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1、妊娠滋養(yǎng)細(xì)胞疾病ContentsDefinition ClassificationEpidemiology and Risk FactorsGTDDiagnosisTreatment 2妊娠滋養(yǎng)細(xì)胞疾病DefinitionSpectum of interrelated but histologically distinct tumors Originating from the placentaCharacterized by tumor marker,-HCGTendencies toward local invasion and spread3妊娠滋養(yǎng)細(xì)胞疾病Classification
2、of GTDMolar lesions Hydatidiform mole 葡萄胎 Complete 完全性葡萄胎 Partial 部分性葡萄胎 Invasive mole 侵蝕性葡萄胎Nonmolar lesions Choriocarcinoma 絨毛膜癌 Plascental site trophoblastic tumor , PSTT 胎盤部位滋養(yǎng)細(xì)胞腫瘤 Epithelioid trophoblastic tumor 上皮樣滋養(yǎng)細(xì)胞腫瘤 4妊娠滋養(yǎng)細(xì)胞疾病Epidemiology and Risk Factors (1)Incidence1 to 2 per 1,000 deliv
3、erySome ethic groups and regions higherMaternal AgeUpper and lower extremes, higher riskHigher rates of abnormal fertilization5妊娠滋養(yǎng)細(xì)胞疾病Obstetric HistoryPrior unsuccessful pregnanciesPersonal history of GTDOther FactorsCombination oral contraceptive(COC) pill useVitamin A and carotene deficiencyHighe
4、r educational levels, smoking, irregular menstrual cycles Epidemiology and Risk Factors (2)6妊娠滋養(yǎng)細(xì)胞疾病Normal villiCytotrophoblast, CTSyncytitrophoblst, STIntermediate tropboblast, IThCG +7妊娠滋養(yǎng)細(xì)胞疾病The Karyotype of Molar Pregnancy多余的父源基因是造成滋養(yǎng)細(xì)胞增生的主要原因。46,XX69,XXY69,XYY8妊娠滋養(yǎng)細(xì)胞疾病Hydatidiform Mole(Molar Pr
5、egnancy) Complete hydatidiform molePartial hydatidiform mole9妊娠滋養(yǎng)細(xì)胞疾病Complete mole vs partial moleComplete molePartial mole10妊娠滋養(yǎng)細(xì)胞疾病Gross finding:Huge amount of grape-like structuresNo embryo componentsMicroscopic findings:Trophoblastic proliferationVillous edemaComplete hydatidiform mole11妊娠滋養(yǎng)細(xì)胞疾病
6、Gross finding:Focal molar, vary size Normal placenta tissueIdentifible fetal tissues Microscopic findings:Marked villous scallopingLess trophoblastic proliferationStromal tropboblastic inclusionPartial hydatidiform mole12妊娠滋養(yǎng)細(xì)胞疾病vaginal bleeding 停經(jīng)后陰道出血 (80%)excessive uterine size 子宮異常增大變軟 (30%)thec
7、a lutein ovarian cysts 卵巢黃素化囊腫 hyperemesis gravidarum 妊娠劇吐 (8%) Preeclampsia 子癇前期征象 (1:74)abdominal pain 腹痛Hyperthyroidism 甲亢征象hCG升高滋養(yǎng)細(xì)胞增生Molar PregnancyClinical Features13妊娠滋養(yǎng)細(xì)胞疾病Features of Complete and Partial Hydatidiform MolesFeatureComplete MolePartial MoleKaryotype46, XX or 46, XY69, XXX or 6
8、9, XXYPathology Fetus/embryoAbsentPresent Villous edemaDiffuseFocal Trophoblastic proliferationVariable, may be markedFocal and minimalClinical Features Typical DiagnosisMolar gestationMissed abortion Post-molar malignant15-20%2-4%14妊娠滋養(yǎng)細(xì)胞疾病DiagnosisClinical features excessive uterine enlargementthe
9、ca lutein cystelevated serum -hCG levels Sonography Complex, echogenic mass containing cystic spacesKaryotype analysis Pathology15妊娠滋養(yǎng)細(xì)胞疾病宮內(nèi)妊娠Intrauterine Pregnancy葡萄胎Molar PregnancyUltrasound Diagnosis16妊娠滋養(yǎng)細(xì)胞疾病1.子宮明顯大于相應(yīng)孕周 2.宮腔內(nèi)“落雪狀“或“蜂窩狀“回聲 3.子宮肌層內(nèi)無或稀疏血流 4.Partial mole:可見到胎兒部分Complete molePartial
