Cervical Segment Nervus Vagus Neurofibroma with Associated Neurofibromatosis Type 1: A Case Report

Abstract

Introduction: 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.

Case Report: 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.

Discussion: 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

Key words

nerve sheath tumors - neurofibroma - von Recklinghausen's disease

References

• 1 Marocchio LS, Oliveira DT, Pereira MC. et al . Sporadic and multiple neurofibromas in the head and neck region: a retrospective study of 33 years.  Clin Oral Investig. 2007 Feb 7; 
• 2 Galli J, Almadori G, Paludetti G. et al . Plexiform neurofibroma of the cervical portion of the vagus nerve.  J Laryngol Otol. 1992;  106 643-648
• 3 Chang SC, Schi YM. Neurilemmoma of the vagus nerve. A case report and brief literature review.  Laryngoscope. 1984;  94 946-949
• 4 St Pierre S, Theriault R, Leclerc JE. Schwannomas of the vagus nerve in the head and neck.  J Otolaryngol. 1985;  14 167-170
• 5 Kline DG, Hudson AR. (eds): Tumors involving nevre, in Nevre injuries: Operative Results for Major Nevre Injuries, Entrapments, and Tumors. Philadelphia, W. B. Saunders Co. 1995: pp 525-574
• 6 Parizel PM, Martin JJ, Van Vyve M. et al . Cerebral ganglioglioma and neurofibromatosis type I. Case report and review of the literature.  Neuroradiology. 1991;  33 357-359
• 7 Oliver DW, Wells FC, Lamberty BG. et al . Massive plexiform neurofibroma of the sympathetic trunk.  Eur J Cardiothorac Surg. 1999;  16 569-572
• 8 Fujino K, Shinohara K, Aoki M. et al . Intracapsular enucleation of vagus nerve-originated tumors for preservation of neural function.  Otolaryngol Head Neck Surg. 2000;  123 334-336
• 9 Gilmer-Hill HS, Kline DG. Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature.  Neurosurgery. 2000;  46 1498-1503
• 10 Donner TR, Voorhies RM, Kline DG. Neural sheath tumors of major nerves.  J Neurosurg. 1994;  81 362-373
• 11 Green  Jr  JD, Olsen KD, DeSanto LW. et al . Neoplasms of the vagus nerve.  Laryngoscope. 1988;  98 648-654
• 12 Hussain SS, Watson MG, Pahor AL. Neurilemmoma of the vagus nerve in the neck.  Ear Nose Throat J. 1989;  68 52-56
• 13 Mukerjee DK. Neurilemmoma of the vagus nevre.  J Laryngol Otol. 1979;  93 187-192
• 14 Sawamura Y, de Tribolet N. Vagal schwannoma associated with syncopal attacks and postural hypotension: a case report.  Neurosurgery. 1990;  27 461-463
• 15 Yumoto E, Nakamura K, Mori T. et al . Parapharyngeal vagal neurilemmoma extending to the jugular foramen.  J Laryngol Otol. 1996;  110 485-489
• 16 Furukawa M, Furukawa MK, Katoh K. et al . Differentiation between schwannoma of the vagus nerve and schwannoma of the cervical sympathetic chain by imaging diagnosis.  Laryngoscope. 1996;  106 1548-1552
• 17 Kawamura Y, Sze G. Totally cystic schwannoma of the tenth cranial nerve mimicking an epidermoid.  AJNR Am J Neuroradiol. 1992;  13 1333-1334
• 18 Leach J, Williams N. Pathologic quiz case 1. Vagal neurilemoma.  Arch Otolaryngol Head Neck Surg. 199;  118 338-340
• 19 Wood BM, McNeil WT. Schwannoma of the vagus nerve.  Anaesthesia. 1986;  41 1130-1132

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