Auris, nasus, larynx
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Auris, nasus, larynx · Oct 2010
Review Case ReportsMalignant peripheral nerve sheath tumor of intracranial nerve: a case series review.
The incidence of malignant peripheral nerve sheath tumor (MPNST) is approximately 0.001%. Those involving intracranial nerves are even more exceptional. Little information is available concerning work up and management. Our objective is: (1) to review all cases of intracranial MPNST described in the literature, (2) to highlight the suspicion of intracranial MPNST, (3) to identify the gross pathology, the histopathology, the immunohistochemistry, (4) to discuss the differential diagnosis, the treatment, the recurrence rate, the follow-up, the incidence of metastasis and the prognosis. ⋯ MPNST of cranial nerves are very rare. In neurofibroma, even though MPNST is mainly associated to type 1, we should keep in mind its association to NF2. Mainstay of treatment is radical resection with adjuvant radiotherapy. Inaccessibility of cranial MPNST may explain the subtotal resection and thus the poor prognosis. Metastasis to the spinal cord is the most frequent one. A close postoperative follow-up is mandatory to eliminate recurrence.