Cervical segment nervus vagus neurofibroma with associated neurofibromatosis type 1: a case report

Neurofibroma occurs as an isolated or multiple lesions frequently associated with neurofibromatosis type 1 (NF-1). Neurofibroma is a benign peripheral nerve sheath tumor composed of a variable mixture of Schwann, perineurial-like, and fibroblastic cells, as well as ones with features intermediate between these various cells, immersed in a collagenous or myxoid matrix.

A 10-year-old boy visited the outpatient clinic with complaints of swelling and pain on the left side of his neck, which has been present for a year. A mass was discovered adjacent to the left internal carotid artery (ICA) and was removed by a microsurgical technique. No postoperative neurological deficit was identified and the pathological diagnosis was once more reported as neurofibroma.

Nerve sheath tumors may be observed in patients with von Recklinghausen's disease, although vagal neurofibromas are noted more frequently than schwannomas among these patients. For unknown reasons, neoplasms associated with von Recklinghausen's disease occur more frequently on the right side. Cervical vagal neurofibromas most often present as asymptomatic, slowly enlarging, lateral neck masses. When symptoms are observed, hoarseness is the most common. Dysphonia, dyspnea, dysphagia, cough, syncopal episodes, tongue weakness and atrophy, and Horner's syndrome have also been reported as presenting complaints.