Malignant schwannoma with rhabdomyoblastic differentiation (Triton tumor) is a very rare variant of the malignant tumors arising in the peripheral nerve sheath. In 70% of the cases, the neoplasm is associated with Von Recklinghausen's neurofibromatosis; in the remaining 30% it is the sole morbid finding. Its biological behaviour is, in any case, more aggressive than that of simple malignant schwannoma; its course is rapid and its prognosis is dismal. Local recurrences are frequent, and distant metastases, preferentially situated in the lung and brain, are present in about 50% of the cases. Post-operative radiotherapy is always indicated. We report a case of a 64-year-old man who presented a right paralatero-nasal Triton tumor. Following exeresis, a cycle of high-dose radiotherapy was prescribed; the 10-year follow-up revealed no sign of recurrence or distant metastases.
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