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This document provides information on gestational trophoblastic disease (GTD), a rare type of disease characterized by abnormal proliferation of trophoblast cells. GTD includes hydatidiform mole (HM), invasive mole, choriocarcinoma, and other rare tumors. HM is usually benign but can develop into malignant tumors if not treated. Complete HM lacks fetal/embryonic tissues and has a paternal chromosomal pattern, while partial HM contains some fetal tissues and has a triploid pattern. Diagnosis involves detecting elevated hCG levels and examining symptoms like abnormal bleeding and enlarged uterus. Precise classification and monitoring of hCG titers is important for determining appropriate treatment, as even widespread GTD can often be cured with chemotherapy
This document provides information on gestational trophoblastic disease (GTD), a rare type of disease characterized by abnormal proliferation of trophoblast cells. GTD includes hydatidiform mole (HM), invasive mole, choriocarcinoma, and other rare tumors. HM is usually benign but can develop into malignant tumors if not treated. Complete HM lacks fetal/embryonic tissues and has a paternal chromosomal pattern, while partial HM contains some fetal tissues and has a triploid pattern. Diagnosis involves detecting elevated hCG levels and examining symptoms like abnormal bleeding and enlarged uterus. Precise classification and monitoring of hCG titers is important for determining appropriate treatment, as even widespread GTD can often be cured with chemotherapy
This document provides information on gestational trophoblastic disease (GTD), a rare type of disease characterized by abnormal proliferation of trophoblast cells. GTD includes hydatidiform mole (HM), invasive mole, choriocarcinoma, and other rare tumors. HM is usually benign but can develop into malignant tumors if not treated. Complete HM lacks fetal/embryonic tissues and has a paternal chromosomal pattern, while partial HM contains some fetal tissues and has a triploid pattern. Diagnosis involves detecting elevated hCG levels and examining symptoms like abnormal bleeding and enlarged uterus. Precise classification and monitoring of hCG titers is important for determining appropriate treatment, as even widespread GTD can often be cured with chemotherapy
ntroduction ntroduction What is GTD ? What is GTD ? t is a rare kind of disease in which t is a rare kind of disease in which abnormal trophoblastic proliferation abnormal trophoblastic proliferation occurs. occurs. t is too among the rare human t is too among the rare human malignancies that can be cured even malignancies that can be cured even in the presence of widespread in the presence of widespread metastases. metastases. Which does it include? Which does it include? t includes a spectrum of interrelated t includes a spectrum of interrelated tumors, including tumors, including hydatidiform mole (HM) hydatidiform mole (HM) invasive mole (M) invasive mole (M) Choriocarcinoma (CH) Choriocarcinoma (CH) Placental Placental- -site trophoblastic tumor site trophoblastic tumor (PSTT, borderline, very rare) (PSTT, borderline, very rare) Relationship of HM. M. CH Relationship of HM. M. CH hydatidiform therapeutic or hydatidiform therapeutic or mole mole spontaneous abortion spontaneous abortion term pregnancy term pregnancy ectopic ectopic invasion mole choriocarcinoma. invasion mole choriocarcinoma. What is GTT (Gestational trophoblastic What is GTT (Gestational trophoblastic tumor)? tumor)? GTT is all GTD GTT is all GTD except hydatidiform except hydatidiform mole. mole. They has its unique pathologic They has its unique pathologic characteristics and biological characteristics and biological behavior. behavior. Even the most malignant case can be Even the most malignant case can be cured by chemotherapy. cured by chemotherapy. Hydatidiform mole Hydatidiform mole Hydatidiform mole Hydatidiform mole Hydatidiform mole Hydatidiform mole t is a neoplastic t is a neoplastic proliferation of proliferation of the trophoblast the trophoblast in which the in which the terminal villi terminal villi are are transformed transformed into vesicles into vesicles filled with clear filled with clear viscid material. viscid material. t is usually benign but has t is usually benign but has malignant potentiality. malignant potentiality. ncidence: ncidence: south east Asia south east Asia is is 11/ /500 500- -600 600 the US and Europe: the US and Europe:11/ /500 500- -2000 2000 China: China:11/ /1238 1238 Classification Classification t is divided into two classification t is divided into two classification complete hydatidiform mole complete hydatidiform mole partial hydatidiform mole partial hydatidiform