What is Primary Pulmonary Synovial Sarcoma?
Primary Pulmonary Synovial Sarcoma (PPSS) is a very rare and aggressive type of cancer that starts in the lungs. Unlike other lung cancers that may spread from different parts of the body, PPSS originates directly in the lung tissue, specifically from cells called mesenchymal cells.
Epidemiology
PPSS is extremely uncommon, accounting for less than 0.5% of all lung cancer cases. It predominantly affects adolescents and young adults, with a slightly higher occurrence in men compared to women.
Causes
The exact cause of PPSS is not well understood. However, certain genetic mutations are believed to play a role in its development. Unlike many other types of lung cancer, PPSS is not associated with environmental risk factors such as smoking.
Signs and Symptoms
Individuals with PPSS may experience:
- Chest Pain: Discomfort or pain in the chest area.
- Difficulty Breathing: Shortness of breath or laboured breathing.
- Pleural Effusion: Accumulation of fluid in the space surrounding the lungs, leading to breathing difficulties.
Diagnosis
To diagnose PPSS, doctors may use several methods:
- Physical Examination: Assessing the patient’s overall health and identifying any physical signs of the tumour.
- Imaging Tests: CT scans or MRIs to determine the tumour’s location, size, and extent.
- Biopsy: Removing a small sample of the tumour tissue to confirm the diagnosis.
- Fine Needle Aspiration Cytology: Using a thin needle to extract cells from the tumour for examination, especially when the tumour is easily accessible.
- Immunohistochemistry: Performing specialised tests on the tumour tissue to identify specific markers that help confirm the tumour type.
Treatment
The primary treatment for PPSS is surgical removal of the tumour. In addition to surgery, radiation therapy and chemotherapy may be used to target any remaining cancer cells. Due to the aggressive nature of PPSS, early diagnosis and prompt treatment are crucial for improving outcomes.
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