Malignant peripheral nerve sheath tumor (MPNST), which originates in Schwann cells, is uncommon in the head and neck. These highly aggressive sarcomas are characterized by local recurrence and distant metastasis. Many are associated with neurofibromatosis type 1; the survival rate in such cases is poorer than the rate associated with tumors that arise de novo. Hypercellularity and pleomorphism are cardinal microscopic features. Immunohistochemical staining is widely used to determine S-100 positivity and a Ki-67 score, either of which may establish the prognosis. We present a case of MPNST of the neck that arose in a 17-year-old girl, who was also found to have a parapharyngeal neurofibroma. The cervical mass was excised (intraoperative complications precluded removal of the parapharyngeal mass) and radiotherapy was administered, but the patient experienced a recurrence within 2 months. Another surgery was performed, and this time both tumors were removed. At 1 year of follow-up, the patient was without disease, but in postoperative year 2, she died from complications of a cervical recurrence and mediastinal metastasis.
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