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{{SK}} Molar pregancy, Complete mole, Incomplete mole, Complete hydatidiform mole, Incomplete hydatidiform mole, Hmole, Complete hmole, Incomplete hmole.


==Overview==
==Overview==


'''Hydatidiform mole''' (or '''mola hydatidiforma''') is 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). It consists of a nonviable embryo which implants and proliferates within the uterus.<ref>''Robbins and Cotran's Pathological Basis of Disease, 7th ed.'', p. 1110</ref>  The term is derived from ''hydatidiform'' ("like a bunch of grapes") and ''mole'' (from Latin ''mola'' = millstone).  
'''Hydatidiform mole''' (or '''mola hydatidiforma''') is a common complication of pregnancy, it consists of a nonviable embryo which implants and proliferates within the uterus.<ref>''Robbins and Cotran's Pathological Basis of Disease, 7th ed.'', p. 1110</ref>  The term is derived from ''hydatidiform'' ("like a bunch of grapes") and ''mole'' (from Latin ''mola'' = millstone).  
 
Most moles present with painless vaginal bleeding during the second trimester of pregnancy. They are diagnosed by ultrasound imaging. Extremely high levels of [[human chorionic gonadotropin]] (HCG) are suggestive, but not diagnostic, of molar pregnancy.<ref>McPhee S. and Ganong W.F. ''Pathophysiology of Disease, 5th ed.'', p. 639.</ref> Hydatidiform moles are surgically removed by [[curettage]], in order to avoid the risks of [[choriocarcinoma]].<ref>''Robbins and Cotran's Pathological Basis of Disease, 7th ed.'', p. 1112</ref>
==Incidence and Mortality==
Hydatidiform mole is 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).<ref>Title of page. Website title (date). URL Accessed on Month Day, 2015</ref>
 
Region No of cases Ratio per 100 000 Ref Pregnancies Deliveries Live births Europe Denmark 1520 110 ·· ·· 6 England and Wales 5124 ·· ·· 154 7 Italy 347 66 ·· ·· 8 Sweden ·· 91 146 ·· 9 North America Canada 171 83 ·· ·· 10 United States ·· ·· 389 ·· 11 128 ·· ·· 460 12 185 108 ·· ·· 13 180 121 ·· ·· 14 939 121 ·· 145 15 Oceania Australia 455 ·· ·· 74 16 New Zealand 350 67 ·· ·· 17 Latin America Brazil 65 465 ·· ·· 18 Mexico 83 ·· ·· 240 19 Paraguay 227 23 ·· ·· 20 ·· 25 ·· ·· 21 Middle East Iran 113 320 ·· 370 22 Saudi Arabia ·· ·· 223 ·· 23 71 ·· ·· 148 24 Turkey 2227 580 260 ·· 25 Asia China 8832 81 ·· ·· 26 Indonesia 406 1299 1754 ·· 27 Japan 91 ·· ·· 165 28 Korea 3831 ·· 230 ·· 29 Pakistan 413 389 212 ·· 30 Singapore 198 ·· 115 ·· 31 Africa Nigeria ·· ·· 543 ·· 32


Most moles present with painless vaginal bleeding during the second trimester of pregnancy. They are diagnosed by ultrasound imaging. Extremely high levels of [[human chorionic gonadotropin]] (HCG) are suggestive, but not diagnostic, of molar pregnancy.<ref>McPhee S. and Ganong W.F. ''Pathophysiology of Disease, 5th ed.'', p. 639.</ref> Today moles are surgically removed by [[curettage]], in order to avoid the risks of [[choriocarcinoma]].<ref>''Robbins and Cotran's Pathological Basis of Disease, 7th ed.'', p. 1112</ref>Gestational trophoblastic disease (GTD) may be classified as follows:[1]
==Classification==
==Classification==
Gestational trophoblastic disease (GTD) may be classified as follows:
Gestational trophoblastic disease (GTD) may be classified as follows:<ref name="abc">Cellular Classification of Gestational Trophoblastic Disease. National Cancer Institute. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq/#section/_5 Accessed on October 8, 2015</ref>
:* Hydatidiform mole (HM)
:* Hydatidiform mole (HM)
::* Complete HM
::* Complete HM
::* Partial HM
::* Partial HM
==Pathophysiology==
==Pathophysiology==
A mole is characterized by a [[conceptus]] of [[hyperplasia|hyperplastic]] [[trophoblast]]ic tissue attached to the [[placenta]]. The conceptus does not contain the [[inner cell mass]] (the mass of cells inside the primordial embryo that will eventually give rise to the fetus).
A [[hydatidiform mole]] is characterized by a [[conceptus]] of [[hyperplasia|hyperplastic]] [[trophoblast]]ic tissue attached to the [[placenta]]. The conceptus does not contain the [[inner cell mass]].
The hydatidiform mole can be of two types: a ''complete mole'', in which the abnormal embryonic tissue is derived from the father only; and a ''partial mole'', in which the abnormal tissue is derived from both parents.
The hydatidiform mole can be of two types:  
 
