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The malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue sarcoma of ectomesenchyme. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of this disease is known to arise in the seventh decades. In human body, trunk and extremities are the most commonly involved site, and only 8-14% of all lesions appears in head and neck region. Because of its non-specific clinical and histopathological presentation, the diagnosis of MPNST has been described as one of the most difficult diagnosis. But this difficulty of diagnosis was solved by the use of immunohistochemistry. The authors report a case of malignant peripheral nerve sheath tumor in nasal cavity with a review of the literature. (Korean J Otorhinolaryngol-Head Neck Surg 2008;51:376-9)
