Extracranial head and neck schwannomas: A single centre retrospective experience of 97 cases .

Prankur Verma*, Jeyashanth Riju*, Natarajan Ramalingam*, Rubine Zeinuddin*, Antony Paulose, Sherin

Susheel**, Meera Thomas**, Rajan Sundaresan***, Rajiv Michael*, Amit Tirkey*, Aparna Irodi****,

Kiranmayi C****

* Department of Head and Neck Surgery, Christian Medical College, Vellore, Tamil Nadu, India, 632002

** Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India, 632002

*** Division of Skull Base Surgery, Department of E.N.T., Christian Medical College, Vellore, Tamil Nadu,

India, 632002

**** Department of Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India, 632002

Corresponding author: Jeyashanth Riju M.S., F.H.N.S.O., Assistant Professor and In-charge, Department

of Head and Neck Surgery Unit- 1, Christian Medical College, Vellore. Phone: +914162282075,

+917406020741. Email: jjriju@yahoo.co.in

Declaration of Conflicting Interests

The Authors declare that there is no conflict of interest.

Funding statement

This work received no funding or any specific grant from any source.

Ethics committee approval

Approval for the study was obtained from the Ethics Committee, Institutional Review Board, Christian

Medical College, Vellore, Tamil Nadu, India, 632002. IRB Number: 15156 dated 25/01/2023
ABSTRACT

Introduction: Schwannomas are rare benign neurogenic tumours. About 25 - 45 % of extra cranial

schwannomas are found in the head and neck region. In head and neck, they can arise from various cranial,

peripheral and autonomic nerves. Due to this, they have varied clinical presentations.

Material and Methods: This retrospective cohort includes 97 patients diagnosed with extra cranial head and

neck schwannoma, and operated in the Otolaryngology and Head & Neck Surgery department, from 2013 till

2022 were recruited as a retrospective cohort. The parameters observed were the age and gender distribution,

location, nerve of origin, size, pressure symptoms, cranial palsies, post operative symptoms, recurrence and

complications. The data were collected from the electronic records of the institute.

Results: The median age of study population was 36 years among them. 51 patients were menale and 46

were womenfemales. The most common tumour location was parapharynxs were varied. The patients had

varied head and neck symptoms of which 23.7% patients had nerve pareses pre operativelyat presentation. A

variety of surgical approaches were used. 22 patients developed Nnew onset cranial nerve pareses immediate

post operativelysurgery was noted in 22.7%. Recurrence was noted in 2% of casespatients.

Conclusions: Extra cranial schwannomas are uncommon neoplasms and have varied presentations.

Comprehensive evaluation with appropriate imaging should be done in all cases. Though benign, patients

can present with nerve paresis. Recurrence is uncommon after complete resection.

 Keywords: Sschwannoma, Nerve Sheath Neoplasms, Head and Neck Neoplasmhead and neck

tumours, cranial nerve palsyresis, extra cranial schwannoma, benign tumoursneoplasm.

INTRODUCTION

Schwannomas are rare benign neurogenic tumours. Along with neurofibroma, they constitute

one of the two most common benign nerve sheath tumours(1). It is reported that 25–45% of extra cranial non

vestibular schwannomas are found to be in the head and neck region(2). They are encapsulated,

slow-growing tumors that arise from Schwann cells of myelinated nerves and so can be found arising from

various cranial, peripheral, and autonomic nerves. They can arise in a variety of locations in the head and

neck region, given the abundance of various nerves, and thus they have varied presentations. The clinical

signs and symptoms vary according to the size and location of the tumour, and the nerve of origin(3). Such

varied presentations pose diagnostic and therepeutic challenges. Surgical resection is the mainstay of

treatment.

This study describes the clinico pathological presentation and outcome following surgical resection in a set

of 97 patients with extra cranial head and neck schwannomas arising in a variety of locations and study the

post operative course. This series is supposed to be one of the largest of its kind till now, to the best of our

knowledge.

MATERIAL AND METHODS

Methodology

A total of 97 patients diagnosed with extra cranial non vestibular schwannomas, operated between January

2013 till December 2022 in the Department of Otorhinolaryngology and Head and Neck Surgery and The

Department of otorhinolaryngology of the Christian Medical College, Vellore, India, were recruited as a

retrospective cohort. Those with a history of radiotherapy to the head and neck region, those who presented

with a recurrent schwannoma, and those who did not undergo surgical intervention were excluded from the

study. Ethical clearance was obtained from theStudy was approved by Institutional Review Board before

conducting the study.