mole complete hydatidiform mole(CHM): complete hydatidiform mole(CHM): the entire the entire uterus uterus filled with filled with abnormal abnormal vesicles, vesicles, no signs of no signs of fetus. fetus. partial hydatidiform mole partial hydatidiform mole partial partial hydatidiform hydatidiform mole with mole with evidence of evidence of a conceptus. a conceptus. tiology tiology Though it is not known a number of Though it is not known a number of associated factors have been noted: associated factors have been noted: the absence of fetal circulation; the absence of fetal circulation; dietary protein deficiency dietary protein deficiency viral infection; viral infection; age:> age:>45 45 years women are years women are 10 10 times times more likely to develop HM than more likely to develop HM than those younger those younger abnormal fertilization process: abnormal fertilization process: the fertilization of a normal ovum the fertilization of a normal ovum with a duplicated haploid with a duplicated haploid sperm: sperm:46 46XX XX the fertilization of an empty egg by the fertilization of an empty egg by two sperms(dispermy): two sperms(dispermy):46 46XY XY Chromosomes Chromosomes complete hydatidiform moles complete hydatidiform moles Cytogenetic studies have demonstrated Cytogenetic studies have demonstrated that complete hydatidiform moles usually that complete hydatidiform moles usually have a have a 46 46xx karyotype, and the molar xx karyotype, and the molar chromosomes are entirely of paternal chromosomes are entirely of paternal origin. origin. Complete moles appear to arise from an Complete moles appear to arise from an ovum that has been fertilized by a haploid ovum that has been fertilized by a haploid sperm, which then duplicates its own sperm, which then duplicates its own chromosomes, and the ovum nucleus may chromosomes, and the ovum nucleus may be either absent or inactivated be either absent or inactivated Although most complete moles have Although most complete moles have a a 46 46xx chromosomal pattern, xx chromosomal pattern, approximately approximately 10 10% % have a have a 46 46xy xy karyotype. karyotype. Chromosomes in a Chromosomes in a 46 46xy complete xy complete mole also appear to be entirely of mole also appear to be entirely of paternal origin, but in this paternal origin, but in this circumstance, an apparently empty circumstance, an apparently empty egg is fertilized by two sperm. egg is fertilized by two sperm. .. partial hydatidiform mole partial hydatidiform mole partial moles usually have a triploid partial moles usually have a triploid karyotype ( karyotype (69 69 chromosomes ), with the chromosomes ), with the extra haploid set of chromosomes derived extra haploid set of chromosomes derived from the father. from the father. When a fetus is present in conjunction When a fetus is present in conjunction with a partial mole, it usually exhibits the with a partial mole, it usually exhibits the stigmata of triploidy, including growth stigmata of triploidy, including growth retardation and multiple congenital retardation and multiple congenital malformations. malformations. !athologic findings !athologic findings compIete hydatidiform moIe compIete hydatidiform moIe pathology pathology Complete moles lack identifiable Complete moles lack identifiable embryonic or fetal tissues, and embryonic or fetal tissues, and the chorionic villi exhibit the chorionic villi exhibit generalized hydatidiform swelling generalized hydatidiform swelling and diffuse trophoblastic and diffuse trophoblastic hyperplasia. hyperplasia. Gross Gross we see a mass of we see a mass of vesicles, vary in vesicles, vary in size, grape size, grape- -like like with stems, blood with stems, blood and clot filling the and clot filling the inter inter- -vesicle space vesicle space partial hydatidiform mole partial hydatidiform mole t are characterized by the following t are characterized by the following pathologic features : pathologic features : Chorionic villi if varying size with Chorionic villi if varying size with focal hydatidiform swelling and focal hydatidiform swelling and cavitation. cavitation. t contain identifiable embryonic or t contain identifiable embryonic or fetal tissues. fetal tissues. Gross Gross we see a we see a mass of mass of vesicles, vesicles, vary in size, vary in size, grape grape- -like like and and identifiable identifiable embryonic embryonic or fetal or fetal tissues. tissues. icroscopic icroscopic trophoblastic proliferation. trophoblastic proliferation. hydropic degeneration of hydropic degeneration of the stroma. the stroma. absence of blood vessels or absence of blood vessels or extreme scantiness of blood extreme scantiness of blood vessels. vessels.
trophoblastic proliferation is trophoblastic proliferation is
considered the considered the most important single most important single criteria. criteria. Ovaries respond to hCG Ovaries respond to hCG stimulation , stimulation ,30 30- -50 50% % theca theca- -lutein lutein cysts develop, bilateral cysts develop, bilateral Clinical course Clinical course t has t has eight eight of of symptoms and symptoms and physical signs. physical signs. amenorrhea amenorrhea because it is a pregnancy. because it is a pregnancy. vaginal bleeding vaginal bleeding after a period of amenorrhea after a period of amenorrhea (average (average 12 12 weeks) may continue weeks) may continue intermittently for several weeks intermittently for several weeks--- --- profuse bleeding profuse bleeding--- ---anemia and anemia and infection. infection. abdominal cramps abdominal cramps abnormally abnormally enlarged and enlarged and soft uterus soft uterus in about half the in about half the cases, the cases, the uterus growth is uterus growth is rapid, it is larger rapid, it is larger than the dates than the dates suggest. suggest. ovarian cyst ovarian cyst torsion torsion when we do pelvic when we do pelvic examination examination adnexal masses adnexal masses may be found. it is may be found. it is theca lutein cyst in theca lutein cyst in about one third of about one third of the cases the cases severe and early severe and early onset PH onset PH ( (Pregnancy nduced Hypertension Pregnancy nduced Hypertension syndrome) syndrome) hyperthyroidism hyperthyroidism plasma thyroxin concentration plasma thyroxin concentration elevates elevates exaggerated early pregnancy exaggerated early pregnancy symptoms symptoms nausea, nausea, vomit etc vomit etc Diagnosis Diagnosis suspicion: suspicion: abnormal bleeding after amenorrhea abnormal bleeding after amenorrhea inappropriately enlarged uterus; inappropriately enlarged uterus; absence of fetal heart sounds or absence of fetal heart sounds or could not feel fetal parts by palpation could not feel fetal parts by palpation between between 16 16- -20 20 th th week week hyperemesis gravidarum hyperemesis gravidarum bilateral ovarian cysts bilateral ovarian cysts serum hCG monitor serum hCG monitor an unusually high titer of chorionic an unusually high titer of chorionic gonadotropin, especially after the gonadotropin, especially after the one one- -hundredth day of pregnancy, hundredth day of pregnancy, help to confirm the diagnosis of HM. help to confirm the diagnosis of HM. Ultrasonography: Ultrasonography: t is a reliable and sensitive technique t is a reliable and sensitive technique for the diagnosis of complete molar for the diagnosis of complete molar pregnancy. Because the chorionic villi pregnancy. Because the chorionic villi exhibit diffuse hydatidiform swelling. exhibit diffuse hydatidiform swelling. Complete moles produce a Complete moles produce a characteristic vesicular sonographic characteristic vesicular sonographic pattern, usually referred to as a pattern, usually referred to as a snowstorm snowstorm pattern. pattern. Ultrasonography may also Ultrasonography may also contribute to the diagnosis of contribute to the diagnosis of partial molar pregnancy by partial molar pregnancy by demonstrating focal cystic demonstrating focal cystic spaces in the placental tissues spaces in the placental tissues and an increase in the transverse and an increase in the transverse diameter of the gestational sac. diameter of the gestational sac. ifferential diagnosis ifferential diagnosis abortion; abortion; multiple pregnancy; multiple pregnancy; polyhydramnios polyhydramnios %reatment %reatment the uterus should be evacuated as the uterus should be evacuated as soon as possible after the diagnosis soon as possible after the diagnosis is made.(by suction curettage) is made.