Neurogenic tumors of the cervical vagus nerve: Report of four cases and review of the literature

OBJECTIVE AND IMPORTANCE: Nerve sheath tumors arising from the cervical vagus nerve are extremely rare. These tumors most often present as asymptomatic, slowly enlarging, lateral neck masses and therefore often come initially to the attention of otolaryngologists and general surgeons. Because they are nerve tumors, however, neurosurgeons must be able to recognize and treat these rare entities. We report three cases of schwannoma and one case of neurofibroma of the cervical vagus nerve that were encountered at our center (Louisiana State University Medical Center) during a 31-year period. CLINICAL PRESENTATION: The patients ranged from 31 to 61 years of age at the time of presentation to Louisiana State University Medical Center. Presenting complaints included hoarseness, Horner's syndrome, and palpation of an enlarging, asymptomatic, cervical mass. Reviews of systems revealed episodes of aspiration for one patient and frequent respiratory illnesses for two patients. These episodes were possibly related to their tumors. Imaging studies demonstrated well-circumscribed masses in the region of the carotid sheath. INTERVENTION: Using microsurgical techniques, gross total resection of all four tumors was accomplished. For one patient, the vagus nerve needed to be divided and an end-to-end anastomosis was performed. For the other three patients, resection of the tumor was achieved with the vagus nerve in continuity. CONCLUSION: Vagal nerve schwannomas and neurofibromas in the neck are rare neoplasms. We present four cases of these benign tumors. The pathological features, epidemiological characteristics, presentation, differential diagnosis, and management are discussed. Cross total resection with preservation of the vagus nerve remains the treatment of choice.