婦產(chǎn)科學課件：妊娠滋養(yǎng)細胞疾病(英文版)

1、Gestational Trophoblastic Diseases (GTDs)22022/8/1234Introduction GTDs include a disease spectrum of hydatidiform mole, invasive mole, choriocarcinoma, and placental-site trophoblastic tumor.They arise from fetal tissue within the maternal host.The first and only disseminated solid tumors highly cur

2、able by chemotherapyUnique and characteristic tumor marker: hCGClassification invasive mole choriocarcinoma placental site trophoblastic tumor (PSTT)GTDsHydatidiform mole (HM)(complete and partial)Gestational Throphoblastic neoplasia (GTN)7Hydatidiform moleMolar pregnancyHydatidiform mole is an abno

3、rmal pregnancy characterized grossly by multiple graplike vesicles filling and distending the uterus, usually in the absence of an intact fetus.Incidence and epidemiologyIts incidence varies worldwide from 1 in 125 deliveries in Mexico and Taiwan to 1 in 1500 deliveries in the US.High risk factorswo

4、men 35-40 yearsLow economic statusNutrition: diets deficient in protein, folic acid, and caroteneEtiologyComplete moles are always euploid, paternal in origin, and absence or inactivation of the ovum nucleus.Partial moles are triploid 細胞遺傳學研究表明: 因染色體變異、卵子不正常，致受 精缺陷， 空卵受精，雙精子受精 2022/8/12142022/8/1215

5、2022/8/1216Pathology Completegrape-like massesPartialgrape-like masses with embroy or fetus2022/8/1218Complete hydatidiform mole demonstrating enlarged villi of various size2022/8/12192022/8/1220Hydatidiform mole: specimen from suction curettage2022/8/1221A large amount of villi in the uterus.2022/8

6、/12222022/8/1223 Here is a partial mole in a case of triploidy. Note the scattered grape-like masses with intervening normal-appearing placental tissue. Pathology microscopically characteredema of the villous stromaAvascular villi Nests of proliferating trophoblasts2022/8/1225The microscopic appeara

7、nce of hydatidiform mole:Hyperplasia of trophobasitc cellsHydropic swelling of all villiVessles are usually absent2022/8/1226Comparison of complete and partial HMComplete IncompleteKaryotype Diploid 46XX(90%)Triploid 69XXY(90%)EmbryoAbsent PresentVillihydropicFew hydropicTrophoblastsDiffuse hyperpla

8、siaMild focal hyperplasiaImplantation-site trophoblastDiffuse atypiaFocal atypia-hCGHigh (50 000)Slight elevation Frequency of classic clinical symptomscommonrareRisk for persistent GTN20%-30%100 000U/LPatient with extremely large uterus or poor follow-upTheca lutein ovarian cyst 6cmwomen 35-40 year

9、sMTX, 5-Fu, Dactinomycin, single agent chemotherapymonitor toxicityC. SurgeryAge40 years, high risk, no desire of pregnancy -hysterectomyTheca lutein cysts: ovaries should remain intact, because regression spontaneously. or aspirate under ultrasound, if torsion for a long time, necrosis, removeFollo

10、w-upThe incidence of malignant disease is 20% -30%.Time Weekly intervals after evacuation until serum hCG declines to nondetectable levels on 3 successive assaysThen monthly for 6 months and once half a year for a total 2 yearsContents Symptom: abnormal vaginal bleeding, cough, hemoptysisPelvic exam

11、inationSerial -hCG determinationUltrasound Chest X-ray or brain CTContinue contraception during surveillance for 1 year, condom or oral contraceptive, not IUDGestational Trophoblastic tumorOne of the rare malignancies that are highly curable even with widespread metastasisMost commonly follow a mola

12、r pregnancy, but they may follow any types of pregnancy.Invasive moleInvasive mole is a hydatidiform mole that invades myometrium or adjacent structure.It may penetrate the myometrium and cause uterine rupture and hemoperitoneum.In 1015% of patients who have had primary molar pregnancy, often within

13、 6 months after molar evacuationChoriocarcinoma 2-5% of all cases of GTN.Antecedent gestation: molar pregnancy(60%), abortion(30%), term pregnancy and less commonly ectopic pregnancyEarly vascular invasion with widespread dissemination, cause hemorrhage and necrosis of tissues or organs. The most co

14、mmon site of metastasis is lung, and then vagina, pelvis, liver, brainThe time between antecedent gestation and choriocarcinoma is variable.2022/8/1255侵蝕性葡萄胎及絨毛膜癌來源及發(fā)生時間 1. Pathology Invasive moleIn gross (picture) Vesicles, locally invasiveMicroscopically, proliferative trophoblast, invasion of the

