Sporadic high-grade malignant peripheral nerve sheath tumor of the hypoglossal nerve

Malignant tumors of peripheral nerve sheaths (MPNSTs) are rare malignant soft tissue tumors arising either from a peripheral nerve or from a pre-existing benign nerve sheath tumor. They occur most often in the context of Neurofibromatosis type-1 (NF-1) and are characterized by poor prognosis and aggressive behavior with a high rate of recurrence and distant metastases. We describe a 50-year-old woman who presented with right neck swelling, progressive dysphagia and tongue paresis. Imaging analysis revealed a mass involving the right parapharyngeal space. The tumor was excissed through a transcervical approach. At surgery, the tumor was strictly adherent to the hypoglossal nerve. Pathologic evaluation of the mass revealed a high-grade MPNST. Based on the pathological diagnosis, a clinical work-up for NF-1 was performed but it resulted negative. Occurrence of sporadic high-grade MPNST in the parapharyngeal space is rare and development from the hypoglossal nerve exceptional. As far as we know, only in two cases, both with clinical features consistent with NF1, the tumor was reported to be arised from the hypoglossal nerve.