What is Pulmonary Lymphoepithelioma-like Carcinoma?
Primary Pulmonary Lymphoepithelioma-like Carcinoma (LELC) is a rare type of non-small cell lung cancer (NSCLC) that closely resembles nasopharyngeal carcinoma. This cancer is strongly linked to the Epstein-Barr virus (EBV), particularly in Asian populations. Because of its unique features, LELC differs from other lung cancers in its behaviour, pathology, and treatment response.
Causes
Primary Pulmonary Lymphoepithelioma-like Carcinoma is primarily associated with EBV infection, making it distinct from many other lung cancers. Unlike typical lung cancers, smoking is not a well-established risk factor. Genetic studies have also found that PD-L1 overexpression and other molecular changes may impact tumour growth and response to immunotherapy.
Signs and Symptoms
Common symptoms of LELC include:
- Persistent cough
- Coughing up blood (hemoptysis)
- Chest pain
- Unexplained weight loss
- Symptoms related to enlarged lymph nodes in the chest
Diagnosis
Diagnosing LELC requires a combination of tests:
- Imaging: Chest X-ray, CT scan, or PET-CT to detect the tumour and assess its spread.
- Biopsy & Immunohistochemistry: A tissue sample is examined to confirm the diagnosis.
- EBV Testing: EBV-encoded RNA (EBER) testing is crucial for confirming the virus’s role.
- Molecular Testing: PD-L1 expression testing helps determine if immunotherapy is a suitable treatment option.
Treatment
Treatment depends on the stage of the disease:
- Surgery: The best option for early-stage LELC, aiming for complete tumour removal.
- Chemotherapy: Platinum-based regimens are commonly used, especially for advanced or metastatic cases.
- Immunotherapy: PD-L1 inhibitors have shown promising results, particularly in tumours with high PD-L1 expression.
- Radiation Therapy: Used for locally advanced disease or when surgery isn’t an option, helping to slow tumour growth and manage symptoms.
Prognosis
The outlook for LELC is generally better than for many other lung cancers, especially if the patient is EBV-positive. Early-stage treatment is often highly effective, and newer immunotherapy options have improved survival in advanced cases. However, the prognosis depends on how far the cancer has spread and how well the patient responds to treatment.
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