* '''Complete moles''' usually occur when an empty ovum is fertilized by a sperm that then duplicates its own DNA. A 46, XY [[genotype]] may occur when 2 sperm (one 23, X and the other 23, Y) fertilize an empty egg.<ref name="aaa">{{cite journal |author=Woo J, Hsu C, Fung L, Ma H |title=Partial hydatidiform mole: ultrasonographic features |journal=Aust N Z J Obstet Gynaecol |volume=23 |issue=2 |pages=103-7 |year=1983 |pmid=6578773}}</ref> Their DNA is purely paternal in origin, and is diploid. Ninety percent are 46,XX, and 10% are 46,XY. In a complete mole, the [[fetus]] fails to develop.
* '''Complete moles''' usually occur when an empty ovum is fertilized by a sperm that then duplicates its own DNA (a process called ''androgenesis''). This explains why most complete moles are of the 46,XX genotype. A 46, XY genotype may occur when 2 sperm (one 23, X and the other 23, Y) fertilize an empty egg. They grossly resemble a bunch of grapes ("cluster of grapes" or "honeycombed uterus" or "snow-storm"<ref>{{cite journal |author=Woo J, Hsu C, Fung L, Ma H |title=Partial hydatidiform mole: ultrasonographic features |journal=Aust N Z J Obstet Gynaecol |volume=23 |issue=2 |pages=103-7 |year=1983 |pmid=6578773}}</ref>). Their DNA is purely paternal in origin (since all chromosomes are derived from the sperm), and is diploid (i.e. there are two copies of every chromosome). Ninety percent are 46,XX, and 10% are 46,XY. In a complete mole, the fetus fails to develop, thus on gross examination there are no signs of fetal tissue. All of the [[chorionic villi]] are enlarged. T
* '''Partial moles''' can develop either if a normal [[haploid]] ovum is fertilized by two [[sperm]] or if an ovum is [[fertilize]]d by one sperm which further duplicates its [[chromosomes]]. Thus their DNA is both maternal and paternal in origin. They can be triploid (e.g. 69 XXX, 69 XXY) or even tetraploid.
 
* '''Partial moles''' can occur if a normal [[haploid]] ovum is fertilized by two sperm, or, if fertilized by one sperm, if the paternal chromosomes become duplicated. Thus their DNA is both maternal and paternal in origin. They can be triploid (e.g. 69 XXX, 69 XXY) or even tetraploid. Fetal parts are often seen on gross examination.
 
 
==Natural history==
A hydatidiform mole is a pregnancy/[[conceptus]] in which the [[placenta]] contains grapelike vesicles that are usually visible with the naked eye. The vesicles arise by distention of the [[chorionic villi]] by fluid. When inspected in the microscope, [[hyperplasia]] of the [[trophoblastic]] tissue is noted. If left untreated, a hydatidiform mole will almost always end as a spontaneous abortion.
 
Based on [[morphology (biology)|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).<ref>{{cite journal |author=Di Cintio E, Parazzini F, Rosa C, Chatenoud L, Benzi G |title=The epidemiology of gestational trophoblastic disease |journal=Gen Diagn Pathol |volume=143 |issue=2-3 |pages=103–8 |year=1997 |pmid=9443567 |doi=}}</ref>
 
In rare cases a hydatidiform mole co-exists in the uterus with a normal, viable fetus. These cases are due to [[twins|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.<ref>{{cite journal |author=Sebire NJ, Foskett M, Paradinas FJ, ''et al'' |title=Outcome of twin pregnancies with complete hydatidiform mole and healthy co-twin |journal=Lancet |volume=359 |issue=9324 |pages=2165–6 |year=2002 |month=June |pmid=12090984 |doi=10.1016/S0140-6736(02)09085-2 |url=}}</ref>


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]].<ref>{{MedlinePlus|000909|Hydatidiform mole}}</ref> 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.<ref>{{cite journal |author=Paoloni-Giacobino A. |title=Epigenetics in reproductive medicine |journal=Paediatr Res. |volume=May 61 |issue=5 Pt 2 |pages=51R–57R |year=2007 |pmid=17413849 |doi=}}</ref>
==Pathology==
 
Shown below is a table depicting the gross and microscopic pathology of hydatidiform mole.<ref>Gestational trophoblastic disease. http://librepathology.org/wiki/index.php/Gestational_trophoblastic_disease#Hydatidiform_moles. URL Accessed on Nov 2, 2015</ref>
===Parental origin===
{| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;"
In most hydatidiform moles, the parental origin of the [[gene]]s in the cellular [[Cell nucleus|nucleus]] is abnormal.
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|''' Type of hydatidiform mole'''}}
 
