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18.ppt十八、滋养细胞疾病

2011-04-16 50页 ppt 5MB 21阅读

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18.ppt十八、滋养细胞疾病nullGestational Trophoblastic Disease (GTD)Gestational Trophoblastic Disease (GTD)nullTypes of GTDTypes of GTDBenign Hydatidiform mole/molar pregnancy (complete or incomplete) malignant Invasive mole Choriocarcinoma (chorioepithelioma) Placental site trophoblastic ...
18.ppt十八、滋养细胞疾病
nullGestational Trophoblastic Disease (GTD)Gestational Trophoblastic Disease (GTD)nullTypes of GTDTypes of GTDBenign Hydatidiform mole/molar pregnancy (complete or incomplete) malignant Invasive mole Choriocarcinoma (chorioepithelioma) Placental site trophoblastic tumorTypes of GTDTypes of GTDThe term Gestational Trophoblastic Tumors has been applied the latter three conditions Arise from the trophoblastic elements Retain the invasive tendencies of the normal placenta or metastasis Keep secretion of the human chorionic gonadotropin (hCG) nullPathologic and clinical classifications for gestational trophoblastic diseaseHydatidiform Mole (molar pregnancy)Hydatidiform Mole (molar pregnancy)Definition and Etiology Definition and Etiology Hydatidiform mole is a pregnancy characterized by vesicular swelling of placental villi and usually the absence of an intact fetus. The etiology of hydatidiform mole remains unclear, but it appears to be due to abnormal gametogenesis and fertilization Definition and Etiology Definition and Etiology In a ‘complete mole’ the mass of tissue is completely made up of abnormal cells There is no fetus and nothing can be found at the time of the first scan. Definition and Etiology Definition and Etiology In a ‘partial mole’, the mass may contain both these abnormal cells and often a fetus that has severe defects. In this case the fetus will be consumed ( destroyed) by the growing abnormal mass very quickly. (shrink) Incidence Incidence 1 out of 1500-2000 pregnancies in the U.S. and Europe 1 out of 500-600 (another report 1%) pregnancies in some Asian countries. Complete > incompleteIncidence Incidence Repeat hydatidiform moles occure in 0.5-2.6% of patients, and these patiens have a subsequent greater risk of developing invasive mole or choriocarcinoma There is an increased risk of molar pregnancy for women over the age 40 Incidence Incidence Approximately 10-17% of hydatidiform moles will result in invasive mole Approximately 2-3% of hydatidiform moles progress to choriocarcinoma ( most of them are curable) Not definitely benign disease , has a tight relationship with GTT Clinical risk factors for molar pregnancyClinical risk factors for molar pregnancyCytogenetics Cytogenetics Complete molar pregnancy Chromosomes are paternal , diploid 46,XX in 90% cases 46,XY in a small part Partial molar pregnancy Chromosomes are paternal and maternal, triploid. 69,XXY 80% 69,XXX or 69,XYY 10-20%Wrong life message , so can not develop normallyComparative Pathologic Features of Complete and Partial Hydatidiform MoleComparative Pathologic Features of Complete and Partial Hydatidiform MoleComplete hydatidiform mole demonstrating enlarged villi of various sizeComplete hydatidiform mole demonstrating enlarged villi of various sizenullHydatidiform mole: specimen from suction curettageA large amount of villi in the uterus.A large amount of villi in the uterus.nullThe microscopic appearance of hydatidiform mole: Hyperplasia of trophobasitc cells Hydropic swelling of all villi Vessles are usually absentnullA sonographic findings of a molar pregnancy. The characteristic “snowstorm” pattern is evident.nullTransvaginal sonogram demonstrating the “ snow storm” appearance. nullColor Dopplor facilitates visualization of the enlarged spiral arteriesclose proximity to the “ snow storm” appearanceColor Doppler image of a hydatidiform mole and surrounding vessels. The uterine artery is easily identified from its anatomical location.Color Doppler image of a hydatidiform mole and surrounding vessels. The uterine artery is easily identified from its anatomical location.nullnullDopplor waveform analysis demonstrates low vascular resistance(RI=0.29) in the spiral arteries, much lower than that obtained in normal early pregnancynullnullPartial hydartidiform molenullMicroscopic image of partial molar pregnancy. Here is a partial mole in a case of triploidy. Note the scattered grape-like masses with intervening normal-appearing placental tissue. Here is a partial mole in a case of triploidy. Note