Trophoblastic diseaseTrophoblastic disease

-This is a group of disorders -This is a group of disorders characterized by characterized by

1-abnormal placental 1-abnormal placental development.development.

2- The chorionic villi are hydropic 2- The chorionic villi are hydropic with vacuolation of the placentawith vacuolation of the placenta

3- destruction of the normal 3- destruction of the normal stroma.stroma.

PathologyPathology

1-Benign trophoblastic disease:1-Benign trophoblastic disease: -is usually either a complete -is usually either a complete

hydatidiform mole, where there hydatidiform mole, where there is no evidence of an embryo,is no evidence of an embryo,

- or a partial hydatidiform mole - or a partial hydatidiform mole which may be associated with which may be associated with an embryo (usually abnormal) an embryo (usually abnormal)

Other types of trophoblastic Other types of trophoblastic disease include :disease include :

1-invasive moles1-invasive moles 2-, placental site reactions,2-, placental site reactions, 3- trophoblastic tumours and 3- trophoblastic tumours and

hydropic change.hydropic change.

Malignant trophoblastic Malignant trophoblastic disease (choriocarcinomadisease (choriocarcinoma):):

complicates approximately 3% complicates approximately 3% of complete moles although in of complete moles although in 50% of cases of 50% of cases of choriocarcinoma there is no choriocarcinoma there is no history of immediately preceding history of immediately preceding trophoblastic disease.trophoblastic disease.

It may also occur following It may also occur following normal pregnancy. Blood-borne normal pregnancy. Blood-borne metastases may occur locally in metastases may occur locally in the vagina but most commonly the vagina but most commonly appear in the lungsappear in the lungs

AetiologyAetiology

Trophoblastic disease is thought Trophoblastic disease is thought to arise by fertilization of the to arise by fertilization of the oocyte by a single diploid oocyte by a single diploid spermatozoon or by two haploid spermatozoon or by two haploid sperm. sperm.

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If this occurs in the absence of If this occurs in the absence of any female nuclear material the any female nuclear material the resulting conceptus pregnancy resulting conceptus pregnancy is a complete mole with a diploid is a complete mole with a diploid karyotype (46XX).karyotype (46XX).

-A partial mole is thought to -A partial mole is thought to occur where fertilization occurs occur where fertilization occurs with the maternal chromosome with the maternal chromosome and the conceptus is triploid and the conceptus is triploid (69XXX or 69XXY). (69XXX or 69XXY).

-Older women and those with a -Older women and those with a previous history of trophoblastic previous history of trophoblastic disease or an A type blood group disease or an A type blood group are at increased risk are at increased risk

PresentationPresentation

-Molar pregnancy most commonly -Molar pregnancy most commonly presents as bleeding in the first presents as bleeding in the first half of pregnancy,half of pregnancy,

- is usually diagnosed initially as a - is usually diagnosed initially as a threatened miscarriage.threatened miscarriage.

-Occasionally, the passage of a -Occasionally, the passage of a ‘grape-like’ tissue raises a clinical ‘grape-like’ tissue raises a clinical suspicion suspicion

. .

- the diagnosis is normally made - the diagnosis is normally made on ultrasound or after uterine on ultrasound or after uterine evacuation. evacuation.

-The uterus is larger than dates in -The uterus is larger than dates in about half the cases.about half the cases.

- Associated conditions include - Associated conditions include severe hyperemesis, pre-severe hyperemesis, pre-eclampsia and unexplained eclampsia and unexplained anaemia and ovarian cysts. anaemia and ovarian cysts.

-The high circulating levels of -The high circulating levels of hCG have a TSH like action and hCG have a TSH like action and can cause clinical can cause clinical thyrotoxicosis. thyrotoxicosis.

-Choriocarcinomas may present -Choriocarcinomas may present with the symptoms of distant with the symptoms of distant metastases (cerebral, metastases (cerebral, pulmonary).pulmonary).