15、 myometrium with identifiable villous structureChoriocarcinomaGrossly red and granular, extensive necrosis and bleedingMicroscopically, no villi, but instead sheets of trophoblasts on a background of hemorrhage and necrosis.2022/8/1257Invasive hydatidiform mole infiltrating the myometrium2022/8/1258

16、 Invasive mole: the tissue invades into the myometrial layer. No obvious borderline, with obvious bleeding.2022/8/1259子宮肌層深部受水泡狀絨毛侵犯，滋養(yǎng)葉細胞輕度增生，并有異型。HE40 2022/8/1260Microscopic image of choriocarcinoma2022/8/1261Microscopic image of choriocarcinomaabsence of chorionic villi2. Clinical findingsNonmeta

17、static gestational trophoblastic tumor-invasive mole or choriocarcinoma after HM, confined to the uterusVaginal bleeding: irregularEnlargement or subinvolution of uterus Theca lutein cystsAbdominal pain: rarely, perforation of uterus cause pain and hemorrhage, torsion or rupture of cystsSymptoms of

18、pregnancy: pseudopregnancyMetastatic gestational trophoblastic tumor-choriocarcinoma (picture)Local hemorrhage: destroy blood vesselsLung metastasis: chest pain, cough, dyspnea, hemoptysis Vagina: anterior wall and fornix, violet blue nodular LiverBrain: headache, vomiting, dizziness, coma, death Ot

19、her organs: spleen, kidney, bladder and et al3. DiagnosisSymptoms and signsAfter molar evacuation, 1 year, choriocarcinoma; 9 weeks after molar evacuation; 4 weeks after abortion, delivery, ectopic pregnancy, titer is high, or decrease then increase Ultrasound High echoes in myometrium, no capsuleX-

20、rayPulmonary metastasisCT, MRI Brain, lung, liver and renal metastasisHistology: with villi-invasive mole without villi-choriocarcinoma 2022/8/1266A case of invasive mole: inside the uterine cavity the typical “snow storm” appearance can be detected, The location of blood flow suggest an invasive mo

21、le.2022/8/1267Doppler image of invasive mole. Doppler waveform analysis depicts low vascular resistance (RI= 0.35)2022/8/1268Doppler image of choriocarcinoma2022/8/1269Doppler image of choriocarcinoma4. Differential diagnosisInvasive mole and choriocarcinomaTime and nature of previous pregnancyHisto

22、logy: villi present or notterm pregnancyabortionectopic pregnancy1y after evacuationhydatidiformmoleDifferentiation from other diseasesHMInvasive molechoriocarcinomaPSTTReaction of placentaRetained placentaPrevious pregnancynoHMvariousvariousvariousAbortion, termLatent phaseno12m1ynonovillihavehaven

23、ononoHave, degenerationProliferative trophoblastSlightsevereSlightsevere clusterSever, clusterIntermediate trophoblastDispersed, no proliferationnoDepth of infiltrationdeciduamyometriummyomyoSuperficial myometriumdeciduanecrosis nohavehaveno nonometastasis nohavehavea little no nometastasis of liver

24、, brain noa little easilya little +or HCG + + or + or 5. Staging according to anatomy I stage: confined to the uterusII stage: extends outside of the uterus but is limited to the genital structuresIII stage: extends to the lung, with or without known genital tract involvement IV stage: all other met

25、astatic site2022/8/12742022/8/12752022/8/12762022/8/12776. Clinical staging and Prognositic scoring0124age4040-Antecedent pregnancyHMabortionterm-Interval (m)44 77 1313hCG(IU/ml) BT1,0001,000-10,00010,000-100,000 100,000Largest tumor-3 8Prior failure chemotherapy-One drugTwo or more drugs6 low risk,

26、 7 high risk7. Treatment Principle: chemotherapy is the first choice, sometimes accompanied with hysterectomy or radiotherapyMortality decreased dramatically, from 90% to 20-30%Chemotherapy Medicines 5-fluorouracil(5-Fu)Methotrexate (MTX), Leucovorin calciumDactinomycin Cyclophosphamide(CTX)Vincrist

27、ine (VCR) Cis-platinum (CDDP)Etoposide (VP-16)Single-agent chemotherapy: MTX or dactinomycin, low riskCombined chemotherapy: EMA-CO, high riskSide effectsBone marrow depressionDigestive system symptomsLesion of liver and renal functioncalvitiesJudgment of therapeutic effectDetermine -HCG every week after one course of treatment, decrease one logarithmIndication of drug withdrawTill symptom and sign disappear, primary and secondary focus disappear, determine HCG weekly, 3 weeks continuously normal, th