cervical vagal schwannoma patient post op portal

What is the pathophysiology of cervical vagal schwannoma?

Schwannoma originating from the cervical vagus nerve is an extremely rare neoplasm. Vagal nerve schwannoma usually occurs between the third and fifth decades of life, it does not show sex predilection both sexes being equally affected and it most often presents as a painless, slow-growing, lateral neck mass.

What do we know about vagal schwannomatosis?

A schwannoma, also known as an acoustic neuroma, is a benign nerve sheath tumor composed of schwann cells, which normally produce the insulating myelin sheath covering the peripheral nerves. Schwannoma, originating from the cervical vagus nerve, is an extremely rare neoplasm that usually occurs in men between the 3rd and 6th decades of life.

What is the standard of care for vagal schwannoma?

May 05, 2013 · Schwannoma originating from the cervical vagus nerve is an extremely rare neoplasm. They usually occur between the third and fifth decades of life, with no definite sex predilection. The pre-operative diagnosis of schwannoma is a clinical challenge and is …

Is intermittent nerve mapping the future standard of care for vagal schwannoma?

ASTRAT Schwannomas arising from the cervical vagus nerve are extremely rare benign neurogenic tumours. They usually occur in ages between third and sixth decades, affect both sexes equally and most often present as painless, slow-growing, lateral neck masses. The treat- ment of choice is the complete surgical exci- sion.

Abstract

Cervical vagal schwannoma is a benign, slow-growing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in general population, but diagnosis is still a challenge. Surgical resection is the treatment of choice even if its close relationship with nerve fibres, from which it arises, threats vagal nerve preservation.

Conclusions

Schwannoma arising from the cervical vagus is a rare, benign pathology usually giving unspecific symptoms and requiring surgical excision, but frequently postoperative complications can affect patients lifelong, so, surgical indications should be based carefully on the balance between risks and benefits.

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About this article

Cavallaro, G., Pattaro, G., Iorio, O. et al. A literature review on surgery for cervical vagal schwannomas. World J Surg Onc 13, 130 (2015). https://doi.org/10.1186/s12957-015-0541-6

Epidemiology

Schwannoma arising from the vagus nerve is an uncommon (2–5%) benign nerve tumour.

Diagnosis

Diagnosis is based on clinical suspicion and confirmation obtained by means of surgical pathology.

Differential diagnosis

Schwannomas of the vagus nerve must be differentiated from the carotid body and glomus vagale tumors because the distinction may influence treatment planning.

Treatment

Surgical excision is the treatment of choice for vagal schwannoma, with recurrence being rare.

Outcome

Nerve damage during surgical resection is associated with significant morbidity 5).

Literature review

In a comprehensive literature review on 197 articles reporting 235 cases of cervical vagal schwannomas. Presenting symptoms, treatment approach, and postoperative outcomes were recorded and analyzed.

Case series

Case series of three patients who underwent vagal schwannoma excision utilizing a IONM technique. The recurrent laryngeal and vagus nerves were monitored via the laryngeal adductor reflex (LAR) using an electromyographic endotracheal tube.

What is Schwannoma of the vagus nerve?

