What is Epithelioid MPNST (Malignant Peripheral Nerve Sheath Tumour)?
Epithelioid MPNST is a rare type of MPNST (malignant peripheral nerve sheet tumour) that accounts for almost 5% of all cases and has a unique immunophenotypic characteristic. A malignant peripheral nerve sheath tumour (MPNST) is a type of connective tissue (tissues that bind, separate, or support other tissues or organs) cancer that surrounds the nerves. Tumours develop mostly at the lower legs, thighs, arm, forearm, and Para-spinal region. Immunophenotyping is a technique used to identify cells on the behalf of the type of markers or antigens (a toxin or a substance that induces immune response) found on the surface of the cell. This technique is used to diagnose the disease. For example, this technique is used for the detection of tumour markers in leukemia (blood cancer).
Disease Etiology (Causes)
There is no well-known cause of epithelioid–MNPST, however, certain genetic mutations are held responsible for the development of these tumours. Unlike MNPST, its development from pre-existing peripheral nerve sheath tumour and association with Neurofibromatosis factor-1(a genetic /hereditary disorder of the brain and spinal cord) is uncommon.
Diagnosis and Treatment
Diagnostic tests are:
- Imaging techniques like CT-scan, PET-scan, MRI, and X-ray.
- Tumour or tumour site biopsy
- Immunohistochemistry
Treatment methods include surgical removal of the tumour, chemotherapy, and radiotherapy.
What Support can we Give for Epithelioid MPNST?
Epithelioid MPNST is 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 Epithelioid MPNST, 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:
