What is Malignant Extrarenal Rhabdoid Tumour?
Malignant extrarenal rhabdoid tumour (MERT) is a rare and highly aggressive cancer that originates outside of the kidneys. Rhabdoid tumours were first identified in the kidneys but can occur in other soft tissues throughout the body, including the liver, brain, and soft tissues of the trunk and extremities. This form of cancer is most commonly diagnosed in infants and young children, though it can occasionally occur in adults.
Symptoms
The symptoms of Malignant Extrarenal Rhabdoid Tumour depend on the tumour’s location but can include:
- A noticeable lump or swelling in the affected area.
- Pain or discomfort in the region of the tumour.
- Symptoms related to the compression of nearby organs or structures can vary widely based on the tumour’s location.
- General symptoms like fatigue, weight loss, and malaise.
Disease Aetiology (Causes)
The exact cause of MERT is not clearly understood. However, genetic mutations, particularly in the SMARCB1 gene, have been associated with this disease. These mutations are generally considered sporadic and are not thought to be inherited.
Diagnosis
Diagnosing Malignant Extrarenal Rhabdoid Tumour involves:
- Physical Examination: Noting any visible or palpable abnormalities.
- Imaging Studies: MRI, CT scans, and ultrasound to identify and assess the tumour’s extent.
- Biopsy: A sample of the tumour is examined under a microscope to confirm the diagnosis.
- Genetic Testing: This can be helpful to identify characteristic genetic mutations associated with MERT.
Treatment
Treatment typically includes:
- Surgery: To remove the tumour, often the first line of treatment if it is operable.
- Chemotherapy: Due to the aggressive nature of MERT, chemotherapy is usually a key component of treatment.
- Radiation Therapy: May be used in certain cases, depending on the tumour’s location and response to other treatments.
- Targeted Therapy: As research progresses, treatments targeting specific pathways in the cancer cells are being explored.
What support can we give for Malignant Extrarenal Rhabdoid Tumour?
Malignant Extrarenal Rhabdoid Tumour is a rare cancer, meaning it is not as well-known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Malignant Extrarenal Rhabdoid Tumour, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
You can help us with your donation:
Without a Ribbon is a charity that works hard to aid those who suffer from rare cancers. You can help our cause in a variety of ways:
