Abstract
Neurofibromatosis type 1 （NF-1） is an autosomal dominant inherited disease, in which approximately 3-5％ of patients develop malignant peripheral nerve sheath tumor （MPNST）. In this report, we present a case of MPNST associated with NF-1. The patient was a 26-year-old man presenting with a lump on his right neck. Based on his café-au-lait spots and family history, neurofibroma associated with NF-1 was suspected. The tumor showed rapid growth and pain, and so the patient underwent surgical resection. The final histopathological diagnosis was MPNST. The surgical margin was close, and postoperative radiation therapy was administered. MPNST is a disease with a poor prognosis due to frequent local recurrence and distant metastasis. Although surgical resection for MPNST carries the risk of neurological deficit, early diagnosis and treatment including surgery are important.