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Gross pathology'''}}
In most complete moles, all [[nuclear gene]]s are inherited from the father, only (androgenesis). In approximately 80% of these androgenetic moles, the most probable mechanism is that an empty [[ovum|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.
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Microscopic pathology'''}}
 
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Immunohistochemistry'''}}
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.<ref>{{cite journal |author=Lawler SD, Fisher RA, Dent J |title=A prospective genetic study of complete and partial hydatidiform moles |journal=Am J Obstet Gynecol. |volume=164 |issue=5 Pt 1 |pages=1270–7 |year=1991 |month=May |pmid=1674641 |doi= |url=}}</ref><ref>{{cite journal |author=Wallace DC, Surti U, Adams CW, Szulman AE |title=Complete moles have paternal chromosomes but maternal mitochondrial DNA |journal=Human genetics |volume=61 |issue=2 |pages=145–7 |year=1982 |pmid=6290372 |doi= |url=http://www.springerlink.com/link.asp?id=w2555w4k4805h4h9}}</ref><ref>{{cite journal |author=Slim R, Mehio A |title=The genetics of hydatidiform moles: new lights on an ancient disease |journal=Clin Genet. |volume=71 |issue=1 |pages=25–34 |year=2007 |month=January |pmid=17204043 |doi=10.1111/j.1399-0004.2006.00697.x |url=}} Review.</ref>
 
 
==Etiology==
 
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]].
 
 
==Staging==
According to the Féderation Internationale de Gynécologie et d’Obstétrique (FIGO) [[cancer staging]] system, there are 4 stages of choriocarcinoma.<ref name= eee>Stage Information for Gestational Trophoblastic Disease. National Cancer Institute. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq#section/_11 URL Accessed on October 7, 2015</ref>
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
|valign=top|
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Stage}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|FIGO Anatomical Staging}}
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
I
| style="padding: 5px 5px; background: #F5F5F5;" |
Disease confined to the uterus
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
| Complete hydatidiform mole||
II
* Snowstorm
| style="padding: 5px 5px; background: #F5F5F5;" |
| '''Chorionic villi''':  
GTN extends outside of the uterus, but is limited to the genital structures (adnexa, vagina, broad ligament)
* All abnormal, bizarre; often not ovoid
|-
* Fissures/slit-like gaps
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
'''Cisterns''':  
III
* Well-developed
| style="padding: 5px 5px; background: #F5F5F5;" |
'''Blood vessels'''
GTN extends to the lungs, with or without known genital tract involvement
* Canalicular (thin walled) / few
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
IV
| style="padding: 5px 5px; background: #F5F5F5;" |
All other metastatic sites
|}


The modified World Health Organization [WHO] prognostic scoring system is as follows:
|p57(KIP2) -ve
{| {{table}}
| align="center" style="background:#f0f0f0;"|'''Modified WHO Prognostic Scoring System as Adapted by FIGO'''
|-
| Scores||0||1||2||4
|-
| Age||<40||≥40||–||–
|-
| Antecedent pregnancy||mole||abortion||term||–
|-
| Interval months from index pregnancy||<4||4–6||7–12||>12
|-
| Pretreatment serum hCG (iu/1)||<103||103–104||104–105||>105
|-
| Largest tumor size (including uterus)||<3||3–4 cm||≥5 cm||–
|-
|-
| Site of metastases||lung||spleen, kidney||gastrointestinal||liver, brain
|Incomplete hydatidiform mole||
* Grape-like clusters
|'''Chorionic villi''':
* Large villi, villi with cisterns, villi with cytotrophoblastic inclusions
* Jagged, still quasi ovoid
'''Cisterns''':
* Poorly developed / small
'''Blood vessels'''
* Present
|p57(KIP2) +ve
|-
|-
| Number of metastases||–||1–4||5–8||>8
|-
| Previous failed chemotherapy||–||–||single drug||≥2 drugs
|}
|}
{{#ev:youtube|QO3QF6n4xuo}}


