부비동에서 발생한 악성 말초 신경초종 1예

Abstract

Malignant peripheral nerve sheath tumor, a highly aggressive neoplasm of nerve sheath origin (also known as malignant schwannoma, malignant neurilemoma), is an uncommon entity that may occasionally occur in the head and neck. In the majority of cases, malignant peripheral nerve sheath tumors are found in the extremities, followed by the occurrence in the paravertebral region of the thorax and abdomen. The head and neck are involved in less than 10% of cases, which is especially true in rare diseases of the nasal cavity and paranasal sinuses. We present a case of malignant peripheral nerve sheath tumor of the paranasal sinus without a definite evidence of von Recklinghausen’s disease or benign preexisting peripheral nerve sheath tumor with a review of literatures.