Re-treatment of tumours in already irradiated locations

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Malignant nerve sheath tumours, also known as MPNST (Malignant Peripheral Nerve Sheath Tumours), are rare soft tissue sarcomas originating from peripheral nerves or cells associated with nerve sheaths. As they may originate from different cell types, sometimes it may be particularly difficult to diagnose them.

Also called malignant schwannomas, malignant peripheral nerve sheath tumours are often particularly aggressive, tend to recur or metastasize, and can affect several anatomical sites. In most cases, MPNST originate in the main trunk nerves such as the sciatic nerve or the brachial and sacral plexuses; in other cases, they occur at the head and neck level, and near the limbs. They may arise spontaneously or develop from an already existing neurofibroma.

Malignant nerve sheath tumours, which account for around 5%-10% of all soft tissue sarcomas, have an estimated incidence of 1: 100,000. They can affect both men and women equally, with an average age of onset between 20 and 50 years in general.