(by suction curettage) suction; suction; oxytocin administration:we can use oxytocin administration:we can use blood transfusion or/and fluid blood transfusion or/and fluid infusion.it is used to decrease the infusion.it is used to decrease the size of the uterus; size of the uterus; tissue sent for histology: tissue sent for histology: it should be routine it should be routine practice with all cases of practice with all cases of incomplete miscarriage; incomplete miscarriage; acute pulmonary acute pulmonary complications complications total abdominal total abdominal hysterectomy hysterectomy in older in older multiparas multiparas hysterectomy may hysterectomy may be indicated. be indicated. management of theca management of theca- -lutein lutein cysts cysts these tumors should not be these tumors should not be excised because they excised because they regress after the regress after the trophoblastic tissue has trophoblastic tissue has been removed. been removed. chemotherapy chemotherapy HM don HM don t need usually t need usually chemotherapy because chemotherapy because HM is benign disease. HM is benign disease. ollow ollow- -up examinations up examinations follow up mode in the follow up mode in the 2 2 years after discharge years after discharge on each follow on each follow- -up up check, check, the following the following should be addressed should be addressed symptom symptom abnormal abnormal vaginal bleeding, vaginal bleeding, cough, cough, hemoptysis hemoptysis signs of metastasis signs of metastasis pelvic examination pelvic examination hCG evaluation hCG evaluation BB- -ultrasound ultrasound chest X chest X- -ray film ray film contraceptive method contraceptive method required for required for 11- -2 2 years years condom is recommended. condom is recommended. UD (intrauterine device)and pills UD (intrauterine device)and pills are relatively contraindicated for are relatively contraindicated for their potentiality of causing their potentiality of causing abnormal vaginal bleeding. abnormal vaginal bleeding. sk question sk question 1. 1. What is the etiology of GTD? What is the etiology of GTD? 2. 2. What is the classification of HM? What is the classification of HM? 3. 3. What is What is the main pathologic the main pathologic changes of HM? changes of HM? 4. 4. What is the clinical course of What is the clinical course of HM? HM? 5. 5. How Follow How Follow- -up examinations up examinations will be done? will be done? About About 80 80% % of the cases of HM of the cases of HM have a benign course. one have a benign course. one- -half half of patients become pregnant of patients become pregnant subsequently. about subsequently. about 16 16% % of HM of HM become invasion moles and become invasion moles and some some 22..55% progress into % progress into choriocarcinoma choriocarcinoma nvasive Mole nvasive Mole ntroduction ntroduction nvasive Mole nvasive Mole arises from HM arises from HM it has malignant potentialities, it has malignant potentialities, invades the invades the myometrium myometrium and and penetrates the uterine wall, penetrates the uterine wall, extends into the broad ligament extends into the broad ligament or peritoneal cavity. or peritoneal cavity. in half or more of all cases in half or more of all cases invasive mole metastasizes invasive mole metastasizes through the peripheral through the peripheral circulation to distant sites, circulation to distant sites, mostly to the lung. mostly to the lung. !athologic findings !athologic findings excessive trophoblastic excessive trophoblastic proliferation and proliferation and invasiveness invasiveness the degree of anaplasia is the degree of anaplasia is variable: completely benign variable: completely benign- - -- --highly malignant highly malignant differentiation between invasive differentiation between invasive mole and choriocarcinoma lies in mole and choriocarcinoma lies in whether the villous pattern is whether the villous pattern is preserved: preserved: if we see villi, it must be if we see villi, it must be invasion mole; invasion mole; if we can if we can t see villi, it is t see villi, it is choriocarcinoma. choriocarcinoma. Clinical course Clinical course Symptoms caused by primary