==Symptoms==
==Symptoms==
===Early symptoms of gestational trophoblastic disease===
===Early symptoms of gestational trophoblastic disease===
* [[Vaginal bleeding]]
* [[Vaginal bleeding]]<ref name="xxx">Signs and symptoms of gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/signs-and-symptoms/?region=ns Accessed on October 10, 2015</ref>
* [[Nausea]] and [[vomiting]]  
* [[Nausea]] and [[vomiting]]  
* Passing of  tissue resembling a “bunch of grapes” from the vagina
* Passing of  tissue resembling a “bunch of grapes” from the vagina
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==Laboratory Findings==
==Laboratory Findings==
===Quantitative serum HCG===  
===Quantitative serum HCG===  
* [[Human chorionic gonadotropin]] (HCG or b-HCG) is the most common [[tumor]] marker test used to diagnose GTD<ref name=abc> Diagnosing gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/diagnosis/?region=ns Accessed on October 13, 2015</ref>  
* [[Human chorionic gonadotropin]] (HCG or b-HCG) is the most common [[tumor]] marker test used to diagnose GTD<ref name="abc">Diagnosing gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/diagnosis/?region=ns Accessed on October 13, 2015</ref>  
* HCG is a very sensitive test for diagnosing most gestational trophoblastic tumors. HCG is usually measured in the blood, but it can also be measured in the [[urine]]
* HCG is a very sensitive test for diagnosing most gestational trophoblastic tumors. HCG is usually measured in the blood, but it can also be measured in the [[urine]]
* HCG levels are much higher in women with complete hydatidiform mole compared to HCG levels in women with a normal [[pregnancy]]
* HCG levels are much higher in women with complete hydatidiform mole compared to HCG levels in women with a normal [[pregnancy]]
Line 139: Line 98:
* [[Complete blood count]] can check for [[anemia ]] from long-term (chronic) [[vaginal bleeding]].
* [[Complete blood count]] can check for [[anemia ]] from long-term (chronic) [[vaginal bleeding]].
===Thyroid-stimulating hormone===
===Thyroid-stimulating hormone===
Sometimes symptoms of [[hyperthyroidism]] are seen, due to the extremely high levels of hCG, which can mimic the normal [[Thyroid-stimulating hormone]] (TSH).<ref>McPhee S. and Ganong W.F. ''Pathophysiology of Disease, 5th ed.'', p. 639.</ref>
Sometimes symptoms of [[hyperthyroidism]] are seen, due to the extremely high levels of hCG, which can mimic the normal [[thyroid-stimulating hormone]] (TSH).<ref>McPhee S. and Ganong W.F. ''Pathophysiology of Disease, 5th ed.'', p. 639.</ref>


==Diagnostic Findings==
==Diagnostic Findings==


=== Ultrasonography ===
=== Ultrasonography===
 
[[Image:Molar_pregnancy.jpg|thumb|left|300px|Snowstorm appearance (hydatidiform mole) <ref>https://en.wikipedia.org/wiki/Molar_pregnancy</ref>]]
*Complete hydatidiform mole has a classic sonographic appearance of a solid collection of echoes with numerous anechoic spaces (snowstorm appearance).
<br clear="left" />
*In partial moles, the placenta is enlarged and contains areas of multiple, diffuse anechoic lesions.
'''Complete hydatidiform mole'''
 
* Enlarged [[uterus]]<ref>Hydatidiform mole. http://radiopaedia.org/articles/hydatidiform-mole. URL Accessed on October 28, 2015</ref>
[[Image:snowstorm ultrasound.jpg|thumb|left|300px|Snow storm ultrasound (hydatidiform mole) <ref>http://picasaweb.google.com/mcmumbi/USMLEIIImages</ref>]]
* Classic sonographic appearance is that of a solid collection of echoes with numerous small (3-10 mm 6) anechoic spaces (snowstorm or granular appearance)
<br clear="left"/>
* The term snowstorm sign usually dates back to earlier ultrasound equipment with inferior resolution
* The molar tissue demonstrates the bunch of grapes sign which represents hydropic swelling of [[trophoblastic]] [[villi]]
* Variable appearance
* Normal interface between abnormal trophoblastic tissue and [[myometrium]]
* No identifiable fetal tissue or gestational sac is seen
* Color doppler interrogation may show high velocity, low impedance flow
'''Partial mole'''
* [[Placenta]] is enlarged and contains areas of multiple, diffuse anechoic lesions
* A [[fetus]] with severe structural abnormalities or growth restriction, oligohydramnios, or a deformed gestational sac may be noted
* Color doppler interrogation may show high velocity, low impedance flow
'''Other features may include'''
* [[Ovarian]] theca lutein cysts: may be seen bilaterally in 25-60% of cases


===CT===
===CT===


A CT scan usually demonstrates a normal-sized uterus with areas of low attenuation, an enlarged inhomogeneous uterus with a central area of low attenuation, or hypoattenuating foci surrounded by highly enhanced areas in the myometrium.
A CT scan usually demonstrates a normal-sized uterus with areas of low attenuation, an enlarged inhomogeneous [[uterus]] with a central area of low attenuation, or hypoattenuating foci surrounded by highly enhanced areas in the [[myometrium]].<ref>Hydatidiform mole. http://radiopaedia.org/articles/hydatidiform-mole. URL Accessed on October 28, 2015</ref>


[http://www.radswiki.net Images courtesy of RadsWiki]
[http://www.radswiki.net Images courtesy of RadsWiki]
 