10、 mole17妊娠滋養(yǎng)細(xì)胞疾病Follow up of Hydatidiform Mole -HCG measurementPhysical and Gynecologic examinationSonography, X-ray, CTContraception 1 year19妊娠滋養(yǎng)細(xì)胞疾病 2 4 6 8 10 12 14持續(xù)性GTDPersistent trophoblastic diseasePersistent trophoblastic disease人工流產(chǎn)Therapeutic abortion足月產(chǎn)Term pregnancyWeeks after evacuation1
11、0001001010.10.01葡萄胎自然轉(zhuǎn)歸Hydatidiform mole-spontaneous regressionHCG -Marker for GTD20妊娠滋養(yǎng)細(xì)胞疾病妊娠滋養(yǎng)細(xì)胞腫瘤Gestational Trophoblastic Neoplasia, GTDGestational Trophoblastic Tumor, GTT侵蝕性葡萄胎 Invasive Hydatidiform Mole絨毛膜癌 Choriocarcinoma胎盤部位滋養(yǎng)細(xì)胞腫瘤 Placental Site Trophoblastic Tumor, PSTT上皮樣滋養(yǎng)細(xì)胞腫瘤 Epithelioi
12、d Trophoblastic Tumor21妊娠滋養(yǎng)細(xì)胞疾病Molar villi infiltrated into myometrium;Atypical trophoblastic proliferation;Apparent molar vesiclesInvasive Mole Histology22妊娠滋養(yǎng)細(xì)胞疾病Diffuse hemorrhage and necrosisWithout chorionic villiLack of vaculazationAtypia ST, CT and ITChoriocarcinomaHistology23妊娠滋養(yǎng)細(xì)胞疾病Clinical
13、 FindingsAbnorminal virginal bleedingAbnorminal uterine sizeTheca-lutein cystAbdominal painPersistently elevated serum-hCG levelsMetastatic foci : lung(80%), virginal(30%), pelvis(20%), hepatic (10%), brain (10%). 24妊娠滋養(yǎng)細(xì)胞疾病Criteria for the Diagnosis of GTN1. Plateau of -hCG lasts for four measureme
14、nts over a period of 3 weeks of longer (days 1, 7, 14, and 21)2. Rise of -hCG of 3 weekly consecutive measurements or longer, over a period of 2 weeks or more (days 1, 7, and 14)3. -hCG remains elevated for 6 months or more4. Histologic diagnosis of choriocarcinoma25妊娠滋養(yǎng)細(xì)胞疾病FIGO StagingStageDisease
15、confined to the uterusStageGTN extends outside of the uterus but is limited to the genital structures (adnexa, vagina, broad ligament)StageGTN extends to the lung, with or without known genital tract involvementStageAll other metastatic sites (brain, liver)26妊娠滋養(yǎng)細(xì)胞疾病Modified WHO Prognostic Scoring S
16、ystem as Adapted by FIGOScores0124Age(yr)4040Antecedent pregnancyMoleAbortionTermInterval months from indexpregnancy44671213Pretreatment serum -hCG(mIU/mL)103103104104105105Largest tumor size (including uterus)38Previous failed chemotherapy drugs12Low risk = WHO score of 0 to 6; High risk = WHO scor
17、e of727妊娠滋養(yǎng)細(xì)胞疾病Treament of GTNChemotherapyLow risk GTT: MTX5FU/KSMHigh risk GTT: EMA/COEMA/EPTE/TPPVB/BEPSurgical management28妊娠滋養(yǎng)細(xì)胞疾病Surgical managementHysterectomyPSTTEpithelioid trophoblastic tumorEmergent indicationsSurgical resection of metastasesChemotherapy-resistantRepeat uterine curettageMa
18、nagement of post-molar GTN in Europe29妊娠滋養(yǎng)細(xì)胞疾病PSTTRare GTT;Pathologic characters;ITIHC: hCG(),hPL ()Insensitive to chemotherapySurgical treatmentHigh risk factors:MI5/HPInterval months from index pregnancy 2yrExtrauterine metastasis30妊娠滋養(yǎng)細(xì)胞疾病Follow up-HCG measurementPhysical and Gynecologic examinationSonography, X-ray, CTDuration 1st year: 1/3months;2nd-3rd year: 1/6months;Five years: 1/every 1 year;Later: 1/every 2 year.31妊娠滋養(yǎng)細(xì)胞疾病Summary-1葡萄胎是一種妊娠滋養(yǎng)細(xì)胞疾??;完全性葡萄胎多為二倍體,部分性葡萄胎多為三倍體;停經(jīng)后陰道流血為常見癥狀;診斷依靠病理,輔助超聲、血hCG等;處理以清宮為主;隨訪血hCG很重要。32妊娠滋養(yǎng)細(xì)胞疾病Summary-2侵蝕性葡萄胎絨毛膜癌
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