<unk> <unk>  is a form of cancer of the connective tissue surrounding nerves. On gross pathology, a large firm mass with necrosis and hemorrhage is the characteristic finding of<unk> . On microscopic histopathological analysis, spindle cells, nuclear atypia, mitoses, and herring bone pattern are characteristic findings of<unk> . Neurofibromatosis type I (NF1) gene is involved in the pathogenesis of<unk> .<unk>  must be differentiated from neurofibroma and schwannoma. The incidence of<unk>  is approximately 0.1 per 100,000 individuals worldwide. The incidence of<unk>  increases with age.<unk>  affects men and women equally. Common risk factors in the development of<unk>  are pre-existing plexiform neurofibromas, pre-existing perineuriomas, radiation therapy, and neurofibromatosis type 1. Symptoms of<unk>  include<unk>  edema, difficulty in moving the extremity, pain, and numbness. MRI may be diagnostic of<unk> . Findings on MRI suggestive of<unk>  include isointense on T1 weighted image and low signal on T2 weighted image. The predominant therapy for<unk>  is surgical resection. Adjunctive chemotherapy and radiation therapy may be required. Common complication of<unk>  is metastasis. Prognosis is generally poor.
Malignant peripheral nerve sheath tumor
Nerve sheath tumor
Neuroma
Primary central nervous system lymphoma
Choroid plexus tumor
Glomus tumor
Primitive neuroectodermal tumor
Schwannoma