Gestational Trophoblastic Neoplasia Flashcards
What are two types of Gestational trophoblastic neoplasia’s?
- Complete mole
- Partial Mole
What are two types of Complete moles?
- 0 eggs, 1 Haploid sperm that duplicates
- 0 Eggs, 2 Haploid sperm
What are two examples of Partial moles?
- 1 egg, 2 sperm
- 2 egg, 1 sperm (NOT GTN No molar placenta)
What is the main function of the trophoblast?
To gain access to maternal circulation
Besides gaining access to maternal circulation, trophoblasts can also do what? 3
- infiltrate into maternal tissues
- Invade vessels
- Can be transported to the lungs (embolism)
What does a Hydatidiform mole refer to?
The cystic degeneration of chorionic villi in molar pregnancy
(Molar pregnancy with many cysts)
What does hydatidiform mole encompasses? 2
- Complete molar pregnancy
- Partial molar pregnancy
What is a molar pregnancy?
Abnormal proliferation of pregnancy- related trophoblast with progressive malignant potential
When an non viable fertilized egg implants into the uterus
What are four types of molar pregnancies?
- Complete molar pregnancy
- Partial molar pregnancy
- Invasive mole
- Choriocarcinoma
How often is molar pregnancies?
1/1000 pregnancies
What are three risk factors of molar pregnancies?
- Advanced maternal age
- Prior molar pregnancies
- Asian ancestry
What is the most common form of GTN?
Complete molar pregnancy
What happens to a complete molar pregnancy if treated early?
Benign (if treated early)
What is the karyotype for a complete mole?
46xx or 46yy- occurs 80% of the time
What are the chromosomal origin like for complete Moles? (maternal or paternal)
exclusively paternal in origin
Why is the chromosomes of complete moles paternal?2
- Ovum with absent or inactive maternal chromosomes is fertilized by a normal haploid sperm
- The Paternal chromosomes duplicate to produce the diploid karyotype of 46xx or 46yy
For a complete mole how often can a 46xy happen?
20% of the time
What is the origin for a 46xy complete mole? (maternal or paternal)
Exclusively paternal in origin
How can a complete mole be 46xy?
Occasionally an empty ovum can be fertilized by 2 haploid sperm (dyspermy)
What is the sonography appearance of a complete molar pregnancy?3
- No fetus
- No normal placenta
- Placenta is replaced by abnormal hydropic chorionic villi with excessive trophoblastic proliferation
What are s/s for complete molar pregnancies? 7
- Large for dates uterus
- Vaginal bleeding (90%)
- Passage of hydropic villi (80%)
- ++hCG serum >100,000 miu/ml
- Hyperemesis gravidarum
- Toxemia or pre-eclampsia before 24 weeks
- Respiratory failure
What is normal hCG serum levels?
<60,000 miu/ml
What does a complete mole appear like sonographically during the 1st trimester?
incomplete abortion
What does a complete mole appear like during the 2nd trimester sonographically?
Echogenic tissue that expands the endometrial canal with multiple cystic spaces that range in size up to 2cm giving a grape like appearance
In complete moles what might occur bilaterally up to 46% of the time? Why? (what kind of cysts we see)
- Ovarian theca lutein cysts
- Due to elevated hCG
What is the karyotype for partial molar pregnancies?
Triploid karyotype
What are examples of triploid karyotype?
69xxx, 69xxy, 69xyy
How does a partial molar pregnancy form? 2
- Fertilization of a diploid (46 chromosome)
- 1 Normal Ovum fertilized by 2 haploid sperm
With a partial molar pregnancy, what can be said about the fertilization of a diploid egg? 2 (origin, GTN)
- Maternal or digyny triploidy origin
- No GTN
In terms of a partial mole, what can be said about 1 normal ovum fertilizied by 2 haploid sperm?2 (Maternal or paternal origin, what kind of disease is it)
- Parternal or diandric tripoidy
- GTN
What is a diandric triploidy? What kind of origin does it have?
- Abnormal fetus with triploid karytype
- Paternal origin
What abnormalities can be seen with children with diandric triploidy?4
- Hypertelorism
- Hydrocephalus
- Holoprosencephaly
- Syndactly
What is seen with Diandric triploidy?2 (s/s)
- Symmetric IUGR
- Large and hydropic placental tissue (partial mole)
What is the origin of digyny triploidy?
Maternal origin
What is a digyny triploidy?
Abnormal fetus with triploidy syndrome
What can be said about the placenta size of digyny tripoidy?
Placenta is small in size therefore, No GTN
When would we see digyny triploidy?
2nd and 3rd trimester
What would we see with digyny triploidy with the fetus?
Severe asymmetric IUGR fetus
What might the mother develop with digyny triploidy? (s/s)
Early pre-eclampsia
What are treatments of complete and partial mole? 3
- Evacuation of uterine contents
- hCG titer sampling weekly or monthly for a year
- Avoid pregnancy for at least one year
What are two ways to evacuate uterine contents?
- Induced labour
- D and C
What is the occurrence rate for complete molar pregnancy?
80%
What is the occurrence rate for partial molar pregnancies?
95%
Is it possible to have a complete mole with a coexistent twin pregnancy?
Although rare, a normal pregnancy with a normal placenta and molar placenta seen separately
Invasive mole and choriocarcinomas are referred to as what?
Persistent trophoblastic neoplasia (PTN)
What is the malignancy of PTN?
