Abstract: A 72-year-old male presented with tumor on the left lower limb for more than 10 years and aggravated for 1 year. Immunohistochemical examination after local surgery showed CD34(-), CD31(-), CD117(-), NF(-), Desmin(-), SMA(-), P53(-), CK-Pan(-), Ki-67, more than 40%), S-100(+). Combined with family hitory and clinical manifestation, the diagnosis of malignant peripheral nerve sheath tumors with neurofibromatosis type 1 was macle.

Key words: malignant peripheral nerve sheath tumors, neurofibromatosis type 1