lesions Symptoms caused by primary lesions vaginal bleeding vaginal bleeding pelvic examination reveals delayed pelvic examination reveals delayed involution of the uterus, persisting involution of the uterus, persisting cyst . cyst . abdominal pain abdominal pain intra intra--abdominal hemorrhage, abdominal hemorrhage, penetration of the uterus . penetration of the uterus . Metastatic symptoms Metastatic symptoms - -cough, cough, hemoptysis hemoptysis--- ---positive X positive X- -ray ray signs signs - -profuse vaginal bleeding profuse vaginal bleeding--- ---vaginal vaginal or cervical metastasis, we can see or cervical metastasis, we can see bluish nodule bluish nodule in vagina in vagina - -headache, nausea, headache, nausea, vomit, paralysis vomit, paralysis or coma or comait is caused by cerebral it is caused by cerebral lesion. lesion. iagnosis iagnosis history and clinical manifestation history and clinical manifestation hCG assay: hCG assay: diagnosis suspected if hCG titers diagnosis suspected if hCG titers persist to be high persist to be high 12 12 weeks after weeks after evacuation of a HM, or once evacuation of a HM, or once regress to normal range but rise regress to normal range but rise rapidly. rapidly. possible reasons : retained HM possible reasons : retained HM pregnancy pregnancy huge theca huge theca- -lutein lutein cyst persist cyst persist when we remove these reasons when we remove these reasons we can diagnose invasive mole we can diagnose invasive mole other measurement other measurement BB- -ultrasound ultrasound X X- -ray ray !rophylaxis !rophylaxis respond well to chemotherapeutic respond well to chemotherapeutic agents agents main causes of death: main causes of death: hemorrhage, hemorrhage, metastasis and metastasis and infection infection %reatment: %reatment: dentical to that for dentical to that for choriocarcinoma choriocarcinoma Choriocarcinoma Choriocarcinoma t is highly malignant GTT t is highly malignant GTT t may follow HM, t may follow HM, invasion mole, abortion, invasion mole, abortion, normal pregnancy, ectopic normal pregnancy, ectopic pregnancy. pregnancy. !athologic findings !athologic findings ross inspection ross inspection irregular or circumscribed irregular or circumscribed hemorrhagic growth hemorrhagic growth in the uterine in the uterine wall wall ulcerating surface opens into the ulcerating surface opens into the endometrial cavity (rarely endometrial cavity (rarely embedded in myometrium) embedded in myometrium) penetration into broad ligament or penetration into broad ligament or the peritoneal cavity the peritoneal cavity dark red blood:.it is filled dark red blood:.it is filled metastatic nodules metastatic nodules ulcerating ulcerating surface opens surface opens into the into the endometrial endometrial cavity (rarely cavity (rarely embedded in embedded in myometrium) myometrium) istologic findings istologic findings we see masses of anaplastic we see masses of anaplastic trophblastic cells in microscopy; trophblastic cells in microscopy; invasion into the uterine wall, invasion into the uterine wall, destroying vessels, muscle tissue destroying vessels, muscle tissue prominent necrosis and prominent necrosis and hemorrhage hemorrhage villi can not be recognized villi can not be recognized spread through circulation spread through circulation Clinical Manifestations Clinical Manifestations irregular bleeding after irregular bleeding after preceding gestation; preceding gestation; malignant tumor cells enter the malignant tumor cells enter the circulation through the open circulation through the open blood vessels and are blood vessels and are transported to lungs, brain or to transported to lungs, brain or to other distant sites. other distant sites. metastatic symptoms metastatic symptoms pulmonary lesions pulmonary lesions cerebral lesions cerebral lesions metastatic nodule in the vagina, metastatic nodule in the vagina, vulva or cervix ,it is vulva or cervix ,it is bluish bluish nodule nodule filled dark red