DiagnosisDiagnosis

A ‘snowstorm’ appearance with A ‘snowstorm’ appearance with multiple highly reflective echoes multiple highly reflective echoes and areas of vacuolation within and areas of vacuolation within the uterine cavity on ultrasound the uterine cavity on ultrasound examination usually suggests examination usually suggests molar disease.molar disease.

- In a partial mole, a gestation - In a partial mole, a gestation sac with a fetus may also be sac with a fetus may also be presentpresent

- Other imaging, such as chest - Other imaging, such as chest X-rays, CT or MRI may be X-rays, CT or MRI may be indicated to exclude pulmonary indicated to exclude pulmonary or cerebral metastases if or cerebral metastases if choriocarcinoma is suspected. choriocarcinoma is suspected.

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-The diagnosis of trophoblastic -The diagnosis of trophoblastic disease is confirmed by disease is confirmed by histological examination of histological examination of products of conception removed products of conception removed at the time of uterine evacuationat the time of uterine evacuation

ManagementManagement

-Once the diagnosis is -Once the diagnosis is established, the pregnancy is established, the pregnancy is terminated by suction curettage.terminated by suction curettage.

- Occasionally, repeat - Occasionally, repeat evacuation may be required if :evacuation may be required if :

1-there is persistent bleeding1-there is persistent bleeding 2- or a raised serum hCG but 2- or a raised serum hCG but

routine second evacuation is not routine second evacuation is not helpful.helpful.

- All cases of molar pregnancy in - All cases of molar pregnancy in the UK should be registered with the UK should be registered with one of the trophoblastic disease one of the trophoblastic disease screening centres who will screening centres who will arrange follow-up.arrange follow-up.

-The aim of follow-up is to detect -The aim of follow-up is to detect persistent trophoblastic disease persistent trophoblastic disease and choriocarcinoma.and choriocarcinoma.

- Because all trophoblastic - Because all trophoblastic tumours produce hCG patients tumours produce hCG patients can be monitored by can be monitored by measurement of urinary or measurement of urinary or serum hCG levels,serum hCG levels,

Levels are checked fortnightly until Levels are checked fortnightly until the serum level is <2 IU/L,the serum level is <2 IU/L,

- then monthly for 6 months - then monthly for 6 months following this if the hCG is negative following this if the hCG is negative within 6 weeks of treatment.within 6 weeks of treatment.

- If the hCG takes longer than 6 - If the hCG takes longer than 6 weeks to become negative, follow-weeks to become negative, follow-up is continued for a further 12 up is continued for a further 12 months months

-Patients should be counselled -Patients should be counselled to avoid pregnancy until 6 to avoid pregnancy until 6 months after the serum hCG months after the serum hCG levels fall to normal.levels fall to normal.

-There is a 0.8–2.9% risk of -There is a 0.8–2.9% risk of recurrence in subsequent recurrence in subsequent pregnancies after one mole,pregnancies after one mole,

- 15–28% after two moles and - 15–28% after two moles and serum hCG levels should be serum hCG levels should be checked 6 weeks after any checked 6 weeks after any subsequent pregnancy.subsequent pregnancy.

-Chemotherapy with -Chemotherapy with methotrexate (with folinic acid methotrexate (with folinic acid rescue) is indicated for a rising rescue) is indicated for a rising hCG levelhCG level

in the absence of a new in the absence of a new pregnancy, an hCG pregnancy, an hCG persistentlypersistently >20 000 IU/L by 4 >20 000 IU/L by 4 weeks after treatment, weeks after treatment,

-persistent symptoms or -persistent symptoms or evidence of metastatic disease. evidence of metastatic disease. Subsequent fertility does not Subsequent fertility does not appear to beappear to be

impaired by chemotherapy and impaired by chemotherapy and there does not seem to be an there does not seem to be an increased incidence of other increased incidence of other chromosomal abnormalities.chromosomal abnormalities.

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