The objective of the study was to investigate the clinico pathological features of various extra cranial non

vestibular schwannomas; and to analyse the immediate and long termlong-term complications following the

surgery.

The parameters that were analysed were the age and gender distribution, location and side of the tumour, the

nerve of origin. The symptomatology due to the mass effect and nerve dysfunction was noted. Local
examination findings with emphasis on cranial and autonomic nerve functions were recorded. DPost

operative diagnostic workup including imaging and cytopathology were noted. Surgical approaches to the

tumour were noted. Various immuno-histochemical markers that aided in the pathology reporting were

studied. Post operative complications that included occurrence of nerve dysfunction were noted. The

patient’ss’ routinely followed after up upto 6 months or 1 year post surgery, or earlier, if symptomatic, was

studied. Further follow up was based if symptomaticon symptom or at the discretion of the treating physician

was noted. The data were collected from the electronic records of the institute and entered in the spreadsheet

software for analysis.

RESULTS

Population characteristics and the tumour location

The study included 97 patientss studied, of them, 52.6% were male and 47.4% were female. Overall median

age was 36 years, the median age of males being 34 and that of females being 40 years. Excluding the

tumours situated midline, 42.3% of the tumours were present on the right side, while 53.6% tumours were on

the left, and 1 of the patient had bilateral tumours. The tumour predominantly involved the left side in men

(62.5%) but was evenly distributed in females(Table 1). Considering the location of the tumours; 49.5%

were parapharyngeal, 19.6% were cervical, 10.3% arose from the parotid region, 6.2% were located in the

nose or paranasal sinus region, and 14.4% from other unusual sites (Fig 1).like supra glottis, nasal cavity,

external auditory canal, palate, base of the tongue and medial canthus(Fig 1).

NERVE OF ORIGIN?

MEAN FOLLOWUP PERIOD?

Symptomatology and presentation

The most common presenting symptom was a swelling in the neck or mouth (68%). Pain over the side or site

of the mass lesion was noted in 20.6% of the patients presented with pain over the side or site of the mass

lesion. Out of the 67 patients with deep neck space (cervical or parapharyngeal) schwannomas, 19% had

change in voice. 19% presented with difficulty in swallowing. We noticed Ear related complaints were

present in 10.3% of the total patients. 15.5% of the patients had symptoms related to a nerve paresis like

voice change, facial weakness, or coughing just after swallowing foodon having liquid diet or facial
weakness(IS IT CORRECT? WHY DO WE HAVE FACIAL WEAKNESS DATA HERE). Among those

with schwanomma arising from the facial nerve, 33.3% had facial nerve weakness (4 out of 12), 3 (25%) had

ear healed related complaints, 1 had headache as the sole symptom, while one was incidentally detected on

imaging. Among the 6 with tumour arising from the nose or paranasal sinuses, nasal obstruction was present

in 2 cases , nasal bleeding in 1 case, and loosening of teeth in 1 case. Ear related complaints were present in

10.3% of the total patients. Other uncommon presentations that were related to the location of the tumour

were nasal block, tingling sensation over the tongue, facial pain, vision loss, oral bleeding, tingling sensation

in the arm, or swelling in the medial canthal region(MENTION PERCENTAGE IN BRACKET eg nasal

block (1%) facial pain(2%) etc.).

Out of the deep neck space schwnnomasschwannomas, neck swelling was noted in 88% and oropharyngeal

mass in 47.6%. Among those with facial nerve schwannoma, 58.3% (7/12) had facial swelling and

33.3%(4/12) had facial paresis. Uncommon locations of the tumour were supra glottis, nasal cavity, external

auditory canal, palate, base of the tongue and medial canthus (Fig 1).

Various single or multiple nerve paresis were found in 23.7% of the total patients on presentation. Among

them, the most commonly involved nerve was vagal (43.5%) followed by hypoglossal nerve (34.8%). A

total of 34.8% of those with nerve paresis had multiple nerve involvement (Table 2).

Pre operative evaluation

Contrast enhanced Computed Tomography and Magnetic Resonance Imaging were the mainstay of cross

sectional imaging methods that were performed in 92.8% of the cases; in the rest, ultrasonography was used.

The mean size of the tumour was 5.4 cm. Radiological investigations could categorise 68.9% of the tumours

to be of nerve sheath origin.

