A case of malignant peripheral nerve sheath tumor with rapid progress

We report a case of malignant peripheral nerve sheath tumor (MPNST) of the nape. A 52-year-old woman suffering from neurofibromatosis type I since her childhood noticed a tumor on the nape six years ago. The tumor gradually became enlarged and elevated. On her visit to our hospital on July 25th, 2002, physical examination revealed an elastic soft, 15×13×6cm sized tumor on the left side of her nape. The tumor grew rapidly and ruptured with bleeding on August 23rd. Immediate excision of the tumor was performed. Histologically, the tumor mainly consisted of spindle cells showing palisading arrangement.A part of the specimen showed nuclear atypia and mitoses. Immunohistochemically, the spindle cells were positive for S-100 protein and NSE. The tumor was diagnosed as MPNST. Multiple lung and liver metastases developed and the patient died of respiratory failure caused by lung metastases in 5 months after her first visit to our institution. [Skin Cancer (Japan) 2004; 19: 229-232]