the scattered grape-like masses with intervening normal-appearing placental tissue. null Large bilateral theca lutein cysts resembling ovarian germ cell tumors. With resolution of the human chorionic gonadotropin(HCG) stimulation, they return to normal-appearing ovaries.Signs and Symptoms of Complete Hydatidiform MoleSigns and Symptoms of Complete Hydatidiform MoleVaginal bleeding Hyperemesis ( severe vomit) Size inconsistent with gestational age( with no fetal heart beating and fetal movement) Preeclampsia Theca lutein ovarian cystsSigns and Symptoms of Partial Hydatidiform MoleSigns and Symptoms of Partial Hydatidiform MoleVaginal bleeding Absence of fetal heart tones Uterine enlargement and preeclampsia is reported in only 3% of patients. Theca lutein cysts, hyperemesis is rare. Diagnosis of hydatidiform moleDiagnosis of hydatidiform moleQuantitative beta-HCG Ultrasound is the criterion standard for identifying both complete and partial molar pregnancies. The classic image is of a “snowstorm” patternDiagnosisDiagnosisThe most common symptom of a mole is vaginal bleeding during the first trimester however very often no signs of a problem appear and the mole can only be diagnosed by use of ultrasound scanning. (rutting check) Occasionally, a uterus that is too large for the stage of the pregnancy can be an indication. NOTE: Vaginal bleeding does not always indicate a problem! Differential diagnosis Differential diagnosis Abortion Multiple pregnancy Polyhydramnios Treatment Treatment Suction dilation and curettage :to remove benign hydatidiform moles When the diagnosis of hydatidiform mole is established, the molar pregnancy should be evacuated. An oxytocic agent should be infused intravenously after the start of evacuation and continued for several hours to enhance uterine contractilityTreatment Treatment Removal of the uterus (hysterectomy) : used rarely to treat hydatidiform moles if future pregnancy is no longer desired. Treatment Treatment Chemotherapy with a single-agent drug Prophylactic (for prevention) chemotherapy at the time of or immediately following molar evacuation may be considered for the high-risk patients( to prevent spread of disease ) High-risk postmolar trophoblastic tumorHigh-risk postmolar trophoblastic tumorPre-evacuation uterine size larger than expected for gestational duration Bilateral ovarian enlargement (> 9 cm theca lutein cysts) Age greater than 40 years Very high hCG levels(>100,000 m IU/ml) Medical complications of molar pregnancy such as toxemia, hyperthyrodism and trophoblastic embolization (villi come out of placenta ) repeat hydatidiform mole Follow-upFollow-upPatients with hudatidiform mole are curative over 80% by treatment of evacuation. The follow-up after evacuation is key necessary uterine involution, ovarian cyst regression and cessation of bleeding Follow-upFollow-upQuantitative serum hCG levels should be obtained every 1-2 weeks until negative for three consecutive determinations, Followed by every 3 months for 1 years. Contraception should be practiced during this follow-up periodInvasive moleInvasive moleDefinition Definition This term is applied to a molar pregnancy in which molar villi grow into the myometrium or its blood vessels, and may extend into the broad ligament and metastasize to the lungs, the vagina or the vulva. null Invasive mole: the tissue invades into the myometrial layer. No obvious borderline, with obvious bleeding.nullInvasive hydatidiform mole infiltrating the myometriumnullA case of invasive mole: inside the uterine cavity the typical “snow storm” appearance can be detected, The location of blood flow suggest an invasive mole.nullThe same patient owing to the myometrial invasion. Reduced vascular resistance is detected in the uterine artery.nullTransvaginal color Doppler scan of a patient with invasive mole Following uterine curettage, Persistent color signals within the myometeriunnullDoppler image of invasive molenullPower Doppler easily detects a vascular echogenic nodule within the myometrium, suggesting invasive molenullDoppler image of invasive mole. Doppler waveform analysis depicts low vascular resistance (RI= 0.35)Common Sites for Metastatic Gestational Trophoblastic TumorsCommon Sites for Metastatic Gestational Trophoblastic TumorsChoriocarcinoma Choriocarcinoma Definition Definition A malignant form of GTD which can develop from a hydatidiform mole or from placental trophoblast cells associated with a healthy fetus ,an abortion or