[A case of malignant peripheral nerve sheath tumor originating from the cervical vagus nerve]

A 45-year-old female presented a peripheral nerve sheath tumor (MPNST), which had progressed to malignancy from a neurofibroma arising in the left cervical vagus nerve. Neuroimaging showed that a tumor grew in the left neck area of the patient, involving the internal carotid and vertebral arteries, and invading the intracranium through the left jugular foramen. The tumor was extensively removed using a combination of the lateral suboccipital approach and the neck incision, and the frontotemporal approach accompanied with EC-IC highflow bypass and EC-MCA anastomosis. Postoperatively, pathological features demonstrated a MPNST arising from the cervical vagus nerve. Here, we discuss the clinical behavior of MPNST by quoting previous reports, and conclude by emphasizing 2 points in the treatment of MPNST; 1) that prognosis of patients with MPNST is greatly affected by the time between presenting symptoms and diagnosis, and 2) that it is very important to carefully observe neurofibroma patients, because there is a possibility of progression to malignant MPNST.