What is Malignant Triton Tumour?
Malignant Triton Tumour (MTT) is a rare and aggressive type of nerve sheath tumour that resembles muscle cells. It falls under the category of malignant peripheral nerve sheath tumours (MPNSTs) and is often associated with Neurofibromatosis Type 1 (NF1). Due to its rapid growth and high recurrence rate, MTT has a poor prognosis.
Causes
Genetic Factors
- 50-70% of MTT cases are linked to mutations in the NF1 gene, which affects the tumour-suppressing protein neurofibromin.
- Some cases involve mutations in other tumour suppressor genes.
Radiation Exposure
- Previous radiation therapy can increase the risk of developing MTT and other MPNSTs.
Gender and Age
- MTT is more common in middle-aged adults.
- Men have a slightly higher risk compared to women.
Signs and Symptoms
Individuals with MTT may experience:
- A rapidly growing, painless mass, often located near major nerves in the arms, legs, or head/neck region.
- Nerve-related symptoms, such as numbness, weakness, or tingling in the arms and legs.
- Restricted movement if the tumour is near a joint.
Diagnosis
Imaging Tests
- CT Scans or MRIs help determine the tumour’s location, size, and blood supply.
Biopsy
- A small tissue sample is examined under a microscope to confirm muscle-like characteristics.
- Markers such as myogenin, desmin, and S-100 help identify MTT.
Immunohistochemistry
- A special test to differentiate MTT from other tumours.
Genetic Testing
- NF1 mutation analysis is conducted, particularly in suspected cases of Neurofibromatosis Type 1.
Treatment Options
Surgery
- The primary treatment involves surgical removal of the tumour and surrounding affected tissues.
Radiation Therapy
- Often used alongside surgery to reduce recurrence risk.
Chemotherapy
- Limited effectiveness, but may be considered for advanced or spreading cases.
- Medications such as ifosfamide, doxorubicin, and vincristine may be used.
Targeted & Experimental Therapies
- mTOR inhibitors and MEK inhibitors (e.g., selumetinib) are being researched as potential treatments.
Prognosis
- Poor overall outlook, with a 5-year survival rate of only 10-20%.
- High recurrence and metastasis rates, commonly spreading to soft tissues, bones, and lungs.
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