an ectopic pregnancy. Definition Definition Characterized by abnormal trophoblastic hyperplasia and anaplasia , absence of chorionic villinullGross specimen of choriocarcinomanullMicroscopic image of choriocarcinomaabsence of chorionic villinullMicroscopic image of choriocarcinomanullDoppler image of choriocarcinomanullDoppler image of choriocarcinomaSymptoms and signs Symptoms and signs Bleeding Infection Abdominal swelling Vaginal mass Lung symptoms Symptoms from other metastasesWHO Prognostic Scoring SystemWHO Prognostic Scoring System0-4 low risk, 5-7 intermediate risk, >8 high risk for death FIGO Staging System for Gestational Trophoblastic TumorsFIGO Staging System for Gestational Trophoblastic TumorsFIGO Staging System for Gestational Trophoblastic TumorsFIGO Staging System for Gestational Trophoblastic TumorsSubstages assigned for each stage as follows: A: No risk factors present B: One risk factor C: Both risk factors Risk factors used to assign substages: 1. Pretherapy serum hCG > 100,000 mlU/ml 2. Duration of disease >6 months nullnullIIbIIanullIIIa<3cm or locate in half lung IIIb disease beyond IIIanullDiagnosis and evaluationDiagnosis and evaluationGestational trophoblastic tumor is diagnosed by rising hCG following evacuation of a molar pregnancy or any pregnancy event Once the diagnosis established the further examinations should be done to determine the extent of disease ( X-ray, CT, MRI)Treatment Treatment Nonmetastatic GTD Low-Risk Metastatic GTD High-Risk Metastatic GTDTreatment of Nonmetastatic GTD Treatment of Nonmetastatic GTD Hysterectomy is advisable as initial treatment in patients with nonmetastatic GTD who no longer wish to preserve fertility This choice can reduce the number of course and shorter duration of chemotherapy. Adjusted single-agent chemotherapy at the time of operation is indicated to eradicate any occult metastases and reduce tumor dissemination. Treatment of Nonmetastatic GTD Treatment of Nonmetastatic GTD Single-agent chemotherapy is the treatment of choice for patients wishing to preserve their fertility. Methotrexate(MTX) and Actinomycin-D are generally chemotherapy agents Treatment is continued until three consecutive normal hCG levels have been obtained and two courses have been given after the first normal hCG level. To prevent relapse or metastasisTreatment of Low-Risk Metastatic GTD Treatment of Low-Risk Metastatic GTD Single-agent chemotherapy with MTX or actinomycin-D is the treatment for patients in this category If resistance to sequential single-agent chemotherapy develops, combination chemotherapy would be taken Approximately 10-15% of patients treated with single-agent chemotherapy will require combination chemotherapy with or without surgery to achieve remissionTreatment of High-Risk Metastatic GTD Treatment of High-Risk Metastatic GTD Multiagent chemotherapy with or without adjuvant radiotherapy or surgery should be the initial treatment for patients with high-rist metastatic GTD EMA-CO regimen formula is good choice for high-rist metastatic GTD Adjusted surgeries such as removing foci of chemotherapy-resistant disease, controlling hemorrhage may be the one of treatment regimen EMA-CO Chemotherapy for poor Prognostic DiseaseEMA-CO Chemotherapy for poor Prognostic DiseasePrognosisPrognosisCure rates should approach 100% in nonmetastatic and low-risk metastatic GTD Intensive multimodality therapy has resulted in cure rates of 80-90% in patients with high-risk metastatic GTD Follow-up After Successful TreatmentFollow-up After Successful TreatmentQuantitative serum hCG levels should be obtained monthly for 6 months, every two months for remainder of the first year, every 3 months during the second year Contraception should be maintained for at least 1 year after the completion of chemotherapy. Condom is the choice.Placenta Site Trophoblastic Tumor (PSTT)Placenta Site Trophoblastic Tumor (PSTT)Definition Definition Placenta Site Trophoblastic Tumor is an extremely rare tumor that arised from the placental implantation site Tumor cells infiltrate the myometrium and grow between smooth-muscle cellsnullDignosis and treatment Dignosis and treatment Surum hCG levels are relatively low compared to those seen with choriocarcinoma. Several reports have noted a benign behavior of this disease. They are relatively chemotherapy-resistant, and deaths from metastasis have occurred. Surgery has been the mainstay of treatment
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