Gestational trophoblastic disease.doc

8/21/2019 Gestational trophoblastic disease.doc

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Gestationaltrophoblastic disease (GTD) encompasses a largegroup of neoplastic diseases arising fromtrophoblastic tissue. The most common diseases arethe following:

1 Hydatidiform mole-omplete and !ncomplete

mole" !n#asi#e mole (horioadenoma destruens)$ horiocarcinoma% &pithelioid trophoblastic tumor' lacental site trophoblastic tumor

!n addition there are some proliferations of

trophoblastic tissue which are benign but mayhistologically mimic disease.

*iscellaneous trophoblastic lesions:&+aggerated placental sitelacental site nodule or pla,ue

Patients with molar pregancy, the mostcommon form of GTD, may present withvaginal bleeding and excessive uterine

enlargement for gestational age, usuallybetween 11-2 wee!s of pregnancy" Pregnancy induced hypertension may occur in 1#$ of patients" %ther presenting signs includehyperthyroidism and hyperemesis gravidarum"&haracteristically, the serum '&G is elevated"


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(n ultrasound of the uterus reveals acharacteristic snowstorm pattern, representingthe hydropic chorionic villi which characteri)esthe disease" (fter evacuation of the uterus,

careful clinical followup with serialmeasurements of the serum '&G is necessaryto ensure there is no recurrence and all of thedisease has been removed"

The clinical presentations of the other forms of GTD are covered in the following discussionand tables"

OUTLINE

Epidemiology

Disease

Associations

Pathogenesis

Gross Appearanceand ClinicalVariants

Histopathologicaleat!res andVariants

La"oratory#$adiologic#OtherDiagnostic Testing

Di%erentialDiagnosis


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Prognosis andTreatment

Commonly Used

Terms

Internet Lin&s

EPIDE'IOLOG(

CHA$ACTE$I)ATION

INCIDENCE

Completemole

11 pregnancies*ost fre,uent form ofmolar pregnancy andGTD

Incompletemole

"'-/%0 of all molarpregnancies

Choriocarcinoma

1" pregnancies

The more abnormal thepregnancy the moreliely this will occur

112 normalgetstations11'$32 abortions1'$$$ ectopic

pregnancies1% molar pregnancies

Epithelioidtropho"lastic

t!mor4ery rare

Placental5arest form of GTD


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sitetropho"lastic

t!mor

AGE $ANGE*'EDIAN

6sually disorders ofreproducti#e age

!ncreased ris of molesat the e+tremes ofreproducti#e age

Completemole

11-"' wees ofpregnancy

Incompletemole7-$%th wees ofpregnancy

In+asi+emole

*ay occursimultaneously withintraca#itary molarpregnancy

GEOG$APH(*Hydatidi,orm

mole

5ate per 1

Indonesia7.7 pregnancies

Tai-an3.$ deli#eries

Philippines'. deli#eries

'e.ico%.2 pregnancies

Nigeria".2 deli#eries

/apan1.7 pregnancies

A!stralia1.% pregnancies

U0A1.1 pregnancies

Israel.3 li#e births

0-eeden.2 pregnancies


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Parag!ay." pregnancies

EPIDE'IOLOGIC

A00OCIATION0

Lo-socioeconomic conditions

Dietossible dietaryde8ciency of carotene

1lood gro!ps

A

horiocarcinoma morefre,uent

9tronger for women oftype and husbands oftype ; and con#erselyfor group ; women andtype husbands

1 or A1


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pregnancyand li+e"irths

!ncreases with anincreased number of li#ebirths

PATHOGENE0I0

CHA$ACTE$I)ATION

CO'PLETE'OLE

%2>> with both >chromosomes androgenicor paternal origin-5esultsfrom duplication of thehaploid sperm in anempty o#um

$-1$0 ha#e normal %2>?but both >?chromosomes arepaternal-5esults fromdispermy fertili@ation of

an empty o#um by twosperm

Hydatidiform moles arepregnanciescharacteri@ed byabnormal de#elopment of

both embryonic ande+traembryonic tissuesand are associated withthe mise+pression ofimprinted genes. The#ast maAority of complete


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hydatidiform moles arediploid and androgeneticwhereas partialhydatidiform moles aretriploid with an e+tra setof chromosomes ofpaternal origin.

INCO'PLETE'OLE

Triploidy (27chromosomes) withmaternal chromosomecomplement

*ost are 27>>? 27>>>and rarely 27>??5esults from fertili@ationof an egg with a haploidset of chromosomes by

either two sperms eachwith a haploid set ofchromosomes or by asingle sperm with adiploid genome of %2 >?(diandric-paternallyderi#ed triploidy)

PLACENTAL

0ITET$OPHO1LA0TIC TU'O$


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lacental sitetrophoblastic tumour(9TT) is a neoplasticproliferation of theimplantationintermediate trophoblast.lthoughclinicopathologicalstudies are notuncommon in casereports or small seriesmolecular and genetic

studies are ,uite limited.

LA1O$ATO$ (#$ADIOLOGIC#OTHE$

CHA$ACTE$I)ATION

I0H

Determination o, DNAploidy "y2!orescence in sit!hy"ridi3atio

!n the past " years thediagnosis of hydatidiformmoles has become morediBcult because of the


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n 4I0H5 inhydatidi,orm moles6E+al!ationo, I0H onisolatedn!clei7

widespread use of earlyuterine e#acuation.Di=erentiating hydropicdegeneration partial andcomplete moles isimportant because oftheir di=erent prognosis.Howe#er clinicaldiagnosis is less ob#iousand the pathologist has toseparate the di=erententities on the basis of

#ery subtle morphologiccriteria.

!n diBcult cases ploidymay be determined by#arious methodsincluding Cuorescence insitu hybridi@ation (!9H)

on routine histologicalsections from paraBn-embedded specimens.Howe#er !9H analysis isoften diBcult because ofthe presence of numeroustruncated nuclei. !n thisconte+t we ha#e tested

the ad#antages of !9H onisolated nuclei a well-nown #ariant of thetechni,ue that might bemore sensiti#e.


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G$O00APPEA$ANCE#CLINICAL

VA$IANT0

CHA$ACTE$I)ATION

Completemole

Hydropic chorionic #illiEarge for dates uterus

Incompletemole

;ften small for datesuterus resembling amissed abortion

4olume of tissue usually

F" ml

4illi are grossly enlargedbut smaller than seen in acomplete moleragments of normalplacenta and a fetus maybe found

In+asi+emole

5e,uires demonstrationof molar #illi in#ading intothe myometrium ordeported to e+trauterinesites

Choriocarcin 6sually presents with


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oma

abnormal uterinebleeding but symptoms of metastases may be the8rst symptom

Dar red hemorrhagicmass with a shaggyirregular surface

Epithelioidtropho"lastic t!mor

9imilar presentation aschoriocarcinoma andin#ades the myometriumin an e+pansile rather

than permeati#e growth

ot associated withcentral necrosis orhemorrhage

&le#ated serum HG

Placentalsitetropho"lastic t!mor

4ariable in si@e but may

present with di=usenodular enlargement ofthe myometrium-occasionally polypoidproAecting into the uterineca#ity

!n#asi#e tumor fre,uentlye+tends into the uterine

serosa and adne+alstructures

0ITE0

1$OADLIGA'ENT


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EpithelioidTropho"lastic n7

TE0TI0

Placentalsitetropho"lasti

c t!mor in alaterec!rrenceo, anonseminomato!sgerm cellt!mor o, thetestis7

lacental sitetrophoblastic tumor(9TT) is a well-de8nedentity in the femalegenital tract. !n the malegenital tract a singlecase of 9TT in the testisof a young boy has beenreported. Despite its #eryrare occurrence 9TT ofthe testis has beenincorporated in the latest


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circumferentialtrophoblastic proliferationaround the #illus

Incomplete

mole

*olar change is focal witha mi+ture of edematous#illi and small relati#elynormal si@ed #illi

entral cisterns are lessconspicuous

9maller #illi with stromal

8brosis

!n#aginations oftrophoblast into the#illous stroma gi#ing ascalloped appearanceappearing as inclusionswithin the stroma

In+asi+emole

*olar #illi withtrophoblast within themyometrium or at ane+trauterine stie

4illi are usually %-' mm indiameter but hydropicchanges are not asmared as in complete

moles

Choriocarcinoma

*asses and sheets oftrophoblastic cells without#illi in#ading thesurrounding tissue and


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permeating #ascularspaces

entral hemorrhage and

necrosis with a rim of#iable tumor

dmi+ture ofintermediate cyto- andsyncytio-trophoblasticcells with considerablenuclear atypia

Epithelioidtropho"lastic t!mor

EpithelioidTropho"lasti

c7A7

Placentalsitetropho"lastic t!mor

bundant e+tracellular

eosinophilic 8brinoid ispresent within the tumor

lood #essel walls aree+tensi#ely replaced bytrophoblastic cells and8brinoid material

&+tensi#e necrosis may

be present


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P$OGNO0I0ANDT$EAT'ENT

CHA$ACTE$I)ATION

Prognosticactors

Completemole

!n more than 20 ofpatients who re,uiredchemotherapy therewere large for dates uteriand o#arian enlargmentsecondary to theca-lutein

cysts

120 de#elop into in#asi#emoles and ".'0 de#elopinto choriocarcinoma

.2-1.'0 of patients areat ris of ha#ing recurrentmolar pregnancies

Incompletemole

'0 will ha#e persistent ormetastatic GTD re,uiringchemotherapy

horiocarcinoma is rare

In+asi+emole

horiocarcinoma occurs2-1+ more fre,uentlythan following a

hydatidiform mole

*etastases may occur in"-%0 of cases

Choriocarcinoma

*etastases especially tobrain and li#er can occur


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in "-20 of patients


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I1on8ned to uterus with 1ris factor

ICon8ned to uterus with "

ris factors

II

Tumor e+tending outsideuterus but limited togenital structures(adne+a #agina broadligament)

IIAo ris factors

II11 ris factor

IIC" ris factors

III Tumor e+tending to lungswith or without nowngenital tract in#ol#ement

IIIAo ris factors

III11 ris factor

IIIC" ris factors

IV ll other metastatic sites

IVAo ris factors

IV11 ris factor

IVC" ris factors

8 (ear0!r+i+al

!n diseases treated withsurgery and combinationchemotherapy:

Disease con8ned touterus-10*etastatic disease-3$0

'etastasis Eungs brain li#er


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Treatment

ombination surgery withchemotherapy*-methotre+ateactinomycin D andchlorambucil

.

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Gestational trophoblastic disease.doc