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The etymology is derived from hydatis (Greek "a drop of water"), referring to the watery
contents of the cysts, and mole (from Latin mola = millstone/false conception).[3] The term,
however, comes from the cyst's appearance looking very much like a hydatid cyst in an
Echinococcosis.
Contents
[hide]
• 1 Natural history
o 1.1 Parental origin
• 2 Clinical presentation and diagnosis
• 3 Treatment
• 4 Prognosis
• 5 See also
• 6 References
• 7 External links
Based on morphology, hydatidiform moles can be divided into two types: In complete moles, all
the chorionic villi are vesicular, and no sign of embryonic or fetal development is present. In
partial moles some villi are vesicular, whereas others appear more normal, and embryonic/fetal
development may be seen but the fetus is always malformed and is never viable.
Hydatidiform moles are a common complication of pregnancy, occurring once in every 1000
pregnancies in the US, with much higher rates in Asia (e.g. up to one in 100 pregnancies in
Indonesia).[4]
In rare cases a hydatidiform mole co-exists in the uterus with a normal, viable fetus. These cases
are due to twinning. The uterus contains two conceptuses: one with an abnormal placenta and no
viable fetus (the mole), and one with a normal placenta and a viable fetus. Under careful
surveillance it is often possible for the woman to give birth to the normal child and to be cured of
the mole.[5]
The etiology of this condition is not completely understood. Potential risk factors may include
defects in the egg, abnormalities within the uterus, or nutritional deficiencies. Women under 20
or over 40 years of age have a higher risk. Other risk factors include diets low in protein, folic
acid, and carotene.[6] The diploid set of sperm-only DNA means that all chromosomes have
sperm-patterned methylation suppression of genes. This leads to overgrowth of the
syncytiotrophoblast whereas dual egg-patterned methylation leads to a devotion of resources to
the embryo, with an underdeveloped syncytiotrophoblast. This is considered to be the result of
evolutionary competition with male genes driving for high investment into the fetus versus
female genes driving for resource restriction to maximise the number of children.[7]
In most complete moles, all nuclear genes are inherited from the father only (androgenesis). In
approximately 80% of these androgenetic moles, the most probable mechanism is that an empty
egg is fertilized by a single sperm, followed by a duplication of all chromosomes/genes (a
process called "endoreduplication"). In approximately 20% of complete moles the most probable
mechanism is that an empty egg is fertilised by two sperms. In both cases, the moles are diploid
(i.e. there are two copies of every chromosome). In all these cases, the mitochondrial genes are
inherited from the mother, as usual.
Most partial moles are triploid (three chromosome sets). The most probable mechanism is that a
normal haploid egg is fertilized by two sperms. Thus the nucleus contains one maternal set of
genes and two paternal sets.
In rare cases, hydatidiform moles are tetraploid (four chromosome sets) or have other
chromosome abnormalities.
A small percentage of hydatidiform moles have biparental diploid genomes, as in normal living
persons; they have two sets of chromosomes, one inherited from each biological parent. Some of
these moles occur in women who carry mutations in the gene NLRP7, predisposing them towards
molar pregnancy. These rare variants of hydatidiform mole may be complete or partial.[8][9][10]
The diagnosis is strongly suggested by ultrasound (sonogram), but definitive diagnosis requires
histopathological examination. The mole grossly resembles a bunch of grapes ("cluster of
grapes" or "honeycombed uterus" or "snow-storm"[12]). There is increased trophoblast
proliferation and enlarging of the chorionic villi.[13] Angiogenesis in the trophoblasts is impaired
as well. [13]
Sometimes symptoms of hyperthyroidism are seen, due to the extremely high levels of hCG,
which can mimic the normal Thyroid-stimulating hormone (TSH).[11]
[edit] Treatment
Hydatidiform moles should be treated by evacuating the uterus by uterine suction or by surgical
curettage as soon as possible after diagnosis, in order to avoid the risks of choriocarcinoma.[14]
Patients are followed up until their serum human chorionic gonadotrophin (hCG) level has fallen
to an undetectable level. Invasive or metastatic moles (cancer) may require chemotherapy and
often respond well to methotrexate. The response to treatment is nearly 100%. Patients are
advised not to conceive for one year after a molar pregnancy. The chances of having another
molar pregnancy are approximately 1%.
Management is more complicated when the mole occurs together with one or more normal
fetuses.
