Multidisciplinary Approach to Malignant Peripheral Nerve Sheath Tumor of the Trigeminal Nerve: 2-Dimensional Operative Video

Malignant peripheral nerve sheath tumors (MPNSTs) of the trigeminal nerve are uncommon lesions that pose a surgical challenge to neurosurgeons. The case described in this video involved a 67-yr-old man who presented with a 2-yr history of left-sided facial numbness spreading from his chin along the left mandible to the preauricular area. He also reported left-sided tongue numbness and decreased taste on the left side of the tongue. On examination, he had left-sided facial numbness along the V3 distribution to pinprick and light touch. Magnetic resonance imaging (MRI) revealed an enhancing left V3 lesion extending from Meckel's cave to the angle of the mandible. The patient underwent a left temporal craniotomy for biopsy of the lesion, formalizing the diagnosis of a MPNST of the left trigeminal nerve. A multidisciplinary resection of his lesion was performed. Left infratemporal fossa approach with neck dissection, mandibulectomy, and frontotemporal craniotomy were performed. Additionally, a frontal external ventricular drain was placed for 3 d to aid in CSF diversion to avoid CSF leak, and free-flap reconstruction was undertaken. The patient tolerated the procedure well. Postoperatively, he retained his facial numbness, dysphagia, and dysarthria. The patient was discharged to inpatient rehabilitation on postoperative day 12. Postoperative computed tomography and MRI depicted complete resection of the left trigeminal nerve MPNST. At his last follow-up appointment, 3 mo after surgery, the patient reported significant improvement in his symptoms. Neuroimaging demonstrated no residual tumor and adjuvant radiotherapy was recommended. The patient provided consent for publication.