Malignant peripheral nerve sheath tumor of the parotid gland.

Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, usually having a poor prognosis, and only a few cases been described in the literature. In this article, we report the diagnostic and therapeutic challenges related to a new case of MPNST of the parotid. Diagnosis was made based on clinical, imaging (computed tomography scan), histologic, and immunohistochemistry findings. Despite comprehensive treatment--complete surgical resection and radiotherapy--the tumor displayed a highly aggressive course.