[[Image:Complete-mole-001.jpg|thumb|left|300px|Complete mole]]
[[Image:Complete-mole-001.jpg|thumb|left|300px|Complete mole]]
<br clear="left"/>
<br clear="left" />
[[Image:Complete-mole-002.jpg|thumb|left|300px|Complete mole]]
[[Image:Complete-mole-002.jpg|thumb|left|300px|Complete mole]]
<br clear="left"/>
<br clear="left" />
[[Image:Complete-mole-003.jpg|thumb|left|300px|Complete mole]]
[[Image:Complete-mole-003.jpg|thumb|left|300px|Complete mole]]
<br clear="left"/>
<br clear="left" />
[[Image:Complete-mole-004.jpg|thumb|left|300px|Complete mole]]
[[Image:Complete-mole-004.jpg|thumb|left|300px|Complete mole]]
<br clear="left"/>
<br clear="left" />
[[Image:Complete-mole-005.jpg|thumb|left|300px|Complete mole]]
[[Image:Complete-mole-005.jpg|thumb|left|300px|Complete mole]]
<br clear="left"/>
<br clear="left" />
 
===Pathology===
 
{{#ev:youtube|QO3QF6n4xuo}}


==Treatment==
==Treatment==
'''Dilation and curettage''' (D&C)
<ref>{{cite book |author=Cotran RS, Kumar V, Fausto N, Nelso F, Robbins SL, Abbas AK |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |isbn=0-7216-0187-1 |edition=7th ed. |page=1112}}</ref>
* D&C is a treatment option for women diagnosed with complete or partial hydatidiform moles by [[human chorionic gonadotropin]] (HCG or b-HCG) testing or [[ultrasound]].
* D&C will not be done if a gestational choriocarcinoma is suspected because this type of tumor bleeds very easily.
* Management is more complicated when the mole occurs together with one or more normal [[fetus]]es.
* D&C is a treatment option for women diagnosed with complete or partial hydatidiform moles by human chorionic gonadotropin (HCG or b-HCG) testing or ultrasound. D&C will not be done if a gestational [[choriocarcinoma]] is suspected because this type of tumor bleeds very easily.


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]].<ref>{{cite book |author=Cotran RS, Kumar V, Fausto N, Nelso F, Robbins SL, Abbas AK |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |isbn=0-7216-0187-1 |edition=7th ed. |page=1112}}</ref> 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%.  
'''Chemotherapy '''
*Indications:<ref name="xxx">Hydatidiform Mole Management. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq#section/_86. URL Accessed on October 26, 2015</ref>
:* Chemotherapy is necessary when patients present with the following:
:* A rising [[beta-hCG]] titer for 2 weeks (3 titers)
:* A tissue diagnosis of [[choriocarcinoma]]
:* A plateau of the beta-hCG for 3 weeks
:* Persistence of detectable beta-hCG 6 months after mole evacuation
:* Metastatic disease
:* An elevation in beta-hCG after a normal value
:* Postevacuation hemorrhage not caused by retained tissues
* [[Chemotherapy]] is ultimately required for persistence or neoplastic transformation in about 15% to 20% of patients after evacuation of a complete HM but for fewer than 5% of patients with partial HM. Chemotherapy is determined by the patient's modified World Health Organization score.


Patients are advised not to conceive for one year after a molar pregnancy. The chances of having another molar pregnancy are approximately 1%.
* Studies have shown that a single course of prophylactic [[dactinomycin ]] or [[methotrexate]] can decrease the risk of a postmolar gestational trophoblastic disease (GTD). However, there is concern that chemoprophylaxis increases tumor resistance to standard therapy in the women who subsequently develop GTD. Therefore, this practice is generally limited to countries in which a large number of women do not return for follow-up.
 
Management is more complicated when the mole occurs together with one or more normal [[fetus]]es.
 
[[Carboprost]] medication may be used to contract the [[uterus]].


==Prognosis==   
==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.
* 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. These 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.
* In 10 to 15% of cases, hydatidiform moles may develop into invasive moles. These 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.


In 2 to 3% of cases, hydatidiform moles may develop into [[choriocarcinoma]], which is a malignant, rapidly-growing, and metastatic (spreading) form of cancer. Despite these factors which normally indicate a poor prognosis, the rate of cure after treatment with chemotherapy is high.
* In 2 to 3% of cases, hydatidiform moles may develop into [[choriocarcinoma]], which is a malignant, rapidly-growing, and metastatic (spreading) form of cancer. Despite these factors which normally indicate a poor prognosis, the rate of cure after treatment with chemotherapy is high.


Over 90% of women with malignant, non-spreading cancer are able to survive and retain their ability to have children. In those with metastatic (spreading) cancer, remission remains at 75 to 85%, although the ability to have children is usually lost.
* Over 90% of women with malignant, non-spreading cancer are able to survive and retain their ability to have children. In those with metastatic (spreading) cancer, remission remains at 75 to 85%, although the ability to have children is usually lost.
 