Pre operative fine needle aspiration cytology was performed in 54.6%. Among them, 49.1% showed spindle

cell/ neurogenic origin, 11.3% showed benign tumour, 1.9% showed necrosis. In 35.8%, FNAC material was

inadequate for characterisation. 3 cases were hybrid nerve sheath tumours characterised as schwannoma-

neurofibroma.

Surgical approaches and post operative period

Surgical excision was performed using the following approaches: transcervical with or without mandibular

subluxation approach (62 cases), transcervical with mandibulotomy (7 cases), trans parotid (13 cases), trans

oral approach (10 cases, 2 had laser assisted excision and others(CORRECT?) had TOUSS assisted excision)

and endonasal (5 cases) (Fig 2). Uncommon approaches that were used were sub occipital, transmastoid,

craniotomies and Fisch type A depending upon the location of the tumour. None of the patients who

underwent surgery required tracheostomy except for one with a supraglottic schwannoma that required

temporary tracheostomy, who was successfully weaned off. During the immediate post operative period,

Nasogastric tube feeding was needed in 10.3% of the patients due to aspiration while 3.1% of them required

it in the long term. All these were related to parapharyngeal schwannoma excision. Fig 3 shows __(CONT

FIND IMAGE)

Among the immunohistochemical markers, S100 and SOX10 were used as an aid in the diagnosis in 17.5%

and 14.4% cases respectively. CD 34 was positive in 4 out of 7 cases (57.1%). Other markers that turned

negative included SMA (3 cases), synaptophysin (1 case), desmin (1 case), EMA (2 cases). MIB 1 index was

performed in 9 cases in whom the median value was 6% (range 1 - 30%). Hybrid nerve sheath tumours was

noted in 3.1%.

Surgical complications and post operative morbidity

Immediately following the surgery, 1 patient required re exploration and evacuation of haematoma, and one

patient developed surgical site infection that was managed conservatively with antibiotics.

Among the new onset nerve dysfunctions (Fig 4), 9 developed Horner’s syndrome, 8 developed hypoglossal

palsy, 13 developed vagal palsy, 10 developed facial paresis(among them 8 were facial nerve schwannomas)

and 2 had trigeminal paresis. Among those whom underwent deep neck space schwannoma excision,

dysphonia, aspiration and dysphagia was present noted in 25.4% (17 cases), 17.9% (12 cases) and 9% (6

cases) respectively. 10.3% of these required nasogastric tube insertion.

Analysing the long term neural dysfunction and morbidity among the deep neck space schwannomas;

dysphonia, aspiration and dysphagia was present in 7.5% (5 cases), 6% (4 cases), and 3% (2 cases)

respectively. One case patient developed trismus. Two had chronic pain at the surgical site and temporo-

mandibular joint respectively. In those patients who developed new onset nerve palsies; recovery was noted
in 80% of facial nerve, 62.5% of hypoglossal nerve, and 67% of sympathetic chain dysfunctions. Recovery

was not noted in trigeminal schwannomas. Good compensation was noted in 61.5% of vagal palsies(HOW

MUCH VAGUS RECOVERED). Fig 4 shows new onset nerve deficits and residual nerve deficits in the

follow up period. (IN FIG 4. OPTIC AND GLOSSOPHARYNGEAL were mentioned New onset long term

only)

Among the pre operativepreoperative nerve deficits, one case with hypoglossal weakness and one with

glossopharyngeal deficit improved. Three patients had first bite syndrome.

Recurrence and mortality

Two patients treated for trigeminal schwannoma showed recurrence along the skull base, who required

revision surgery and one of them underwent adjuvant radiotherapy. The other patient patient was not willing

for further treatment, and interestingly had a MIB 1 index of 30%.

A patient with a parapharyngeal schwannoma who passed the balloon occlusion test underwent trans oral -

trans cervical excision suffered cerebro-vascular accident and developed hemiplegia, and died of respiratory

infection and septicaemia in the ICU.

We encountered an interesting case of a bilateral parapharyngeal schwannoma who underwent excision via

transcervical approach and endoscopic trans pterygoid - trans maxillary approach. Two years later, there was

development of left sided temporal lobe glioblastoma which required surgical intervention followed by

chemo radiation.

