Malignant peripheral nerve sheath tumors of the head and neck: A clinicopathological study

Abstract

Twenty-seven cases of malignant peripheral nerve sheath tumors involving the head and neck region over a period of 7 years were reviewed. They were graded from 1-3 based on necrosis, mitosis, cellularity, and pleomorphism. Mean age of occurrence was 42 years, with a range of 12-70 years. Male preponderance was noted (M:F = 3.5:1). The most common site of involvement was the neck (44.6%). The main presenting symptom was an enlarging mass. The nerve of origin could be identified in 33% of patients. Treatment consisted of wide excision. The 5-year observed survival was 33%. Fifty-two percent of patients developed local recurrence of disease. Fifteen percent of patients died due to advanced local disease within 18 months of treatment. Distant metastasis was seen in 18.5% of patients. Lymph node metastasis was not seen. At the end of 5 years 15% of patients remained disease free. Large tumor size (> 5 cm) adversely affected the prognosis (P = < 0.1). No significant correlation was noted between the grade of tumor and survival.

Fine Needle Aspiration Cytology of a Primary Malignant Peripheral Nerve Sheath Tumor Arising in the Parotid Gland

“…1 Approximately 15% of all MPNSTs occur in the head and neck area, arising from the nerve sheath of cranial nerves. [2][3][4][5] Primary location in the parotid gland is rare. [2][3][4][5][6][7] Vege et al, 5 in a retrospective analysis of 27 cases of MPNSTs in the head and neck area, found 2 in the parotid region.…”

Recurrent giant malignant peripheral nerve sheath tumor of the scalp with intracranial extension

“…Local recurrences have been reported to vary from 52 to 88.9% for different sites, whereas metastasis (mainly in the lungs and liver) ranged from 11.1 to 18%. The 5-year survival rate among patients with MPNSTs ranges from 30 to 50% (Angelov et al, 1998;Ferner and Gutmann., 2002;Vege et al, 1994). In this case, it recurred locally and there was no metastatic lesion elsewhere in the body.…”

“…The International Consensus Group has recommended that the current management (Angelov et al, 1998;Vege et al, 1994). Local recurrences have been reported to vary from 52 to 88.9% for different sites, whereas metastasis (mainly in the lungs and liver) ranged from 11.1 to 18%.…”

Schwannoma of the Sinonasal Tract and the Pterygopalatine Fossa with or without Intracranial Extension

“…He received chemotherapy; however, he developed severe pneumonia and heart failure and deceased. In the overwhelming majority of cases, sinonasal schwannomas do not metastasize [21,22]. Complete ablation of the tumor in this patient at that time was difficult because of the extension of the tumor into surrounding vital structures.…”