Abstract

Results: Malignant triton tumor is a subtype of malignant schwannoma with rhabdomyoblastic differentiation. A total of 16 cases have been reported so far in the sinonasal tract localization, and less than 200 in all localizations. Unlike other localizations, the sinonasal tract is not associated with neurofibromatosis type 1. The mean age of presentation is 61 years (42 years for head and neck, younger than 35 years for NF-1). Eight of the 16 patients were reported alive at 5 years, with a mean follow-up of 46 months (1.5–84 months). Radical resection is the mainstay of treatment, and radiotherapy is not associated with increased survival.

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