DISCUSSION

We analysed 10 year data retrospectively in our study comprising 97 patients. To the best of our knowledge,

this is one of the largest study comprising extra cranial non vestibular schwannomas. The mean age of the

patient was 37.4 years which is in accordance with the other studies(5 - 7) but we noticed there was a slight

male preponderance and occurs at a younger age group. Gender distribution was balanced. There was a

predilection to the left side in the males, while the laterality was balanced in females. The reason for this

laterality is not yet known but couldmight be related to some embryological process. Hybrid schwannoma-

neurofibroma were noted in 3 female patients that involved facial or vagal nerve(NEED TO HAVE A

REFFERNCE ARTICLE FOR HYBRID).

Almost half of the schwannomas arose in the parapharyngeal space followed by cervical and facial regions,

and the rest in unusual locations like nose or maxillary sinus, tongue, supra glottis, anterior skull base,

brachial plexus, middle ear, orbit and sub occipital region. This pattern is consistent with other similar

studies(3, 8, 9).

The usual presentation is a mass lesion over the head and neck region including or bulge in the oropharynx,

which was noted in 68% in our study. Around 20% cases presented with pain over the lesion or in the

surrounding structures. The incidence of pain has been reported to be 3 - 15% according to various series(10,

11). The symptomatology can be varied and usually depends on the nerve affected and the pressure

symptoms caused by the tumour, such as voice change, dysphagia, ear fullness, vision loss, facial paresis,

tingling sensation or hypoaesthesia, loosening of teeth, proptosis, breathing difficulty or headache. In the

study by Wang et al, brachial plexus nerves were the most common nerve of origin, while it was the

sympathetic trunk in Liu’s series(3, 12).

Among the 10 patients with schwannoma arising from the extra temporal part of the facial nerve, 60% had

facial swelling, 20% presented with facial weakness, 10% had facial swelling and weakness and 10% were

suspected to have a salivary neoplasm.

We encountered single or multiple nerve dysfunction in 23.7% of the cases, the most common being vocal

cord paralysis, followed by hypoglossal nerve paresis. Almost all those with multiple palsies on presentation

were parapharyngeal schwannomas, while 2 arose in the orbit and the anterior skull base respectively.

Multiple nerve palsies are associated with extensive or large tumours. Since the involved nerve can render

permanent deficits, the knowledge of the nerve of origin aids in pre operative counselling of the patients and

expected outcomes(12).

Cross sectional imaging, particularly the MRI, was found to characterisecharacterize the lesion as a nerve

sheath tumour in almost 70% of the cases. It is therefore the preferred method of pre operative diagnosis in

the current times(8, 12). MRI can also help to identify the nerve of origin(3, 12). We found out that the

accuracy of FNAC to identify a schwannoma was 49.1% in our study, so it is not recommended as a

diagnostic modality for suspected schwannomas(1, 8, 9, 13). The yield of FNAC was often inadequate,

which is consistent with other studies(3, 9, 13). Furthermore, tumours in unusual locations may not be

amenable to aspiration cytology. FNAC may have a role in those cases where there is a suspicion of

malignancy or metastasis. Dongbin et al suggest core needle biopsy as a sensitive pre operative diagnostic
modality, but warn about the risks of nerve and organ damage, though there was no permanent nerve injury

in their study(13).

We used a variety of approaches given the wide variety of tumour locations, the most commonly employed

being transcervical approach with or without mandibular subluxation. Complete surgical excision remains

the standard treatment of choice(1, 3), preferably intracapsular enucleation whenever possible, to preserve

the nerve function(9, 12, 14).

One important goal of our study was to analyse the short term and long term complications following the

surgery. The most common nerve related immediate complication was vagal palsy followed by facial

weakness and Horner’s syndrome.

Analysis of cranial nerve function in the long run showed an interesting trend, especially vocal cord palsy, in

that due to contralateral compensation and return of nerve laryngeal function. in many cases caused only a

limited number of total long term deficits. In most of the cases with vagal dysfunction, Aaspiration and

dysphagia had considerably improved with only ~3% requiring long term nasogastric tube insertionfeed.

Such findings should encourage surgical intervention in such cases, Ssince a significant number of cases

present with nerve deficits, and complete surgical excision causes minimal morbidity in the long term,

almost a negligible risk of recurrence if complete resection has been performed. A considerable amount of

return of nerve related functions can be expected even if deficits occur post operatively. In contrast, pre

operative nerve deficits do not usually improve, which provides an impetus for early surgery before

permanent deficits take place.

