Definition/General

Introduction:
-Placental choriocarcinoma is a highly malignant trophoblastic tumor composed of both syncytiotrophoblast and cytotrophoblast without chorionic villi
-It represents the most aggressive form of gestational trophoblastic disease
-The tumor has high metastatic potential
-It occurs in 1 in 20,000-40,000 pregnancies
-Excellent response to chemotherapy when diagnosed early.
Origin:
-Arises from abnormal trophoblastic proliferation
-May develop after any gestational event (normal pregnancy, abortion, molar pregnancy)
-Malignant transformation of trophoblastic tissue
-Loss of normal growth controls
-Invasive and metastatic from onset.
Classification:
-Part of gestational trophoblastic neoplasia: Invasive mole
-Choriocarcinoma
-Placental site trophoblastic tumor
-Epithelioid trophoblastic tumor
-By origin: Post-molar (50%)
-Post-abortion (25%)
-Post-term pregnancy (25%).
Epidemiology:
-Incidence: 1:20,000-40,000 pregnancies
-Higher in Asian countries
-Risk after complete mole: 15-20%
-Risk after partial mole: <2%
-Bimodal age distribution
-More common at extremes of age.

Clinical Features

Presentation:
-Irregular vaginal bleeding
-Persistently elevated β-hCG
-Uterine enlargement
-Theca lutein cysts
-Pulmonary symptoms (metastases)
-Neurological symptoms (brain metastases)
-Abdominal pain.
Symptoms:
-Abnormal bleeding (90% cases)
-Amenorrhea followed by bleeding
-Pelvic pain
-Cough, hemoptysis (lung metastases)
-Headache, seizures (brain metastases)
-Abdominal distension.
Risk Factors:
-Previous molar pregnancy (major risk)
-Extremes of age (<20, >40 years)
-Asian ethnicity
-ABO blood group incompatibility
-Previous miscarriages
-Oral contraceptive use
-Dietary factors (low carotene).
Screening:
-Serial β-hCG monitoring
-Pelvic ultrasound
-Chest X-ray (lung metastases)
-CT/MRI (staging)
-Complete blood count
-Liver function tests.

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Gross Description

Appearance:
-Hemorrhagic, friable mass
-Dark red to purple color
-Soft, necrotic areas
-No identifiable chorionic villi
-Extensively necrotic and hemorrhagic
-Infiltrative growth pattern.
Characteristics:
-Bulky, irregular masses
-Areas of necrosis and hemorrhage
-Myometrial invasion
-Vascular invasion prominent
-Absence of villous structures.
Size Location:
-Variable size: few cm to large masses
-Uterine corpus most common
-Cervical involvement possible
-Extrauterine sites: lungs, brain, liver
-Metastatic deposits.
Multifocality:
-Multifocal growth common
-Extensive metastases
-Hematogenous spread
-Pulmonary nodules
-CNS involvement.

Microscopic Description

Histological Features:
-Biphasic pattern: syncytiotrophoblast and cytotrophoblast
-Absence of chorionic villi
-Extensive necrosis
-Hemorrhage
-Vascular invasion
-High mitotic activity.
Cellular Characteristics:
-Syncytiotrophoblast: multinucleated giant cells
-Cytotrophoblast: mononuclear cells
-Marked pleomorphism
-Prominent nucleoli
-Atypical mitoses
-Extensive necrosis.
Architectural Patterns:
-Sheets and nests of trophoblast
-No villous architecture
-Hemorrhage and necrosis
-Vascular space invasion
-Invasive growth pattern.
Grading Criteria:
-WHO classification: Low-risk vs high-risk
-FIGO staging
-Prognostic scoring: age, β-hCG level, size, sites of metastases
-Risk stratification guides therapy.

Immunohistochemistry

Positive Markers:
-β-hCG (syncytiotrophoblast)
-hPL (human placental lactogen)
-Cytokeratin (trophoblast)
-Inhibin-α
-CD68 (occasional)
-Ki-67 (high proliferation).
Negative Markers:
-p63 (usually negative in syncytiotrophoblast)
-Mel-CAM (extravillous trophoblast)
-EMA (variable)
-CD10 (stromal marker).
Diagnostic Utility:
-Confirms trophoblastic origin
-Distinguishes from other tumors
-β-hCG essential for diagnosis
-Proliferation markers assess aggressiveness.
Molecular Subtypes:
-Post-molar choriocarcinoma
-Post-gestational choriocarcinoma
-Primary ovarian choriocarcinoma
-Non-gestational choriocarcinoma.

Molecular/Genetic

Genetic Mutations:
-p53 mutations
-PTEN alterations
-PIK3CA mutations
-Beta-catenin pathway
-Chromosomal instability.
Molecular Markers:
-High β-hCG production
-Increased VEGF
-Matrix metalloproteinases
-Angiogenic factors
-Drug resistance markers.
Prognostic Significance:
-β-hCG level most important
-Site of metastases
-Time from pregnancy
-Prior chemotherapy
-Histological features.
Therapeutic Targets:
-Methotrexate (single agent)
-Combination chemotherapy (EMA/CO)
-High-dose therapy (resistant cases)
-Surgery (selected cases)
-Immunotherapy (experimental).

Differential Diagnosis

Similar Entities:
-Invasive mole
-Placental site trophoblastic tumor
-Epithelioid trophoblastic tumor
-Carcinoma (poorly differentiated)
-Melanoma
-Sarcoma.
Distinguishing Features:
-Choriocarcinoma: No chorionic villi
-Biphasic pattern
-High β-hCG
-Invasive mole: Villi present
-Intact basement membrane
-PSTT: Intermediate trophoblast
-Lower β-hCG.
Diagnostic Challenges:
-Extensive necrosis obscures morphology
-Small biopsy samples
-Distinguishing from other poorly differentiated tumors
-Clinical correlation essential.
Rare Variants:
-Choriocarcinoma syndrome
-Intraplacental choriocarcinoma
-Choriocarcinoma in teratoma
-Primary extragonadal choriocarcinoma.

Sample Pathology Report

Template Format

Sample Pathology Report

Complete Report: This is an example of how the final pathology report should be structured for this condition.

Specimen Information

[Curettage/Hysterectomy] specimen with choriocarcinoma

Diagnosis

Choriocarcinoma

Clinical Correlation

Elevated β-hCG: [X] mIU/mL, [clinical presentation]

Gross Findings

Hemorrhagic, friable tissue, no identifiable chorionic villi

Microscopic Findings

Biphasic trophoblast population, no villi, extensive necrosis

Immunohistochemistry

β-hCG: positive (syncytiotrophoblast), Ki-67: [X]%

Staging

FIGO Stage [I-IV], WHO Risk Score: [low/high]

Risk Assessment

[Low/High] risk based on prognostic factors

Treatment Recommendation

[Single agent/Combination] chemotherapy indicated

Final Diagnosis

Choriocarcinoma, [risk category]