What is Alveolar Rhabdomyosarcoma of the Mediastinum?
Alveolar Rhabdomyosarcoma of the Mediastinum is a rare type of soft tissue cancer found mostly in children. It develops in the mediastinum, which is the central part of the chest, though it can also arise in other parts of the body.
Causes
The exact cause is not well known. However, certain genetic mutations are believed to play a role in the development of this cancer. These mutations cause changes in specific chromosomes, particularly partial crossovers between chromosomes 2 and 13 or 1 and 13. As a result, abnormal genes are formed, leading to uncontrolled cell growth and the development of cancer.
Signs and Symptoms
Common symptoms may include:
- Chest pain
- Difficulty breathing
- Persistent cough
- Swelling of the face and neck
- A noticeable mass in the chest or other parts of the body
Diagnosis
To diagnose Alveolar Rhabdomyosarcoma, doctors may use the following:
- Complete Blood Count (CBC)
- Serum Tumour Markers
- Imaging tests like ultrasound, CT scan, MRI, and X-rays to identify the location, size, and spread of the tumour.
Treatment
Treatment usually involves a combination of surgery, chemotherapy (using drugs like vincristine, actinomycin, and cyclophosphamide), and radiation therapy. Complete surgical removal can be challenging because the tumour often grows near critical nerves and blood vessels in the chest. Imaging tests like CT scans and MRIs are essential before surgery to determine how close the tumour is to surrounding tissues.
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