novak 34. gestational trophoblastic disease

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NOVAK 34. Gestational Trophoblastic Disease. 부산백병원 산부인과 R1 손영실. # Hydatidiform Mole (H-mole). INDEX. 1. Epidemiology 2. Complete Versus Partial Hydatidiform Mole 3. Clinical Features 4. Natural History 5. Diagnosis 6. Treatment 7. Follow-up. EPIDEMIOLOGY. ◎ Risk Factors - PowerPoint PPT Presentation

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NOVAK 34.

Gestational Trophoblastic Disease

부산백병원 산부인과부산백병원 산부인과

R1 R1 손영실손영실

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# Hydatidiform Mole (H-mole)

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INDEX

1. Epidemiology

2. Complete Versus Partial Hydatidiform Mole

3. Clinical Features

4. Natural History

5. Diagnosis

6. Treatment

7. Follow-up

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◎ ◎ Risk FactorsRisk Factors

- low nutritional and socioeconomic factors- low nutritional and socioeconomic factors

- low dietary intake of carotene- low dietary intake of carotene

- vitamin A deficiency- vitamin A deficiency

- maternal age older than 35 years- maternal age older than 35 years

- use of oral contraceptive- use of oral contraceptive

- history of irregular menstruation- history of irregular menstruation

EPIDEMIOLOGY

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COMPLETE VERSUS PARTIAL HYDATIDIFORM MOLE

Features Complete Mole Partial Mole

Fetal or embryonic tissue Absent Present

Hydatidiform swelling of chorionic villi Diffuse Focal

Trophoblastic hyperplasia Diffuse Focal

Scalloping of chorionic villi Absent Present

Trophoblastic stromal inclusions Absent Present

Karyotype 46,XX (90%); 46,XY

Triploid (90%)

(on the basis of gross morphology, histopathology, and karyotype)(on the basis of gross morphology, histopathology, and karyotype)

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1. Complete H-mole1. Complete H-mole

◎ ◎ PathologyPathology

- lack embryonic or fetal tissues- lack embryonic or fetal tissues

- chorionic villi → generalized hydatidiform swelling- chorionic villi → generalized hydatidiform swelling

& diffuse trophoblastic hyperplasia& diffuse trophoblastic hyperplasia

◎ ◎ ChromosomesChromosomes

- usually have a 46,XX- usually have a 46,XX

- molar chromosomes are entirely of paternal origin- molar chromosomes are entirely of paternal origin

- ovum nucleus may be either absent or inactivated- ovum nucleus may be either absent or inactivated

- 10% : 46,XY- 10% : 46,XY

COMPLETE VERSUS PARTIAL HYDATIDIFORM MOLE

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COMPLETE VERSUS PARTIAL HYDATIDIFORM MOLE

Emptyovum

EndoreduplicationEndoreduplication

Emptyovum

Emptyovum

23X

23X

23X

23X

23Y

23X

46XX

46XY

46YY

46XX

HeterozygoHeterozygousus

HomozygoHomozygousus

Non-viable Non-viable gametegamete

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2. Partial H-mole2. Partial H-mole ◎ ◎ PathologyPathology ① ① Chorionic villi of varying size with focal hydatidiformChorionic villi of varying size with focal hydatidiform swelling, cavitation, and trophoblastic hyperplasiaswelling, cavitation, and trophoblastic hyperplasia ② ② Marked villous scallopingMarked villous scalloping ③ ③ Prominent stromal trophoblastic inclusionsProminent stromal trophoblastic inclusions ④ ④ Identifiable embryonic or fetal tissuesIdentifiable embryonic or fetal tissues

◎ ◎ ChromosomeChromosome - generally have a triploid karyotype (69 chromosomes)- generally have a triploid karyotype (69 chromosomes) - extra haploid set of vhromosome usually is derived- extra haploid set of vhromosome usually is derived from the fatherfrom the father - 90 ~ 93% : triploid- 90 ~ 93% : triploid

COMPLETE VERSUS PARTIAL HYDATIDIFORM MOLE

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COMPLETE VERSUS PARTIAL HYDATIDIFORM MOLE

23X

23X

23Y

23Y

23X

69XXX

23Y

23X

23X

69XXX

23X

69XXX

69YYY

Non-viable Non-viable gametegamete

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1. Complete H-mole1. Complete H-mole

① ① Vaginal bleedingVaginal bleeding - most common symptom- most common symptom - 97% → 84%- 97% → 84% - molar tissue separate from decidua & disrupt maternal- molar tissue separate from decidua & disrupt maternal vessels → large volumes of retained blood may distendvessels → large volumes of retained blood may distend endometrial cavityendometrial cavity

② ② Excessive uterine sizeExcessive uterine size - relative to gestational age- relative to gestational age - one of classic signs of complete mole- one of classic signs of complete mole - expanded by both chorionic tissue & retained blood- expanded by both chorionic tissue & retained blood - generally associated with elevated levels of hCG- generally associated with elevated levels of hCG

CLINICAL FEATURES

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③ ③ PreeclampsiaPreeclampsia

- observed in 27% of patients with complete mole- observed in 27% of patients with complete mole

- associated with HBP, proteinuria, and hyperreflexia- associated with HBP, proteinuria, and hyperreflexia

- eclamptic convulsion rarely occur- eclamptic convulsion rarely occur

- preeclampsia develops almost in patients with- preeclampsia develops almost in patients with

excessive uterine size & markedly elevated hCGexcessive uterine size & markedly elevated hCG

④ ④ Hyperemesis gravidarumHyperemesis gravidarum

- occurred in 25% of women with complete mole- occurred in 25% of women with complete mole

