CHORIOCARCINOMADIAGNOSIS AND MANAGEMENT

DR.ATHUL ABHILASH SMIMS

Gestational choriocarcinoma

A malignant neoplasm composed of large sheets of biphasic, markedly atypical trophoblast without chorionic villi

Gestational choriocarcinoma may occur subsequent to a molar pregnancy (50% of instances), an abortion (25%), a normal gestation (22.5%) or an ectopic pregnancy (2.5%)

In rare cases an intraplacental choriocarcinoma is diagnosed immediately following pregnancy from placental pathological examination

Morphology

The choriocarcinoma is classically a soft, fleshy, yellow-white tumour with a marked tendency to form large pale areas of ischemic necrosis, foci of cystic softening, and extensive haemorrhage

Histopathology

The classic pattern of choriocarcinoma has been described as bilaminar, dimorphic, or biphasic.

Alternating arrangement of mononucleate trophoblastic cells and syncytiotrophoblastic cells characterizes choriocarcinoma.

The intermediate trophoblast in choriocarcinoma may show marked variation in the degree of cytologic atypia.

Vascular invasion often is prominent. Chorionic villi are not a component of choriocarcinoma

that differentiates choriocarcinoma from invasive mole. Choriocarcinoma lacks the intrinsic endothelium-lined

vascular channels in the centre of a tumour, making it a unique malignant solid tumour.

Tumour spread and staging

1. DIRECT SPREAD : to the parametrium, tubes and ovaries.

2. BLOOD SPREAD : occurs early to distant organs. The commonest sites are

1-Lungs(80 %) 2-Vagina(30 %) 3-Brain(10 %) and 4-Liver(10 %).

FIGO classification

Stage I Disease confined to uterus. Stage II Extends outside of the uterus but is limited to

the genital structures (adnexa ,vagina , broad ligament). Stage III Extends to the lungs, with or without known

genital tract involvement. Stage IV metastases to other organs ( brain, liver ,

kidneys, ovaries, bowel)

CLINICAL FEATURES

1- Persistent or irregular vaginal bleeding: it is the commonest symptom occurring after labour, abortion or evacuation of a vesicular mole. Bleeding can occur within days or months but rarely after 2 years.

2- Vaginal discharge: which is blood stained and offensive due to ulceration and infection of the growth .

3- amenorrhea: may be present due to continuous hCG production. 4-Dyspnoea and haemoptysis are noticed with lung metastasis. 5-The appearance of neurological symptoms like hemiplegia,

epilepsy, headache and visual disturbances suggest brain metastasis.

MANAGEMENT

INVESTIGATIONS (1) Uterine curettage: should be done in every case of

persistent or irregular uterine bleeding after labour, abortion or molar pregnancy. However, intramural tumour cannot be detected by curettage.

(2) Serum β-hCG: persistent or rising titres in absence of pregnancy are indicative of trophoblastic neoplasia.(if the level rises more than 100,000 mIU/ml, it is a risk factor)

(4) IMAGING: Regardless of the imaging modality used, choriocarcinoma often appears as a mass enlarging the uterus. Sometimes it manifests as a discrete, central, infiltrative mass. Its heterogeneous appearance correlates with necrosis and haemorrhage that characterise these lesions.

Plain X-ray chest: may show secondaries in the form of " cannon balls" or "snowstorm" appearance

Ultrasonography: to detect tumour, cystic ovaries and exclude remnants of conception.

CT scan: for lungs, liver, brain and bone.

Lumbar puncture: to rule out meningitis and also to measure the hCG level in the CSF.

Blood studies: a- complete blood picture including platelet count. b- Renal, liver and thyroid function tests. c- Blood group.

Treatment:

Chemotherapy is the treatment of choice for choriocarcinoma.

METHOTREXATE is the drug of choice.(this drug interferes with the formation of nucleic acid and mitosis in the malignant cells and thereby arrests the growth).

In low risk patients-single drug i.e.methotrexate is given. If the patient has jaundice then actinomycin D should be

given.

SINGLE DRUG REGIMEN IN LOW RISK CASES

MULTIDRUG THERAPY

Multidrug therapy used most commonly is Bagshaw regime consisting of:-

E=ETOPOSIDE (100 mg/m2 IV infusion in saline over 30 min). M=METHOTREXATE (100mg/m2 IV infusion over 12 hours) A=ACTINOMYCIN D(0.5 mg IV stat) C=CYCLOPHOSPHAMIDE (600 mg IV in saline) O=VINCRISTINE(ONCOVIN) (10 mg/IV stat) High risk patients and patients with stage 4 are to be treated with

combination chemotherapy-EMACO. This course is repeated every 3 weeks.

ROLE OF SURGERY

1. TOTAL HYSTERECTOMY is done if required in choriocarcinoma.The ovaries are not usually involved and if involved, can be effectively cured with postoperative chemotherapy, hence bilateral salpingoopherectomy is not done.

INDICATIONS OF HYSTERECTOMY

Lesions confined to the uterus in women aged ›35 years, not desirous of fertility.

Placental site trophoblastic tumour. Intractable vaginal bleeding. Localized uterine lesion resistant to chemotherapy. Accidental uterine perforation during uterine curettage.

2. LUNG RESECTION 3. CRANIOTOMY

RADIATION

Patients with brain metastasis require whole brain radiation therapy(3000 cGy over 10 days).

Intrathecal high dose methotrexate may be administered to prevent haemorrhage and for tumour shrinkage.

Liver metastasis: Interventional radiology(hepatic artery ligation or embolization) or whole liver radiation(2000 cGy over 10 days) along with chemotherapy may be effective.Hepatic metastasis has a poor prognosis.

PROGNOSIS

The cure rate is almost up to 100 percent in low risk and about 70 percent in high risk metastatic groups.

Follow up is mandatory for all patients at least for 2 years.

Serum hCG is measured weekly until it is negative for three consecutive weeks. Thereafter it is measured monthly for 6 months and 6 months thereafter for life.