[edit] Prognosis
More than 80% of hydatidiform moles are benign. The outcome after treatment is usually
excellent. Close follow-up is essential. Highly effective means of contraception are
recommended to avoid pregnancy for at least 6 to 12 months.
In 10 to 15% of cases, hydatidiform moles may develop into invasive moles. This condition is
named persistent trophoblastic disesase (PTD). The moles may intrude so far into the uterine
wall that hemorrhage or other complications develop. It is for this reason that a post-operative
full abdominal and chest x-ray will often be requested.
Over 90% of women with malignant, non-spreading cancer are able to survive and retain their
ability to conceive and bear children. In those with metastatic (spreading) cancer, remission
remains at 75 to 85%, although their childbearing ability is usually lost.
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).
Pathophysiology
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:
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.8 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.
Frequency
United States
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.10 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.11
Mortality/Morbidity
Race
Differences in the frequency of hydatidiform moles between ethnic groups have been reported
internationally.10,16 In the United States, comparison of frequency of hydatidiform moles in
African Americans and Caucasians have yielded conflicting results.16 If differences exist,
whether they are due to genetic differences or environmental factors is not clear.
Sex
Age
Hydatidiform mole is more common at the extremes of reproductive age. Women in their early
teenage or perimenopausal years are most at risk.17,18,19,10,16 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.
Clinical
History
Physical
• Complete mole
o 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.
o 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.21
o 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.
• Partial mole
o Uterine enlargement and preeclampsia is reported in only 5% of
patients.23
o Theca lutein cysts, hyperemesis, and hyperthyroidism are extremely
rare.
• Twinning
o Twinning with a complete mole and a fetus with a normal placenta has
been reported. Cases of healthy infants in these circumstances have
been reported.24,8
o Women with coexistent molar and normal gestations are at higher risk
for developing persistent disease and metastasis25 . Termination of
pregnancy is a recommended option.
o 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.26
o Prenatal genetic diagnosis by chorionic villus sampling or
amniocentesis is recommended to evaluate the karyotype of the fetus.
Causes
Treatment
Medical Care
Surgical Care
Consultations
A gynecologic oncologist should be consulted if the patient is believed to be at risk for or has
developed malignant disease (ie, gestational trophoblastic neoplasia).
Diet
Activity
Medication
Prophylactic chemotherapy after evacuation of a hydatidiform mole is not routinely
recommended. Only 20% of women with hydatidiform mole will develop malignant sequelae
(i.e., Gestational Trophoblastic Neoplasia). Prophylactic chemotherapy may be considered in
patients who will not adhere to the required close follow up.
Hydatidiform mole
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Uterus
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Choriocarcinoma
Protein in diet
Beta-carotene test
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:
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.
A partial molar pregnancy means there is an abnormal placenta and some fetal development.
Both forms are due to problems during fertilization. Potential causes may include defects in the
egg, problems within the uterus, or a diet low in protein, animal fat, and vitamin A. Women
under age 16 or older than 40 have a higher risk for this condition. You also are more likely to
have a molar pregnancy if you have had one in the past.
Symptoms
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.
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.
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.
Possible Complications
Lung problems may occur after a D and C if the woman's uterus is bigger than 16 weeks
gestational size.
Alternative Names
Hydatidiform Mole
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Pronunciations
curettage
eclampsia
human chorionic
gonadotropin
hydatidiform
hysterectomy
metastasis
methotrexate
preeclampsia
ultrasonography
uterus
• Women appear to be pregnant, but the uterus enlarges much more rapidly
than in a normal pregnancy.
• Most women have severe nausea and vomiting, vaginal bleeding, and
very high blood pressure.
• Ultrasonography, blood tests to measure human chorionic gonadotropin
(which is produced early during pregnancy) and a biopsy are done.
• Moles are removed using dilation and curettage with suction.
• If the disorder persists, chemotherapy is needed.
Most often, a hydatidiform mole is an abnormal fertilized egg that develops into
a hydatidiform mole rather than a fetus (a condition called molar pregnancy).
However, a hydatidiform mole can develop from cells that remain in the uterus
after a miscarriage or a full-term pregnancy. Rarely, a hydatidiform mole
develops when there is a living fetus. In such cases, the fetus typically dies, and a
miscarriage often occurs.
