Hydatidiform mole: Difference between revisions

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


==Overview==
==Overview==
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'''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).  
'''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> 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>
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==
==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).
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


==Classification==
==Classification==
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>
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
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==Pathophysiology==
==Pathophysiology==
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]].
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 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.
* '''Partial moles''' can occur if a normal [[haploid]] ovum is fertilized by two [[sperm]], or if [[fertilize]]d 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.
* '''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.
 
==Gross Pathology==
A molar pregnancy, grows into a mass in the uterus that has swollen chorionic villi. These villi grow in clusters that resemble grapes.


==Microscopic Pathology==
==Pathology==
Hyperplasia of the trophoblastic tissue
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>
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{| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;"
==Staging==
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|''' Type of hydatidiform mole'''}}
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>
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Gross pathology'''}}
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Microscopic pathology'''}}
|valign=top|
| align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Immunohistochemistry'''}}
|+
! 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
'''Cisterns''':  
* Well-developed
'''Blood vessels'''
* Canalicular (thin walled) / few
 
|p57(KIP2) -ve
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
|Incomplete hydatidiform mole||
III
* Grape-like clusters
| style="padding: 5px 5px; background: #F5F5F5;" |
|'''Chorionic villi''':  
GTN extends to the lungs, with or without known genital tract involvement
* Large villi, villi with cisterns, villi with cytotrophoblastic inclusions
* Jagged, still quasi ovoid
'''Cisterns''':
* Poorly developed / small
'''Blood vessels'''
* Present
|p57(KIP2) +ve
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
IV
| style="padding: 5px 5px; background: #F5F5F5;" |
All other metastatic sites
|}
|}
{{#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]]
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==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" />
 
 


==Treatment==
==Treatment==
'''D&C'''
'''Dilation and curettage''' (D&C)
* 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>  
<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 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 tumour bleeds very easily.
* 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.  
* 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 tumour bleeds very easily.
* 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.
 
 
* 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%.
* However, following the diagnosis and treatment of HM, patients should be monitored to rule out the possibility of metastatic gestational trophoblastic neoplasia.
* In almost all cases, this can be performed with routine monitoring of serum beta human chorionic gonadotropin (beta-hCG) to document its return to normal.
* An effective form of contraception is important during the follow-up period to avoid the confusion that can occur with a rising beta-hCG as a result of pregnancy.


'''Chemotherapy '''
'''Chemotherapy '''
* It is necessary when there is the following:
*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>
* A rising beta-hCG titer for 2 weeks (3 titers)
:* Chemotherapy is necessary when patients present with the following:
* A tissue diagnosis of choriocarcinoma
:* A rising [[beta-hCG]] titer for 2 weeks (3 titers)
* A plateau of the beta-hCG for 3 weeks
:* A tissue diagnosis of [[choriocarcinoma]]
* Persistence of detectable beta-hCG 6 months after mole evacuation
:* A plateau of the beta-hCG for 3 weeks
* Metastatic disease
:* Persistence of detectable beta-hCG 6 months after mole evacuation
* An elevation in beta-hCG after a normal value
:* Metastatic disease
* Postevacuation hemorrhage not caused by retained tissues
:* 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.


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.
 
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==   
==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.
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* 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:
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
* Age older than 35 years or age younger than 20 years
* Pre-evacuation serum beta-hCG greater than 100,000 IU/L
* Pre-evacuation serum beta-hCG greater than 100,000 IU/L
* Large-for-date uterus
* Large-for-date [[uterus]]
* Large uterine molar mass
* Large uterine molar mass
* Large (>6 cm) ovarian cysts
* Large (>6 cm) [[ovarian]] cysts
* Pre-eclampsia
* [[Pre-eclampsia]]
* Hyperthyroidism
* [[Hyperthyroidism]]
* Hyperemesis of pregnancy
* Hyperemesis of [[pregnancy]]
* Trophoblastic embolization
* Trophoblastic embolization
* Disseminated intravascular coagulation
* [[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==

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

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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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