Large Cervical Vagus Nerve Tumor in a Patient with Neurofibromatosis Type 1 Treated with Gross Total Resection: Case Report and Review of the Literature

Abstract

Neurofibromas are benign peripheral nerve sheath tumors that occur commonly in individuals with neurocutaneous disorders such as neurofibromatosis type 1. Vagal nerve neurofibromas, however, are a relatively rare occurrence. We present the case of a 22-year-old man with neurofibromatosis type 1 with a neurofibroma of the left cervical vagal nerve. The mass was resected through an anterior approach without major event. In the postoperative course, the patient developed left vocal cord paralysis treated with medialization with injectable gel. We then present a comprehensive review of the literature for surgical resection of vagal nerve neurofibromas.

Epidemiology and Estimated Cost of Microsurgical Treatment of Peripheral Nerve Tumor Conducted by the Brazilian Unified Health Care System (2008–2016)

“…8 Neurofibromas arise from Schwann cells and are also composed of a variety of other cells, including neuronal axons, fibroblasts, mast cells, macrophages, perineural cells, and extracellular matrix materials, such as collagen. 5,8,9 They are classified into localized or solitary, presenting in plexiform or diffuse forms, 8,9 with localized being the most common subtype. 4,8 The tumor tissue is closely associated with the primary nerve and is inseparable from the normal nerve.…”

“…4,8 They occur commonly in pictures of neurocutaneous disorders, such as neurofibromatosis type 1, and 25% of the symptomatic neurofibromas affect the head and neck region. 5 They are commonly referred to as painless, well-defined, fusiform or round, slowgrowing masses < 5 cm in diameter. 4,8 Histologically, they are composed of elongated cells mixed with undulating bundles of collagen and/or of mucin.…”

“…2 In case of involvement of the vagus nerve, they can cause dysphagia. 5 Even with a positive sign, paresthesias and transverse mobility, the clinical identification is somewhat subjective and, for this reason, nerve tumors are often misdiagnosed because of similarities with other soft tissue tumors such as lipomas, fibromas, ganglions, or xanthomas. 2 Although neurogenic tumors can be diagnosed on magnetic resonance imaging (MRI), it is difficult to differentiate between schwannomas and neurofibromas.…”

“…These regions may have some influence in terms of the technique and approach involved in the surgical treatment and of the prognosis. 1,5 The objective of the present study is to describe the epidemiological data regarding the number of annual procedures, hospital and professional expenses, the mean length of hospital stay, and the number of deaths of patients admitted by the Brazilian Unified Health System (SUS, in the Portuguese acronym), in the period from 2008 to 2016, using the code of microsurgical treatment of peripheral nerve tumor/neuroma. The present study will also evaluate the impact of inflation on professional spending during the period studied.…”

Neurofibroma of the cervical part of the vagus nerve: A case report

“…Neurofibroma of the vagus nerve in the cervical region is an extremely uncommon benign tumor [1]. Only ten reported cases were found in literature [2].…”