Malignant peripheral nerve sheath tumor of the parotid gland.

Malignant peripheral nerve sheath tumor (MPNST) has been defined as any malignant tumor arising from or differentiating toward cells of the peripheral nerve sheath. We treated a case of MPNST arising from the right parotid gland that showed a highly aggressive course. We reviewed the English-language literature published since 1990 and found 142 cases of head and neck MPNST reported within the past 13 years. The results of the review suggested that MPNSTs may arise from any organs of the head and neck. Immunohistochemical analysis of various neural markers plays a significant role in the evaluation of the histologic diagnosis. Curative treatment based on radical resection of MPNSTs of head and neck origin is more difficult than treatment of MPNSTs of other origins.