnancy. In some instances, the physician will give the woman a drug called oxytocin to trigger the release of the mole that is not spontaneously aborted.

If this does not happen, however, a vacuum aspiration can be performed to remove the mole. In a procedure similar to a dilatation and curettage (D & C), a woman is given an anesthetic (to deaden feeling during the procedure), her cervix (the structure at the bottom of the uterus) is dilated and the contents of the uterus is gently suctioned out. After the mole has been mostly removed, gentle scraping of the uterus lining is usually performed. If the woman is older and does not want any more children, the uterus can be surgically removed (hysterectomy) instead of a vacuum aspiration because of the higher risk of cancerous moles in this age group. Because of the cancer risk, the physician will continue to monitor the patient for at least two months after the end of a molar pregnancy. Since invasive disease is usually signaled by high levels of hCG that don't go down after the pregnancy has ended, the woman's hCG levels will be checked every two weeks. If the levels don't return to normal by that time, the mole may have become cancerous. If the hCG level is normal, the woman's hCG will be tested each month for six months, and then every two months for a year. If the mole has become cancerous, treatment includes removal of the cancerous issue and chemotherapy. If the cancer has spread to other parts of the body, radiation will be added. Specific treatment depends on how advanced the cancer is. Women should make sure not to become pregnant within a year after hCG levels have returned to normal. If a woman were to become pregnant sooner than that, it would be difficult to tell whether the resulting high levels of hCG were caused by the pregnancy or a cancer from the mole.

Prognosis
A woman with a molar pregnancy often goes through the same emotions and sense of loss as does a woman who has a miscarriage. Most of the time, she truly believed she was pregnant and now has suffered a loss of the baby she thought she was carrying. In addition, there is the added worry that the tissue left behind could become cancerous. In the unlikely case that the mole is cancerous the cure rate is almost 100%. As long as the uterus was not removed, it would still be possible to have a child at a later time.

Supplement

Choriocarcinoma is a quick-growing form of cancer that occurs in a woman's uterus (womb). The abnormal cells start in the tissue that would normally become the placenta, the organ that develops during pregnancy to feed the fetus. Choriocarcinoma is a type of gestational trophoblastic disease.

Causes
Choriocarcinoma is an uncommon, but very often curable cancer that occurs during pregnancy. A baby may or may not develop in these types of pregnancy. The cancer may occur after a normal pregnancy. However, it most often occurs with a complete hydatidiform mole. The abnormal tissue from the mole can continue to grow even after it is removed, and can turn into cancer. About half of all women with a choriocarcinoma had a hydatidiform mole, or molar pregnancy. Choriocarcinomas may also occur after an early pregnancy that doesn't continue (miscarriage), ectopic pregnancy, or genital tumor.

Symptoms
A possible symptom is vaginal bleeding in a woman who recently had a hydatidiform mole or pregnancy. Other symptoms may include: Irregular vaginal bleeding Pain

Exams and Tests

A pregnancy test will be positive even if you are not pregnant. Pregnancy hormone (HCG) levels will be high. A pelvic exam may show uterine swelling or a tumor. Blood tests that may be done include: Quantitative serum HCG Complete blood count Kidney function tests Liver function tests Imaging tests that may be done include: CT scan MRI You should be carefully monitored after a hydatidiform mole or at the end of a pregnancy. Getting diagnosed with choriocarcinoma early can improve the outcome.

Treatment
After you are diagnosed, a careful history and exam will be done to make sure the cancer has not spread to other organs. Chemotherapy is the main type of treatment. A hysterectomy and radiation therapy are rarely needed.

Support Groups
For additional information, see cancer resources.

Outlook (Prognosis)
Most women whose cancer has not spread can be cured and will still be able to have children. A choriocarcinoma may come back within a few months to 3 years after treatment. The condition is harder to cure if the cancer has spread and one or more of the following happens: Disease spreads to the liver or brain