Early View Article: Online published version of an accepted article before publication in the final form.

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1 : Online published version of an accepted article before publication in the final form. Journal Name: International Journal of Case Reports and Images (IJCRI) Type of Article: Case Report Title: Neurofibroma of the cervical part of the vagus nerve: A case report Authors: Samarasinghe A.S, Chathuranga L.S, Niyas S.M.M, Sugathadasa W.D.P doi: To be assigned Early view version published: June 16, 2017 How to cite the article: Samarasinghe A.S, Chathuranga L.S, Niyas S.M.M, Sugathadasa W.D.P. Neurofibroma of the cervical part of the vagus nerve: A case report. International Journal of Case Reports and Images (IJCRI). Forthcoming Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the. The is an online published version of an accepted article before publication in the final form. The proof of this manuscript will be sent to the authors for corrections after which this manuscript will undergo content check, copyediting/proofreading and content formatting to conform to journal s requirements. Please note that during the above publication processes errors in content or presentation may be discovered which will be rectified during manuscript processing. These errors may affect the contents of this manuscript and final published version of this manuscript may be extensively different in content and layout than this. Page 1 of 10

2 TYPE OF ARTICLE: Case Report TITLE: Neurofibroma of the cervical part of the vagus nerve: A case report AUTHORS: Samarasinghe A.S 1, Chathuranga L.S 1, Niyas S.M.M 1, Sugathadasa W.D.P 1 AFFILIATIONS: 1 Department of Surgery, Teaching Hospital Kandy, Sri Lanka CORRESPONDING AUTHOR DETAILS Achala Samarasinghe Department of Surgery, Teaching Hospital Kandy, Sri Lanka achalasamarasinghe@gmail.com Short Running Title: NOT GIVEN Guarantor of Submission: The corresponding author is the guarantor of submission Page 2 of 10

3 TITLE: Neurofibroma of the cervical part of the vagus nerve: A case report ABSTRACT Introduction Neurofibroma of the vagus nerve in the cervical region is an extremely uncommon benign tumour. Only ten reported cases were found in the literature. It is slow growing and most are asymptomatic neck lumps ]. Preoperative imaging aids in arriving at a differential diagnosis and planning the surgery. Complete surgical excision is the standard of care. Case Report A 39 year old female presented to the General Surgical clinic with a painless lump on the Right side of the neck for twelve months duration with recent rapid enlargement. Past medical history was unremarkable. She did not have a family history of neurofibromatosis. Examination revealed a 4 x 3.5cm lump in the upper neck with well-defined margins and smooth surface. Contrast enhanced CT scan confirmed a 3.6 x3.2 x6.9cm hypodence mass with no contrast enhancement. The lesion was in-between Right internal and external carotid artery and extending to the base of the skull. Right internal jugular vein (R/IJV) was displaced anteriorly. Right common carotid artery(r/cca) was stretched. The patient underwent exploration of the neck under general anaesthesia with loupe magnification. The gross pathology showed a greyish homogenous cut surface of a 55x40mm tumour. Pathological examination confirmed the diagnosis of neurofibroma of the vagus nerve. Conclusion Vagus nerve neurofibromas are rare nerve sheath tumours. Gold-standard treatment of symptomatic benign vagus nerve mass is an attempt at GTR with minimal loss of nerve function. Keywords: Neurofibroma, vagus nerve, tumour Page 3 of 10

