Malignant peripheral nerve sheath tumour

Abstract

Sarcomas belong to a diverse group of mesenchymal tumours. They make up for 1% malignant tumours in adults population. There are estimated 1500-2000 new cases of soft tissue and bone sarcomas yearly in Poland. About 5-10% of all sarcomas are malignant peripheral nerve sheath tumours (MPNST). Malignant peripheral nerve sheath tumours (MPNST) are usually located within the torso (about 50% cases), more seldom within the extremities (30%) or head and neck (20%). MPNST is a rare condition, which is why no guidelines regarding the diagnostic process and treatment can be found in literature. The modern treatment for soft tissue sarcomas involves a combination of surgery and radiotherapy, and in some cases chemotherapy. A cure can only be reached by a radical removal of the tumour. A good cooperation between the surgeon and the radiotherapy oncologist increases the chances of successful treatment. At the same time the importance of immunohistochemical assessment should be stressed, with the result determining the further course of therapy.