In women with complete HM, risk of persistence or neoplastic transformation is approximately doubled in the setting of certain characteristics, which include the following:<ref name="xxx">Hydatidiform Mole Management. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq#section/_86. URL Accessed on October 26, 2015</ref>
* Age older than 35 years or age younger than 20 years
* Pre-evacuation serum beta-hCG greater than 100,000 IU/L
* Large-for-date [[uterus]]
* Large uterine molar mass
* Large (>6 cm) [[ovarian]] cysts
* [[Pre-eclampsia]]
* [[Hyperthyroidism]]
* Hyperemesis of [[pregnancy]]
* Trophoblastic embolization
* [[Disseminated intravascular coagulation]]
 
The modified World Health Organization [WHO] prognostic scoring system is as follows:<ref name="eee">Stage Information for Gestational Trophoblastic Disease. National Cancer Institute. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq#section/_11 URL Accessed on October 27, 2015</ref>
{| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;"
| align="center" style="background:#f0f0f0;" |'''Modified WHO Prognostic Scoring System as Adapted by FIGO'''
|-
| Scores||0||1||2||4
|-
| Age||<40||≥40||–||–
|-
| Antecedent pregnancy||mole||abortion||term||–
|-
| Interval months from index pregnancy||<4||4–6||7–12||>12
|-
| Pretreatment serum hCG (iu/1)||<103||103–104||104–105||>105
|-
| Largest tumor size (including uterus)||<3||3–4 cm||≥5 cm||–
|-
| Site of metastases||lung||spleen, kidney||gastrointestinal||liver, brain
|-
| Number of metastases||–||1–4||5–8||>8
|-
| Previous failed chemotherapy||–||–||single drug||≥2 drugs
|}


==References==
==References==
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{{Pathology of pregnancy, childbirth and the puerperium}}
{{Pathology of pregnancy, childbirth and the puerperium}}
{{Soft tissue tumors and sarcomas}}
{{Soft tissue tumors and sarcomas}}
[[ar:حمل عنقودي]]
[[fr:Môle hydatiforme]]
[[de:Blasenmole]]
[[nl:Mola-zwangerschap]]
[[pt:Mola hidatiforme]]
[[sv:Druvbörd]]




Latest revision as of 13:07, 25 February 2019

For patient information, click here

Hydatidiform mole
MOLE, HYDATIDIFORM, HYSTERECTOMY SPECIMEN. VARIABLY SIZED VESICLES, NO NORMAL PLACENTA, NO FETUS. Image courtesy of Professor Peter Anderson DVM PhD and published with permission. © PEIR, University of Alabama at Birmingham, Department of Pathology

Template:Search infobox Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Monalisa Dmello, M.B,B.S., M.D. [2]

Synonyms and keywords: Molar pregancy, Complete mole, Incomplete mole, Complete hydatidiform mole, Incomplete hydatidiform mole, Hmole, Complete hmole, Incomplete hmole.

Overview

Hydatidiform mole (or mola hydatidiforma) is a common complication of pregnancy, it consists of a nonviable embryo which implants and proliferates within the uterus.[1] The term is derived from hydatidiform ("like a bunch of grapes") and mole (from Latin mola = millstone).

Most moles present with painless vaginal bleeding during the second trimester of pregnancy. They are diagnosed by ultrasound imaging. Extremely high levels of human chorionic gonadotropin (HCG) are suggestive, but not diagnostic, of molar pregnancy.[2] Hydatidiform moles are surgically removed by curettage, in order to avoid the risks of choriocarcinoma.[3]

Incidence and Mortality

Hydatidiform mole is 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]

Region No of cases Ratio per 100 000 Ref Pregnancies Deliveries Live births Europe Denmark 1520 110 ·· ·· 6 England and Wales 5124 ·· ·· 154 7 Italy 347 66 ·· ·· 8 Sweden ·· 91 146 ·· 9 North America Canada 171 83 ·· ·· 10 United States ·· ·· 389 ·· 11 128 ·· ·· 460 12 185 108 ·· ·· 13 180 121 ·· ·· 14 939 121 ·· 145 15 Oceania Australia 455 ·· ·· 74 16 New Zealand 350 67 ·· ·· 17 Latin America Brazil 65 465 ·· ·· 18 Mexico 83 ·· ·· 240 19 Paraguay 227 23 ·· ·· 20 ·· 25 ·· ·· 21 Middle East Iran 113 320 ·· 370 22 Saudi Arabia ·· ·· 223 ·· 23 71 ·· ·· 148 24 Turkey 2227 580 260 ·· 25 Asia China 8832 81 ·· ·· 26 Indonesia 406 1299 1754 ·· 27 Japan 91 ·· ·· 165 28 Korea 3831 ·· 230 ·· 29 Pakistan 413 389 212 ·· 30 Singapore 198 ·· 115 ·· 31 Africa Nigeria ·· ·· 543 ·· 32

Classification

Gestational trophoblastic disease (GTD) may be classified as follows:[5]

  • Hydatidiform mole (HM)
  • Complete HM
  • Partial HM

Pathophysiology

A hydatidiform mole is characterized by a conceptus of hyperplastic trophoblastic tissue attached to the placenta. The conceptus does not contain the inner cell mass. The hydatidiform mole can be of two types:

  • Complete moles usually occur when an empty ovum is fertilized by a sperm that then duplicates its own DNA. A 46, XY genotype may occur when 2 sperm (one 23, X and the other 23, Y) fertilize an empty egg.[6] Their DNA is purely paternal in origin, and is diploid. Ninety percent are 46,XX, and 10% are 46,XY. In a complete mole, the fetus fails to develop.
  • Partial moles can develop either if a normal haploid ovum is fertilized by two sperm or if an ovum is fertilized by one sperm which further duplicates its chromosomes. Thus their DNA is both maternal and paternal in origin. They can be triploid (e.g. 69 XXX, 69 XXY) or even tetraploid.

Pathology

Shown below is a table depicting the gross and microscopic pathology of hydatidiform mole.[7]

Type of hydatidiform mole Gross pathology Microscopic pathology Immunohistochemistry
Complete hydatidiform mole
  • Snowstorm
 Chorionic villi:
  • All abnormal, bizarre; often not ovoid
  • Fissures/slit-like gaps

Cisterns:

  • Well-developed

Blood vessels

  • Canalicular (thin walled) / few
 p57(KIP2) -ve
Incomplete hydatidiform mole
  • Grape-like clusters
 Chorionic villi:
  • Large villi, villi with cisterns, villi with cytotrophoblastic inclusions
  • Jagged, still quasi ovoid

Cisterns:

  • Poorly developed / small

Blood vessels

  • Present
 p57(KIP2) +ve

{{#ev:youtube|QO3QF6n4xuo}}

Symptoms

Early symptoms of gestational trophoblastic disease

Rare symptoms of gestational trophoblastic disease

  • Headache
  • Edema of the hands and feet
  • Abdominal or pelvic pain
  • Vaginal discharge
  • Overactive thyroid gland (hyperthyroidism) that causes:

Diagnosis

Laboratory Findings

Quantitative serum HCG

  • Human chorionic gonadotropin (HCG or b-HCG) is the most common tumor marker test used to diagnose GTD[5]
  • HCG is a very sensitive test for diagnosing most gestational trophoblastic tumors. HCG is usually measured in the blood, but it can also be measured in the urine
  • HCG levels are much higher in women with complete hydatidiform mole compared to HCG levels in women with a normal pregnancy
  • With partial moles, the HCG level is higher than normal, but it is not as high as with other types of GTD
  • An HCG test can help find GTD after pregnancy or miscarriage as this hormone should not be present in the blood or urine soon afterward

Complete blood count

Thyroid-stimulating hormone

Sometimes symptoms of hyperthyroidism are seen, due to the extremely high levels of hCG, which can mimic the normal thyroid-stimulating hormone (TSH).[9]

Diagnostic Findings

Ultrasonography

Snowstorm appearance (hydatidiform mole) [10]


Complete hydatidiform mole

  • Enlarged uterus[11]
  • Classic sonographic appearance is that of a solid collection of echoes with numerous small (3-10 mm 6) anechoic spaces (snowstorm or granular appearance)
  • The term snowstorm sign usually dates back to earlier ultrasound equipment with inferior resolution
  • The molar tissue demonstrates the bunch of grapes sign which represents hydropic swelling of trophoblastic villi
  • Variable appearance
  • Normal interface between abnormal trophoblastic tissue and myometrium
  • No identifiable fetal tissue or gestational sac is seen
  • Color doppler interrogation may show high velocity, low impedance flow

Partial mole

  • Placenta is enlarged and contains areas of multiple, diffuse anechoic lesions
  • A fetus with severe structural abnormalities or growth restriction, oligohydramnios, or a deformed gestational sac may be noted
  • Color doppler interrogation may show high velocity, low impedance flow

Other features may include

  • Ovarian theca lutein cysts: may be seen bilaterally in 25-60% of cases

CT

A CT scan usually demonstrates a normal-sized uterus with areas of low attenuation, an enlarged inhomogeneous uterus with a central area of low attenuation, or hypoattenuating foci surrounded by highly enhanced areas in the myometrium.[12]

Images courtesy of RadsWiki

Complete mole


Complete mole


Complete mole


Complete mole


Complete mole


Treatment

Dilation and curettage (D&C) [13]

  • D&C is a treatment option for women diagnosed with complete or partial hydatidiform moles by human chorionic gonadotropin (HCG or b-HCG) testing or ultrasound.
  • D&C will not be done if a gestational choriocarcinoma is suspected because this type of tumor bleeds very easily.
  • Management is more complicated when the mole occurs together with one or more normal fetuses.
  • D&C is a treatment option for women diagnosed with complete or partial hydatidiform moles by human chorionic gonadotropin (HCG or b-HCG) testing or ultrasound. D&C will not be done if a gestational choriocarcinoma is suspected because this type of tumor bleeds very easily.

