Trends in systemic therapy for malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumors are a type of soft tissue sarcoma deriving from Schwann cells that usually appear in type 1 neurofibromatosis patients but also sporadically. Tumors frequently interest the nerves in the limbs. They represent a therapeutical challenge due to difficulty of resection and relative radio-resistance and chemo-resistance. The paper aims to describe targeted therapy used in this setting and news concerning the molecular changes that lead to carcinogenesis initiation and promotion. A short selection of literature data was made using PRISMA criteria on this topic. However, due to the rarity and heterogeneity of these tumors, personalized treatment is necessary.