- particularly with excessive uterine size & markedly- particularly with excessive uterine size & markedly

elevated hCGelevated hCG

CLINICAL FEATURES

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⑤ ⑤ HyperthyroidismHyperthyroidism

- 7% of women in complete mole- 7% of women in complete mole

- Sx : tachycardia, warm skin, and tremor- Sx : tachycardia, warm skin, and tremor

- Dx : serum free T- Dx : serum free T44, T, T33

- If suspected before surgery, β-adrenergic blocking- If suspected before surgery, β-adrenergic blocking

agent should be administeredagent should be administered

(to prevent many of the metabolic and cardiovascular(to prevent many of the metabolic and cardiovascular

complication of thyroid storm)complication of thyroid storm)

CLINICAL FEATURES

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⑥ ⑥ Trophoblastic embolizationTrophoblastic embolization - 2% of women in complete mole- 2% of women in complete mole - Sx : chest pain, dyspnea, tachypnea, tachycardia- Sx : chest pain, dyspnea, tachypnea, tachycardia & severe respiratory distress& severe respiratory distress (during and after molar evacuation)(during and after molar evacuation)

⑦ ⑦ Theca lutein ovarian cystsTheca lutein ovarian cysts - 50% of patients with complete mole- 50% of patients with complete mole - result from high hCG levels, cause ovarian- result from high hCG levels, cause ovarian hyperstimulationhyperstimulation - after molar evacuation, cysts normally regress- after molar evacuation, cysts normally regress spontaneously within 2 to 4 monthsspontaneously within 2 to 4 months

CLINICAL FEATURES

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2. Partial H-mole2. Partial H-mole

• • Do not have the dramatic clinical featureDo not have the dramatic clinical feature

• • In general, patients have the sign and symptoms ofIn general, patients have the sign and symptoms of

incomplete or missed abortionincomplete or missed abortion

• • partial mole can be diagnosed after histologic reviewpartial mole can be diagnosed after histologic review

of the tissue obtained by curettageof the tissue obtained by curettage

CLINICAL FEATURES

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1. Complete H-mole1. Complete H-mole - have a potential for local invasion(15%)- have a potential for local invasion(15%) and metastasis(4%)and metastasis(4%) (after molar evacuation)(after molar evacuation) - following signs- following signs ① ① hCG level hCG level > > 100,000 mIU/ml100,000 mIU/ml ② ② excessive uterine enlargementexcessive uterine enlargement ③ ③ theca lutein cysts 6cm in diametertheca lutein cysts 6cm in diameter - patients with any one of these signs → - patients with any one of these signs → high riskhigh risk

2. Partial H-mole2. Partial H-mole - 4% of patients : persistent tumor, usually nonmetastatic,- 4% of patients : persistent tumor, usually nonmetastatic, chemotherapy is required to achievechemotherapy is required to achieve remissionremission

NATURAL HISTORY

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- Ultrasonography is a reliable and sensitive technique- Ultrasonography is a reliable and sensitive technique

for diagnosisfor diagnosis

- Characteristic vesicular ultrasonographic pattern :- Characteristic vesicular ultrasonographic pattern :

snowstorm pattern (honey-comb appearance)snowstorm pattern (honey-comb appearance)

DIAGNOSIS

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1. Hysterectomy1. Hysterectomy

- if the patient desires surgical sterilization, hysterectomy- if the patient desires surgical sterilization, hysterectomy

may be performedmay be performed

- the ovaries may be preserved, even though prominent- the ovaries may be preserved, even though prominent

theca lutein cysts are presenttheca lutein cysts are present

- hysterectomy does not prevent metastasis,- hysterectomy does not prevent metastasis,

so, still required f/u hCG levelsso, still required f/u hCG levels

TREATMENT

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2. Suction Curettage2. Suction Curettage - preferred method of evacuation, for patients who desire to- preferred method of evacuation, for patients who desire to preserve fertilitypreserve fertility

- the following steps- the following steps ① ① oxytocin infusion : before induction of anesthesiaoxytocin infusion : before induction of anesthesia ② ② cervical dilatation : retained blood in endometrial cavitycervical dilatation : retained blood in endometrial cavity may be expelled during dilatationmay be expelled during dilatation ③ ③ suction curettage : uterus may decrease dramaticallysuction curettage : uterus may decrease dramatically in sizein size ④ ④ sharp curettage : performed to remove any residualsharp curettage : performed to remove any residual molar tissuemolar tissue

TREATMENT

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3. Prophylactic Chemotherapy3. Prophylactic Chemotherapy

prevented metastasisprevented metastasis

reduced the incidence and morbidity of local uterinereduced the incidence and morbidity of local uterine

invasioninvasion

- single course of actinomycin D at time of evacuation- single course of actinomycin D at time of evacuation

- useful in the management of high-risk complete mole- useful in the management of high-risk complete mole

TREATMENT

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1. Human Chorionic Gonadotropin (hCG)1. Human Chorionic Gonadotropin (hCG) - monitored with weekly of hCG levels until these levels are- monitored with weekly of hCG levels until these levels are

normal for 3 consecutive weeksnormal for 3 consecutive weeks

- followed by monthly until levels are normal- followed by monthly until levels are normal

for 6 consecutive monthsfor 6 consecutive months

2. Contraception2. Contraception

- patients should be used effective contraception- patients should be used effective contraception

during the entire interval of hCG f/uduring the entire interval of hCG f/u

- oral contraceptive may be used safely- oral contraceptive may be used safely

FOLLOW - UP

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