What is Malignant External Rhabdoid Tumour?
Malignant external rhabdoid tumour (MERT) is a rare and highly aggressive childhood cancer that primarily affects the kidneys, but it can also arise in other parts of the body, such as soft tissues, muscles, tendons, connective tissues, and the brain. When these tumours occur in the brain, they are called Atypical Teratoid Rhabdoid Tumours (ATRT).
MERT is highly aggressive and has a strong ability to spread to other parts of the body, such as the lungs, liver, and lymph nodes. Due to its rapid spread, the prognosis for this cancer is poor.
Causes
The exact cause of MERT is not well understood. However, researchers have discovered that mutations (false changes) or deletions in the SMARCB1 gene (also known as INI1, SNF5, and BAF47) exist in nearly all rhabdoid tumours.
Malignant external rhabdoid tumours are rare. In the United States, approximately 20 to 25 new cases are diagnosed annually. MERT most commonly affects infants and toddlers. The average age of diagnosis is around 15 months. MERT affects both males and females equally.
Sign and Symptoms
The signs and symptoms of MERT depend on the tumour’s location and spread to body parts. Common symptoms include:
- An abdominal mass or lump is usually the first sign. Pain is present, but it is difficult to assess since this cancer typically occurs in young children who are unable to explain the location of their pain. However, the child often remains irritable and cries, indicating discomfort from the pain.
- Weight loss
- Fatigue
- Fever
- Bloody urine is observed in 50 percent of patients with MERT
- In Atypical Teratoid Rhabdoid Tumors (ATRT) fits/seizures are manifested
Diagnosis
The following diagnostic techniques and procedures can be used to make a diagnosis. These are
- Blood tests such as complete blood count (CBC)
- Urine complete examination (Urine test)
- Diagnostic imaging techniques such as USG, CT-scan, MRI, and PET scan can be used to detect the site, size, and extent of the tumour.
- Tumour biopsy
Treatment
The treatment for MERT is multifaceted, incorporating surgery, chemotherapy, radiation therapy, and stem cell transplantation. These options are determined based on the patient’s condition and family preferences, considering the tumour’s aggressive nature. To boost the child’s immunity, supportive management such as pain relief, nutritional support, and infection protection is also employed.
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