Malignant peripheral nerve sheath tumor arising from solitary neurofibroma

Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas that are strongly associated with neurofibromatosis type I (NF-1). We describe a 71-year-old woman with no stigmata of neurofibromatosis, who presented with recurrent subcutaneous tumor on her left upper back. She received two excisional biopsies on the back of her trunk at our hospital and both pathology reports revealed neurofibromas. Three years after the last skin biopsy, a rapidly growing subcutaneous tumor emerged at the same site. This tumor was totally resected and the histopathology showed an ill-defined tumor in the dermis and subcutaneous tissue. The tumor was composed of spindle cells in a myxoid stroma with a transition from the area of typical neurofibroma to the hypercellular area. The hypercellular area consisted of atypical, hyperchromatic spindled cells with frequent mitotic figures. She was therefore diagnosed with MPNST.