Teachinf Files

Gestational Trophoblastic Disease 葡萄胎

Diagnosis

1. Made on the basis of D & C.

2. Ultrasound: complete moles－a characteristic vesicular sonographic pattern, "snowstorm" pattern; partial moles－focal cystic spaces in the placental tissues and an increase in the transverse diameter of the gestational sac.

Treatment and follow-up

1. Before evacuation, obtain the following laboratory studies: CBC, D/C, PT, APTT, blood chemistries, including lirer function tests, blood type and screen, and b–hCG; and CXR.

2. Evaluation of associated medical complications, including preeclampsia, hypothyroidism, anemia, infection, electrolyte imbalance and coagulopathy.

3. Suction curettage with oxytocin infusion.

4. Weekly serum b-hCG follow-up.

5. 3 consecutive weeks of undetectable serum b-hCG then monthly determinations for at least half a year.

Hydatidiform Mole:

Complete mole

Partiale mole

Hydatidiform moles categorised as either complete or partial moles.

Features of Complete and Partial Hydatidiform moles.

A. Complete Mole :

Fetal or embryonic tissue : Absent

Hydatidiform swelling of chorionic villi : Diffuse

Trophoblastic hyperplasia : Diffuse

Scalloping of chorionic villi : Absent

Trophoblastic stromal Inclusions : Absent

Karyotype : 46XX; 46XY

B. Partial Mole

Fetal or embryonic tissue : Present

Hydatidiform swelling of chorionic villi : Focal

Trophoblastic hyperplasia : Focal

Scalloping of chorionic villi : Present

Trophoblastic stromal Inclusions : Present

Karyotype : 69XXY; 69XYY

Gestational trophoblastic neoplasia:

Nonmetastatic trophoblastic disease

Good prognosis metastatic trophoblastic disease

Poor prognosis metastatic trophoblastic disease

Placental site trophoblastic tumor

Management of hydatidiform mole:

1. Beta-hCG determination every 1-2 weeks until negative two times

a. Then bimonthly for 1 year

b. Contraception for 6-12 months

2. Physical examination including pelvic every 2 weeks until remission

a. Then every 3 months for 1 year

3. Chest film initially

a. Repeat only if hCG titer plateaus or rises

4. Chemotherapy started immediately if:

a. hCG titer rises or plateaus (determined by 3 tests) during follow-up

b. Metastases are detected at any time

Classification of gestational trophoblastic neoplasia:

1. Nonmetastic disease: no evidence of disease outside uterus

2. Metastatic disease: any disease outside uterus

a. Good prognosis metastatic disease

Short duration (last pregnancy < 4 months)

Low pretreatment hCG titer (< 100,000 IU/24 hr or < 40,000 mlU/ml)

No metastasis to brain or liver

No significant prior chemotherapy

b. Poor prognosis metastatic disease

Long duration (last pregnancy > 4 months)

High pretreatment hCG titer (> 100,000 IU/24 hr or > 40,000 mlU/ml)

Brain or liver metastasis

Significant prior chemotherapy

Term pregnancy

WHO Scoring System Score

Prognostic factors 0 1 2 4

Age ≦39 >39

Antecedent pregnancy HM Abortion Term

Months from pregnancy 4 4 to 6 7 to 12 12

hCG (IU/L) 103 103-104 104-105 105

ABO (female×male) O×A B A×O AB

Largest tumor (cm) 3 to 5 5

Site metastases Spleen GI Brain /Kidney/Liver

Number of metastases 1 to 4 4 to 8 8

Prior chemotherapy Single 2 or more

≦4: low risk

5-7: middle risk

≧8: high risk
　

FIGO Staging for trophoblastic tumors

Stage I Disease confined to the uterus

Stage Ia Disease confined to the uterus with no risk factors

Stage Ib Disease confined to the uterus with one risk factor

Stage Ic Disease confined to the uterus with two risk factors

Stage II GTT extends outside of the uterus but is limited to the genital

structures (adnexa, vagina, broad ligament)

Stage IIa GTT involving genital structures without risk factors

Stage IIb GTT extends outside of the uterus but limited to genital structures

with one risk factor

Stage IIc GTT extends outside of the uterus but limited to the genital

structures with two risk factors

Stage III GTT extends to the lungs with or without known genital tract

involvement

Stage IIIa GTT extends to the lungs with or without genital tract involvement

and with no risk factors

Stage IIIb GTT extends to the lungs with or without genital tract involvement

and with one risk factor

Stage IIIc GTT extends to the lungs with or without genital tract involvement

and has two risk factors

Stage IV All other metastatic sites

Stage IVa All other metastatic sites without risk factors

Stage IVb All other metastatic sites with one risk factor

Stage IVc All other metastatic sites with two risk factors

Risk factors affecting staging include the following:

(1) hCG > 100,000 mlU/ml;

(2) duration of disease > 6 months from termination of the antecedent pregnancy.

The following factors should be considered and noted in reporting: (1) prior chemotherapy for known GTT;

(2) placental site tumors should be reported separately:

(3) histological verification of disease is not required.

Single-Agent Chemotherapy

1. Methotrexate 20-25 mg IM every day for 5 days (with a minimum 7-day rest if possible)

2. Methotrexate 1 mg/kg IM on days 1, 3, 5, and 7

Folinic acid 0.1 mg/kg IM on days 2, 4, 6, and 8 (with a minimum 7-day rest if possible)

EMA-CO Chemotherapy

Course 1 (EMA)

Day 1 Etoposide, 100 mg/m2, IV infusion in 200 ml of saline

Dactinomycin, 0.5 mg, IV stat

Methotrexate, 100 mg/ m2, IV stat 200 mg/ m2, IV infusion over 12 hours

Day 2 Etoposide, 100 mg/m2, IV infusion in 200 ml of saline over 30 minutes

Dactinomycin, 0.5 mg, IV stat

Folinic acid, 15 mg, IM or orally every 12 hours for 4 doses

beginning 24 hours after start of methotrexate

Course 2 (CO)

Day 8 Vincristine, 1.0 mg/m2 IV stat

Cyclophosphamide, 600 mg/m2, IV infusion