Chemotherapy

  • Indications:[8]
  • Chemotherapy is necessary when patients present with the following:
  • A rising beta-hCG titer for 2 weeks (3 titers)
  • A tissue diagnosis of choriocarcinoma
  • A plateau of the beta-hCG for 3 weeks
  • Persistence of detectable beta-hCG 6 months after mole evacuation
  • Metastatic disease
  • An elevation in beta-hCG after a normal value
  • Postevacuation hemorrhage not caused by retained tissues
  • Chemotherapy is ultimately required for persistence or neoplastic transformation in about 15% to 20% of patients after evacuation of a complete HM but for fewer than 5% of patients with partial HM. Chemotherapy is determined by the patient's modified World Health Organization score.
  • Studies have shown that a single course of prophylactic dactinomycin or methotrexate can decrease the risk of a postmolar gestational trophoblastic disease (GTD). However, there is concern that chemoprophylaxis increases tumor resistance to standard therapy in the women who subsequently develop GTD. Therefore, this practice is generally limited to countries in which a large number of women do not return for follow-up.

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. These 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.
  • In 2 to 3% of cases, hydatidiform moles may develop into choriocarcinoma, which is a malignant, rapidly-growing, and metastatic (spreading) form of cancer. Despite these factors which normally indicate a poor prognosis, the rate of cure after treatment with chemotherapy is high.
  • Over 90% of women with malignant, non-spreading cancer are able to survive and retain their ability to have children. In those with metastatic (spreading) cancer, remission remains at 75 to 85%, although the ability to have children is usually lost.

In women with complete HM, risk of persistence or neoplastic transformation is approximately doubled in the setting of certain characteristics, which include the following:[8]

The modified World Health Organization [WHO] prognostic scoring system is as follows:[14]

Modified WHO Prognostic Scoring System as Adapted by FIGO
Scores 0 1 2 4
Age <40 ≥40 – –
Antecedent pregnancy mole abortion term –
Interval months from index pregnancy <4 4–6 7–12 >12
Pretreatment serum hCG (iu/1) <103 103–104 104–105 >105
Largest tumor size (including uterus) <3 3–4 cm ≥5 cm –
Site of metastases lung spleen, kidney gastrointestinal liver, brain
Number of metastases – 1–4 5–8 >8
Previous failed chemotherapy – – single drug ≥2 drugs

References

  1. ↑ Robbins and Cotran's Pathological Basis of Disease, 7th ed., p. 1110
  2. ↑ McPhee S. and Ganong W.F. Pathophysiology of Disease, 5th ed., p. 639.
  3. ↑ Robbins and Cotran's Pathological Basis of Disease, 7th ed., p. 1112
  4. ↑ Title of page. Website title (date). URL Accessed on Month Day, 2015
  5. ↑ 5.0 5.1 Cellular Classification of Gestational Trophoblastic Disease. National Cancer Institute. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq/#section/_5 Accessed on October 8, 2015
  6. ↑ Woo J, Hsu C, Fung L, Ma H (1983). "Partial hydatidiform mole: ultrasonographic features". Aust N Z J Obstet Gynaecol. 23 (2): 103–7. PMID 6578773.
  7. ↑ Gestational trophoblastic disease. http://librepathology.org/wiki/index.php/Gestational_trophoblastic_disease#Hydatidiform_moles. URL Accessed on Nov 2, 2015
  8. ↑ 8.0 8.1 8.2 Signs and symptoms of gestational trophoblastic disease. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/gestational-trophoblastic-disease/signs-and-symptoms/?region=ns Accessed on October 10, 2015
  9. ↑ McPhee S. and Ganong W.F. Pathophysiology of Disease, 5th ed., p. 639.
  10. ↑ https://en.wikipedia.org/wiki/Molar_pregnancy
  11. ↑ Hydatidiform mole. http://radiopaedia.org/articles/hydatidiform-mole. URL Accessed on October 28, 2015
  12. ↑ Hydatidiform mole. http://radiopaedia.org/articles/hydatidiform-mole. URL Accessed on October 28, 2015
  13. ↑ Cotran RS, Kumar V, Fausto N, Nelso F, Robbins SL, Abbas AK (2005). Robbins and Cotran pathologic basis of disease (7th ed. ed.). St. Louis, Mo: Elsevier Saunders. p. 1112. ISBN 0-7216-0187-1.
  14. ↑ Stage Information for Gestational Trophoblastic Disease. National Cancer Institute. http://www.cancer.gov/types/gestational-trophoblastic/hp/gtd-treatment-pdq#section/_11 URL Accessed on October 27, 2015

See also


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