Malignant potential
PTN stands for what?
Persistent trophoblastic neoplasia
Can PTN be locally invasive or metastatic?
Either or
What is the most curable gynecological malignancy?
PTN
What kind of complication is PTN? (how dangerous is this)
Life threatening
PTN usually occurs in what kind of setting?
Molar pregnancy
20% of complete molar pregnancies develop into what?
Persistent disease
In rare cases PTN can occur after what?3
- Normal term delivery
- Spontaneous abortion
- Ectopic pregnancy
Can PTNs occur after normal deliveries? When was the last case?
- Some PTNs have been reported to occur years after the pregnancy
- Latest reported case was 23 years after a normal pregnancy
What is another name for invasive moles?
Chorioadenoma destruens
What is the most common form of PTN?
Invasive mole (80-95% of cases)
What are the stages of a invasive mole?3
- Confined to the uterus
- Spread to adjacent organs and vasculature
- Embolize to distant sites including the lung and brain
What do we see with invasive moles?
Presence of chorionic villi and proliferating trophoblast deep in the myometrium
How common are choriocarcinoma?
rare - 1/30,000 pregnancies
What does choriocarcinomas start as?
Molar pregnancies most of the time (50-80%)
How many molar pregnancies become choriocarcinomas?
1/40 pregnancies
When can choriocarcinomas present? 3
After
1. Miscarriages
2. Abortions
3. Normal pregnancies occasionally
What is the histology of choriocarcinoma?
Abnormal proliferating trophoblast with no formed villi (purely cellular lesion)
What are the stages of choriocarcinoma? 4
- Invades the myometrium
- Invades vasculature causing hemorrhage
- Necrosis
- Distant metastases are common
Distant metastases with choriocarcinomas happen how frequently where?
Lungs followed by liver and brain
What are s/s of both choriocarcinomas and invasive moles?2
- Vaginal bleeding
- Respiratory compromise
What is the treatment options for PTNs?2
- Follow serum hCG after evacuation (D and C)
- Diagnosis can be confusing if many systems are affected already
When does hCG disappear with PTN?
About 7-14 weeks following a molar pregnancy
What does a PTN require?
EV scan
What are sonographic features of PTN? 5
- Usually Focal echogenic nodules in the myometrium
- Lesions may appear hyperechoic, complex or multicystic (grape like)
- Uterus is bulky if the tumor replaces entire myometrium
- Uterus will look heterogenous and lobulated
- May see extension of tumor to the other organs or pelvic side walls
What is the DDX for PTN sonographically?3
- Adenomyosis
- Fibroids
- AV malformations
What can be said about Sonographic features of PTN?
U/S does not differentiate between different forms of PTN, pathology does
What are colour doppler features of PTN? 3
- Colour aliasing due to AV shunting
- Chaotic vascular arrangement
- The size of the lesions look larger with colour doppler than on the grey scale
What is duplex doppler features of PTN?2
- Increased peak systolic velocity
- Low resistive index (low impedance)
What does normal systolic velocity look like? What does it look like with PTN?
- <50cm
- > 50cm, usually >100cm/s
What does normal RI look like? What does it look like with PTN?
- Normal RI is 0.7
- RI <0.5
What part of u/s plays the greater role in the diagnosis of PTN over the diagnosis of primary molar pregnancy?
Doppler
Colour and duplex features are typically of what? What same results would be demonstrated with what?
- Colour and duplex doppler features are typically of trophoblastic flow (Placenta) either normal or abnormal
- The same doppler results would be demonstrated with early pregnancy, missed abortion, or retained products of conception
In terms of PTN, other conditions that would have the same high velocity low impedance type flow are what? 3
- PID
- Pelvic abscess
- Benign and malignant ovarian neoplasia
In terms of clinical findings for PTN doppler, clinical findings, serum hCG, and sonographic appearance would help distinguish between what? 3
PTN conditions
1. PID
2. Abscess
3. Ovarian neoplasms
What is sonography used to in terms of PTN and GTN?
Stage and monitor response therapy
What is more preferred, Sonography or angiography to follow these lesions?
Sonography because it is more safe
What is the prognosis of non metastatic PTN?
Excellent
What is PTN treated with? (drug)
Methotrexate
Metastatic PTN has what two categories?
- Low risk - cured with simple chemotherapy
- High risk
What would constitute high risk metastatic PTN? 3
- Having the disease longer than four months
- Having a pretreatment hCG level of >40,000 miu/ml
- Presence of brain or liver metastases
What is the prognosis of high risk metastatic PTN?
Poor prognosis
What is the treatment option for high risk metastatic PTN? 3
- Use multi-agent chemotherapy
- Radiotherapy
- Surgery
Choriocarcinoma can occur with what?
Dysgerminoma of the ovary
How common is dysgerminoma?
Rare
What is dysgerminoma the ovarian counterpart of?
Ovarian counterpart of seminoma
What is a dysgerminoma?
Highly malignant germ cell tumor that produces hCG
What is not the cause of Non GTN?
Pregnancy
Non GTN endometrium responds to what?
The high HCG from the ovarian tumor
What is the prognosis of Non-GTN?
Poor prognosis compared to the gestation choriocarcinoma (PTN)
What does this image represent?
Complete mole
What does this image represent?
Complete mole
What does these images represent?
Partial moles
What does this image represent?
Maternal Triploidy