4 TITLE: Neurofibroma of the cervical part of the vagus nerve, A case report INTRODUCTION Neurofibroma of the vagus nerve in the cervical region is an extremely uncommon benign tumour [1]. Only ten reported cases were found in the literature [2]. It is slow growing and most are asymptomatic neck lumps [3]. Preoperative imaging aids in arriving at a differential diagnosis and planning the surgery. Complete surgical excision is the standard of care. Here we present a case of sporadic neurofibroma of the vagus nerve in the cervical region of a 39 year old lady. She underwent successful surgery without disability such as vocal cord palsy. CASE REPORT A 39 year old female presented to the General Surgical clinic with a painless lump on the Right side of the neck for twelve months duration with recent rapid enlargement. Past medical history was unremarkable. She did not have a family history of neurofibromatosis. Examination revealed a 4 x 3.5cm lump in the upper neck with well-defined margins and smooth surface. Upon palpation paroxysmal cough was not elicited. Ultrasound (US) of the neck showed a well-defined solid soft tissue mass with internal vascularity. Contrast enhanced CT scan confirmed a 3.6 x3.2 x6.9cm hypodence mass with no contrast enhancement. The lesion was in-between Right internal and external carotid artery and extending to the base of the skull. Right internal jugular vein (R/IJV) was displaced anteriorly. Right common carotid artery(r/cca) was stretched (Figure 1). The FNAC of the mass was inconclusive showing only stromal tissue fragments. The patient underwent exploration of the neck under general anaesthesia with loupe magnification. A vertical incision is made along the anterior border of the sternocleidomastoid muscle achieving vascular control. An ovoid whitish tumour was identified in between the R/IJV and the carotid artery displacing them. Both the superior and inferior ends of the mass appeared in continuity with the vagus nerve stretching its nerve fibres. Plane of the tumour was approached splitting the nerve Page 4 of 10

5 fibres in the longitudinal direction. Tumour was completely excised preserving the continuity of the vagus nerve (Figure 2). The gross pathology showed a greyish homogenous cut surface of a 55x50mm tumour. Pathological examination confirmed the diagnosis of neurofibroma of the vagus nerve. Microscopy showed loosely arranged spindle cells containing slender wavy nuclei with pointed edges. Scattered cells showed enlarged hyperchromatic nuclei resembling degeneration. Mitoses were not increased (Figure 1). Patient recovered well without hoarseness and discharged home in the second postoperative day. She was followed-up in the clinic with no major complications. DISCUSSION Neurofibromas of the vagus nerve are an extremely rare peripheral nerve sheath tumour. There occurrence in the cervical region is even uncommon. There are only ten reported cases of such lesions found in the literature [2]. Majority of neoplasms of vagus nerve are schwannomas [4,5]. Neurofibromas of the vagus nerve are slow growing peripheral nerve sheath tumours [3,6]. Generally they are asymptomatic. Patients present with a painless neck lump with no significant disability. Some case studies have documented dysphagia and cough with percussion of the mass [4]. Commonest age range is years with no sex predisposition [12]. There may be an association (60%) with neurofibromatosis type 1 [8, 2]. Growth takes the fusiform shape surrounded by a pseudo-capsule with the stretched neural tissue. Most are benign with a malignant counterpart less frequently. Preoperative imaging with MRI is preferred, to assess the tumour characteristics and extension and arriving at a differential diagnosis. Ultrasound scan and CT are also valuable imaging modalities especially in limited resource setting. Optimal surgical approach and planning of surgery is made according the findings. Cytological assessment with FNAC is non-specific. Biopsy is discouraged due to risk of damaging the surrounding structures, bleeding and because of its effects on future definitive surgery. Page 5 of 10

6 Gross total resection is the definitive treatment (GTR) [5]. Neurofibromas prove to be most difficult nerve sheath tumours to achieve GTR. Removal of the tumour by internal decompression is preferred as it preserves the function and minimizes the damage to closely packed structures in the vicinity [10]. If the neurofibroma is removed enbloc, microsurgical repair of the nerve is indicated with a nerve graft [13]. Following the excision complications such as vocal cord palsy, dysphagia and arrhythmia could occur. Donner et al previously reported a 16% decrease in motor function using the intra-capsular enucleation technique. Among them Up to 10% developed pain syndromes [8]. Green et al reported a post-operative complications like occurrence of dysphagia (40%) and aspiration (46%) after vagus nerve tumour surgery [7]. Gilmer-Hill and Kline stated that nearly all patients who have vagus nerve tumour resections will develop transient hoarseness postoperatively [14]. Our patient did not develop any of these major complications. Patients should be followed-up for local recurrence because of it s a higher possibility [12]. CONCLUSION Vagus nerve neurofibromas are rare nerve sheath tumours. Gold-standard treatment of symptomatic benign vagus nerve mass is an attempt at GTR with minimal loss of nerve function. Postoperative hoarseness and vocal cord paralysis can be avoided with meticulous surgical technique. CONFLICT OF INTEREST None of the authors have any conflicts of interest AUTHOR S CONTRIBUTIONS Samarasinghe A.S Group 1 - Substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data Group 2 Drafting the article Group 3 - Final approval of version to be published 158 Page 6 of 10

7 Chathuranga L.S, Group 1 - Substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data Group 2 Drafting the article Group 3 - Final approval of version to be published Niyas S.M.M Group 1 - Substantial contributions to conception and design, acquisition of data, Group 2 Drafting the article Group 3 - Final approval of version to be published Sugathadasa W.D.P Group 1 - Substantial contributions to conception and design, acquisition of data, Group 2 Drafting the article Group 3 - Final approval of version to be published REFERENCES 1. David P, Andrew K, Chan, Large Cervical Vagus Nerve Tumor in a Patient with Neurofibromatosis Type 1 Treated with Gross Total Resection: Case Report and Review of the Literature, J Brachial Plex Peripher Nerve Inj. 2016; 11(1):e Matejeik V, Steno J. Neurofibroma of the vagus nerve in the cervical portion. Bratisl lek listy 2008;109(10) Kumar V, Abbas A K, Aster J C, Robbins S L. Philadelphia, PA: Elsevier/Saunders; Robbins Basic Pathology. 9th ed. 4. Packer R J, Gutmann D H, Rubenstein A. et al. Plexiform neurofibromas in NF1: toward biologic-based therapy. Neurology. 2002; 58(10): Friedman J M. Epidemiology of neurofibromatosis type 1. Am J Med Genet. 1999;89(1):1 6. McClatchey A I. Neurofibromatosis. Annu Rev Pathol. 2007; 2: Page 7 of 10

8 Marocchio L S, Oliveira D T, Pereira M C, Soares C T, Fleury R N. Sporadic and multiple neurofibromas in the head and neck region: a retrospective study of 33 years. Clin Oral Investig. 2007; 11(2): Green J D Jr, Olsen K D, DeSanto L W, Scheithauer B W. Neoplasms of the vagus nerve. Laryngoscope. 1988; 98(6, Pt 1): Donner T R, Voorhies R M, Kline D G. Neural sheath tumors of major nerves. J Neurosurg. 1994; 81(3): Chang S C, Schi Y M. Neurilemmoma of the vagus nerve. A case report and brief literature review. Laryngoscope. 1984; 94(7): St Pierre S, Theriault R, Leclerc J E. Schwannomas of the vagus nerve in the head and neck. J Otolaryngol. 1985; 14(3): Hruban R H, Shiu M H, Senie R T, Woodruff J M. Malignant peripheral nerve sheath tumors of the buttock and lower extremity. A study of 43 cases. Cancer. 1990; 66(6): Kline D G, Judice D J. Operative management of selected brachial plexus lesions. J Neurosurg. 1983; 58(5): Gilmer-Hill H S, Kline D G. Neurogenic tumors of the cervical vagus nerve: report of four cases and review of the literature. Neurosurgery. 2000;46(6): FIGURE LEGENDS Figure 1: CT view of right sided hypodence mass with no contrast enhancement Figure 2: Ovoid tumour in-between the R/IJV and the carotid artery Figure 3: Loosely arranged spindle cells Page 8 of 10

9 221 FIGURES Figure 1: CT view of right sided hypodence mass with no contrast enhancement Figure 2: Ovoid tumour in-between the R/IJV and the carotid artery Page 9 of 10

10 Figure 3: Loosely arranged spindle cells Page 10 of 10