Chorioepithelioma

Chorioepithelioma, also known as chorioblastoma, choriocarcinoma, or chorionic carcinoma, is a rare and aggressive malignant tumor derived from trophoblastic cells. These cells play a pivotal role in embryo attachment to the uterus and in the formation of the placenta. Though predominantly forming in the uterus post-fertilization, a minority of chorioepitheliomas may develop in the testis or ovary. Notably, chorioepitheliomas are categorized under the umbrella of gestational trophoblastic diseases.

Diagram showing stage 2 choriocarcinoma

Origin and Development

Chorioepitheliomas primarily arise after the fertilization of an egg by a sperm, leading to aberrant proliferation of trophoblastic cells. The exact cause or trigger for this malignant transformation remains a topic of investigation, though certain genetic and environmental factors might influence its onset.

Pathophysiology

The trophoblastic cells are vital in the early stages of pregnancy, assisting the embryo in attaching to the uterine wall and participating in placental formation. When these cells become cancerous, they proliferate uncontrollably, leading to the formation of a chorioepithelioma. Given their origin, these tumors have an inherent ability to invade blood vessels, which facilitates their spread to distant organs.

Clinical Presentation

Patients with chorioepithelioma may present with:

• Vaginal bleeding unrelated to menstrual cycle
• An enlarged uterus
• Symptoms related to metastatic disease, especially in the lungs, such as cough or difficulty breathing
• Elevated levels of human chorionic gonadotropin (hCG) in the blood

Diagnosis

Diagnostic evaluation for suspected chorioepithelioma includes:

• Ultrasound or MRI of the pelvis to visualize the tumor
• Blood tests to measure hCG levels, which are typically elevated in this condition
• Biopsy of the tumor tissue to confirm diagnosis
• Imaging studies like CT scan or PET scan to detect metastatic spread

Treatment

Given the aggressive nature of chorioepithelioma, prompt and comprehensive treatment is paramount. Options include:

• Chemotherapy: The mainstay of treatment, effective in targeting tumor cells spread throughout the body.
• Surgery: To remove the primary tumor, usually a hysterectomy (removal of the uterus) is performed.
• Radiotherapy: Employed in cases where the tumor is resistant to chemotherapy or if there's localized spread.
• Targeted therapies and immunotherapy: Newer modalities being explored in clinical trials.
• Early detection and treatment significantly improve the prognosis of chorioepithelioma.

Prognosis and Follow-up

Though chorioepitheliomas are aggressive, they are also highly responsive to chemotherapy. Regular follow-ups are crucial, involving:

• Monitoring hCG levels to detect any recurrence
• Imaging studies to ensure there's no metastatic spread or return of the tumor

See also

• Gestational trophoblastic disease
• Malignancy
• Human chorionic gonadotropin
• Tumor

External links

• Chorioepithelioma entry in the public domain NCI Dictionary of Cancer Terms