Hydatidiform moles are most common among women under 17 or over 35. In the
United States, they occur in about 1 in 2000 pregnancies in the United States. For
unknown reasons, moles are almost 10 times more common in Asian countries.
Symptoms
Women who have a hydatidiform mole feel as if they are pregnant. But because
hydatidiform moles grow much faster than a fetus, the abdomen becomes larger
much faster than it does in a normal pregnancy. Severe nausea and vomiting are
common, and vaginal bleeding may occur. As parts of the mole deteriorate, small
amounts of tissue, which resemble a bunch of grapes, may pass through the
vagina. These symptoms indicate the need for prompt evaluation by a doctor.
Diagnosis
Often, doctors can diagnose a hydatidiform mole shortly after conception. The
pregnancy test is positive, but no fetal movement and no fetal heartbeat are
detected, and the uterus is much larger than expected.
Prognosis
The cure rate for a hydatidiform mole is virtually 100% if the mole has not
spread. The cure rate is 60 to 80% for choriocarcinoma that has spread widely.
Most women can have children afterwards and do not have a higher risk of
having complications, a miscarriage, or children with birth defects.
About 1% of women who have had a hydatidiform mole have another one. So if
women have had a hydatidiform mole, ultrasonography is done early in
subsequent pregnancies.
Treatment
A chest x-ray is done to see whether the mole has become cancerous (that is, a
choriocarcinoma) and spread to the lungs. After surgery, the level of human
chorionic gonadotropin in the blood is measured to determine whether the
hydatidiform mole was completely removed. When removal is complete, the
level returns to normal, usually within 10 weeks, and remains normal. If the level
does not return to normal (called persistent disease), computed tomography (CT)
of the brain, chest, abdomen, and pelvis is done to determine whether
choriocarcinoma has developed and spread.
Women who have had a hydatidiform mole removed are advised not to become
pregnant for 1 year. Oral contraceptives are frequently recommended, but other
effective contraceptive methods can be used.
Hydatidiform Mole
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A hydatidiform mole is the result of abnormal conception. It may cause bleeding in
early pregnancy and is usually diagnosed on an early pregnancy ultrasound scan. It
needs to be removed and most women can expect a full recovery.
Understanding conception
Most cells in the body have two copies of your individual genetic code. A complete genetic code
is needed for a baby to develop. This code determines what we look like, how our body
functions, whether we are male or female and sometimes even whether we will get certain
diseases. However, the reproductive cells (ova in women and sperm in men) have only one copy
of this code.
In normal conception, one sperm which contains a single copy of the father’s genetic code
fertilises one egg which contains a single copy of the mother’s genetic code. This results in a
fertilised egg or embryo with a complete set of genetic material.
A partial mole occurs when there are two copies of the father’s genetic code and one copy of the
mother’s genetic code. This is usually due to two sperm fertilising one normal ovum. In this
case, some foetal tissue and foetal red blood cells are present.
Partial and complete moles will not produce a live baby. They are non viable pregnancies.
If you do not have bleeding or other symptoms, the diagnosis is usually made when you have a
routine pregnancy ultrasound scan.
About 1 in 200 women with partial moles and 15 in 100 women with complete moles will
develop a cancer called persistent gestational trophoblastic neoplasia (GTN). This cancer can be
detected by a blood test. The blood test checks for the pregnancy hormone human chorionic
gonadotrophin (HCG). This is the hormone that is tested in standard pregnancy tests. When you
are first diagnosed with a hydatidiform mole, your HCG level will be raised as it is in normal
pregnancy. When the hydatidiform mole is treated, the HCG level will return to a normal non-
pregnant level and will remain at this level. If you develop persistent GTN, the HCG level will
rise. So, this blood test is a good way to check for the development of cancer. Your specialist
centre will advise you on how often to have this test done.
If you do develop persistent GTN you will need further treatment. The treatment depends on how
advanced the cancer is. For example, whether the cancer has spread to other parts of the body or
not. Treatment involves injections of different medications (chemotherapy) such as methotrexate,
etoposide and dactinomycin.
After a hydatidiform mole, you will be advised not to fall pregnant until your HCG levels have
been normal for six months. If you have had persistent GTN, you will be advised not to fall
pregnant until the levels have been normal for 12 months.
Only two women in 100 who fall pregnant after previously having a hydatidiform mole will
develop another hydatidiform mole.
Once your HCG levels have returned to normal, it is safe to use the contraceptive pill and HRT,
if needed.