blood. filled dark red blood. iagnosis iagnosis Diagnosis must be suspected as Diagnosis must be suspected as a possible reason for continued a possible reason for continued (irregular) bleeding after any (irregular) bleeding after any form of pregnancy. form of pregnancy. we assay hCG , the time of hCG we assay hCG , the time of hCG change into normal is different in change into normal is different in various diseases. various diseases. hCG change hCG change HM: HM:84 84- -100 100 days days Artificial abortion: Artificial abortion:30 30 days days Spontaneous abortion: Spontaneous abortion:19 19 days days Normal delivery: Normal delivery:12 12 days days Ectopic pregnancy: Ectopic pregnancy:88- -9 9 days days $taging $taging nternational staging of WHO may be nternational staging of WHO may be summarized as follows: summarized as follows: : : lesion localized in uterus, no lesion localized in uterus, no metastasis; metastasis; : : lesion extends beyond uterus, but lesion extends beyond uterus, but still confined to internal genitalias; still confined to internal genitalias; : : pulmonary lesion pulmonary lesion : : metastasis to other distant sites. metastasis to other distant sites. %reatment %reatment highly sensitive to chemotherapy, highly sensitive to chemotherapy, which is invariably the treatment which is invariably the treatment choice. choice. surgery has little place (because of surgery has little place (because of the high vascularity and the the high vascularity and the effectiveness of chemotherapy). it is effectiveness of chemotherapy). it is indicated for tumor resistant to indicated for tumor resistant to chemotherapy and single metastases chemotherapy and single metastases persisting despite chemotherapy. persisting despite chemotherapy. Chemotherapy Chemotherapy most often used drugs most often used drugs methotrexate (MTX) methotrexate (MTX) actinomycin D (Act actinomycin D (Act- -D) D) 55- -fluorouracil ( fluorouracil (55- -Fu) Fu) vincristine (VCR) vincristine (VCR) cyclophosphamide (CTX) cyclophosphamide (CTX) chlo chlo- -ranbucil, ranbucil, etc etc principles principles low low- -risk patients are usually treated with a risk patients are usually treated with a single agent single agent medium medium- -risk patients are usually treated risk patients are usually treated with EMA with EMA- -CO regimen with CO regimen with 80 80- -90 90% % survival rate. (Etoposide, survival rate. (Etoposide, Methotrexate,Actinomycin,Cyclophospham Methotrexate,Actinomycin,Cyclophospham ide,Vincristin) ide,Vincristin) toxic reaction: toxic reaction: marrow depression ; marrow depression ; gastrointestinal ulceration; gastrointestinal ulceration; change in liver and renal function change in liver and renal function $tandard for discharge $tandard for discharge three consecutive weekly assays three consecutive weekly assays for hC are negative for hC are negative two more courses for two more courses for consolidation consolidation all symptoms and signs all symptoms and signs disappear disappear peration peration unresponsive or drug fails to unresponsive or drug fails to reach the tumor; reach the tumor; if the tumor can be eradicated if the tumor can be eradicated by drug therapy, esp.in young by drug therapy, esp.in young women, there is no reason to women, there is no reason to remove the uterus; remove the uterus; the ovaries need not be the ovaries need not be removed removed. . ollow ollow- -up examinations up examinations at at 11- -month interval for month interval for 1 1 year: year: at at 33- -month interval for month interval for 2 2 years years at at 11- -year interval for year interval for 3 3 years years at at 22- -year interval afterwards. year interval afterwards. pelvic examination pelvic examination chest X chest X- -ray film ray film hCG hCG sk question : sk question : What are the basic What are the basic histologic and pathologic histologic and pathologic differences between differences between invasive mole and invasive mole and choriocarcinoma? choriocarcinoma?