Malignant peripheral nerve sheath tumor of lower lip.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, highly aggressive tumors capable of arising de novo or from preexisting benign neurofibromas or schwannomas. The treatment of choice is surgery. Trunk and extremities are the commonest sites involved. Malignant peripheral nerve sheath tumors are rarely encountered in head and neck region. Here, we report a patient with MPNST of lower lip who had neither a family history nor stigmata of neurofibromatosis.