Abstract:Neurofibromatoses Type 1(NF 1) is an autosomal dominant disease characterized by disordered growth of ectodermal tissues, and is part of a group of disorders called Phakomatoses (neurocutaneous syndrome). NF 1 (aka Von Recklinghausen's disease) patients develop both benign and malignant tumors at an increased frequency. Malignant peripheral nerve sheath tumours (MPNST) is a very rare tumor, with an incidence of 1 per 1,00,000 population. MPNSTs occur in about 2% to 5% of neurofibromatosis patients and constitu… Show more
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