Schwannoma is defined as a benign tumor of neural origin derived from the benign nerve sheath composed of Schwann cells, which normally produce the insulating myelin sheath covering the peripheral nerves [ 1#N#G. Simsek, M. Sahan, B. Gunsoy, A. Arikok, and I. Akin, “Schwannoma of the cervical vagus nerve: a rare benign neurogenic tumor,” Online Journal of Otolaryngology, vol. 3, no. 3, pp. 95–103, 2013. View at: Google Scholar#N#See in References#N#]. Although usually synonymous with acoustic neuroma, schwannoma may also arise from other nerves other than the vestibulocochlear nerve, albeit rarely. Schwannoma of the vagus nerve is a rare entity, seen in only a handful of cases [ 2#N#D. Biswas, C. N. Marnane, R. Mal, and D. Baldwin, “Extracranial head and neck schwannomas—a 10-year review,” Auris Nasus Larynx, vol. 34, no. 3, pp. 353–359, 2007. View at: Publisher Site | Google Scholar#N#See in References#N#]. Of the reported cases, most have been seen among middle aged men as an asymptomatic neck swelling [ 1#N#G. Simsek, M. Sahan, B. Gunsoy, A. Arikok, and I. Akin, “Schwannoma of the cervical vagus nerve: a rare benign neurogenic tumor,” Online Journal of Otolaryngology, vol. 3, no. 3, pp. 95–103, 2013. View at: Google Scholar#N#See in References#N#]. Intraoperatively, they have been seen as a solitary swelling arising from the cervical vagus nerve, even though mediastinal origin has also been seen. Schwannomatosis is a term relatively recently introduced that defines a condition of multiple schwannomas related to a cranial nerve, spinal nerve root, or a peripheral nerve [ 3#N#F. D. Westhout, M. Mathews, L. S. Paré, W. B. Armstrong, P. Tully, and M. E. Linskey, “Recognizing schwannomatosis and distinguishing it from neurofibromatosis Type 1 or 2,” Journal of Spinal Disorders & Techniques, vol. 20, no. 4, pp. 329–332, 2007. View at: Google Scholar#N#See in References#N#]. Multiple schwannomas are usually seen in cases of neurofibromatosis 2 but rarely seen otherwise. Other differentials to be considered include paragangliomas, cervical lymphadenopathy, branchial cyst, and malignant neck swellings [ 4#N#E. Langner, A. Del Negro, H. K. Akashi, P. P. C. Araújo, A. J. Tincani, and A. S. Martins, “Schwannomas in the head and neck: retrospective analysis of 21 patients and review of the literature,” Sao Paulo Medical Journal, vol. 125, no. 4, pp. 220–222, 2007. View at: Publisher Site | Google Scholar#N#See in References#N#]. As there are no specific imaging or histological features, a great deal of clinical suspicion is needed to make an accurate pre-op diagnosis [ 1#N#G. Simsek, M. Sahan, B. Gunsoy, A. Arikok, and I. Akin, “Schwannoma of the cervical vagus nerve: a rare benign neurogenic tumor,” Online Journal of Otolaryngology, vol. 3, no. 3, pp. 95–103, 2013. View at: Google Scholar#N#See in References#N#]. We hereby present a rare case of schwannomatosis of the cervical vagus nerve not otherwise associated with neurofibromatosis 2 (NF-2) [ 5#N#A. Ogose, T. Hotta, T. Morita, H. Otsuka, and Y. Hirata, “Multiple schwannomas in the peripheral nerves,” The Journal of Bone & Joint Surgery—British Volume, vol. 80, no. 4, pp. 657–661, 1998. View at: Publisher Site | Google Scholar#N#See in References#N#].

Where are schwannomas most common?

Most of the extracranial schwannomas are present in the head and neck area; the most commonly affected regions are the temporal bone, lateral neck, and paranasal sinuses [ 6#N#G. C. W. Kang, K.-C. Soo, and D. T. H. Lim, “Extracranial non-vestibular head and neck schwannomas: a ten-year experience,” Annals of the Academy of Medicine Singapore, vol. 36, no. 4, pp. 233–240, 2007. View at: Google Scholar#N#See in References#N#]. Schwannomas developing from cranial nerves are mostly from vestibulocochlear nerve; rarely the glossopharyngeal, accessory, and hypoglossal nerves may also be affected. The vagus nerve is involved in approx. 10% of cases [ 6#N#G. C. W. Kang, K.-C. Soo, and D. T. H. Lim, “Extracranial non-vestibular head and neck schwannomas: a ten-year experience,” Annals of the Academy of Medicine Singapore, vol. 36, no. 4, pp. 233–240, 2007. View at: Google Scholar#N#See in References#N#].

Is cervical schwannoma a benign lesions?

Cervical vagal schwannoma is a rare entity among lesions presenting as a neck mass. They are usually slow-growing benign lesions closely associated with the vagus nerve. They are usually solitary and asymptomatic. Multiple schwannomas occurring in patients without neurofibromatosis (NF) are rare and have recently been referred to as schwannomatosis. Here, we present a case of a neck mass that had imaging features suggestive of vagal schwannoma and was operated upon. Intraoperatively, it was discovered to be a case of multiple vagal cervical schwannoma, all directly related to the right vagus nerve, and could be resected from the nerve in toto preserving the function of the vagus nerve. Final HPR confirmed our pre-op suspicion of vagal schwannomatosis.