Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review.

BACKGROUND

This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck.

METHODS

From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST. We retrospectively reviewed presenting symptoms, radiological findings, surgical management, and follow-up status and performed a literature review.

RESULTS

Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation. Two lesions were growing within the sinonasal tract. The most common presenting symptom was a rapidly enlarging cervical mass. Seventy percent of the tumors could be resected completely. Long-term follow-up showed a 2-year disease-specific survival rate of 50% and 5-year survival rate of 20%. Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease. Postoperative adjuvant radiotherapy was found to make no difference in outcome.

CONCLUSIONS

Although rare, MPNST is one of the most aggressive tumors in the head and neck area. Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST. Adjuvant radiotherapy should be used to assist surgical excision in local control. The role of adjuvant chemotherapy remains controversial.