Recurrence is rare following complete intra capsular excision (12, 15, 16). In our series, 2 cases recurred;

both had involvement of the skull base and arose from the trigeminal nerve and complete excision could not

be performed. This finding stresses the need for complete excision to avoid recurrences despite it being a

benign disease. It is therefore necessary to operate such cases when the tumour is not extensive, and so early

surgery, rather than wait and watch, should be advocated. Observational policy can be applied in patients

who maybe unfit for a radical surgical procedure(8).

There is no evidence to suggest malignant transformation of these tumours. A review on intracranial

malignant peripheral nerve sheath tumour (MPNST) suggested that most of the MPNSTs develop

sporadically rather than transforming from benign tumours(17). Since there is evidence that most MPNSTs

are associated with neurofibromatosis type 1(18, 19); this might be the case with our only patent that
presented with bilateral parapharyngeal schwannomas who went on to develop glioblastoma in the temporal

lobe. It is likely that the patient had a syndromic association (Neurofibromatosis/ Schwannomatosis)(4) and

it is not known whether the malignancy arose de novo or was a transformation. Thus it is safe to conclude

that solitary schwannomas do not appear to undergo malignant transformation.

Our study had some limitations, in that it was retrospective, and brachial plexus schwannomas were not

included as they are treated by a different speciality.

CONCLUSION

Extracranial head and neck schwannomas are rare, benign tumors with varied presentations. Surgery using

various approaches depending upon the tumour location is the mainstay of treatment as it carries minimal

morbidity and furthermore long term cranial nerve dysfunction is uncommon. Early surgery should be

advocated while the tumour is still small to prevent new nerve deficits and avoid recurrences. Recurrence is

unusual after complete excision.

REFRERENCES

1. Zhang H, Cai C, Wang S, Liu H, Ye Y, Chen Xet al. Extracranial Head and Neck Schwannomas:

A Clinical Analysis of 33 Patients: The Laryngoscope. 2007 Feb;117(2):278–81.

2. Colreavy MP, Lacy PD, Hughes J, Bouchier-Hayes D, Brennan P, O’Dwyer AJ, et al. Head and

neck schwannomas--a 10 year review. J Laryngol Otol. 2000 Feb;114(2):119–24.

3. Wang B, Yuan J, Chen X, Xu H, Zhou Y, Dong Pet al. Extracranial non-vestibular head and

neck schwannomas. Saudi Med J. 2015 Nov;36(11):1363–6.

4. Dhamija R, Plotkin S, Asthagiri A, Messiaen L, Babovic-Vuksanovic D. Schwannomatosis. In:

Adam MP, Mirzaa GM, Pagon RA, Wallace SE, Bean LJ, Gripp KW, et al., editors.

GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993 [cited 2023

Jun 30]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK487394/

5. Liu R, Fagan P. Facial nerve schwannoma: surgical excision versus conservative management.

Ann Otol Rhinol Laryngol. 2001 Nov;110(11):1025–9.

6. Das A, Bhalla AS, Sharma R, Kumar A, Thakar A, Goyal A. Diffusion-weighted imaging in

extracranial head and neck schwannomas: A distinctive appearance. Indian J Radiol Imaging.

2016;26(2):231–6.

7. Caughey RJ, May M, Schaitkin BM. Intraparotid facial nerve schwannoma: diagnosis and

management. Otolaryngol--Head Neck Surg Off J Am Acad Otolaryngol-Head Neck Surg. 2004

May;130(5):586–92. Caughey RJ, May M, Schaitkin BM. Intraparotid facial nerve schwannoma:

diagnosis and management. Otolaryngology—Head and Neck Surgery. 2004 May;130(5):586-

92.

8. Zubair A, Kumar R, Jat B, Sagar P, Kairo A, Kumar R. Unusual Sites for Extracranial Head–

Neck Schwannomas. Indian J Otolaryngol Head Neck Surg. 2020 Dec;72(4):416–21.

9. Sharma P, Zaheer S, Goyal S, Ahluwalia C, Goyal A, Bhuyan G, et al. Clinicopathological

analysis of extracranial head and neck schwannoma: A case series. J Cancer Res Ther.

2019;15(3):659–64.

10. Kuet ML, Kasbekar AV, Masterson L, Jani P. Management of tumors arising from the

parapharyngeal space: A systematic review of 1,293 cases reported over 25 years. The

Laryngoscope. 2015 Jun;125(6):1372–81.

11. Sandler ML, Sims JR, Sinclair C, Sharif KF, Ho R, Yue LE, et al. Vagal schwannomas of

the head and neck: A comprehensive review and a novel approach to preserving vocal cord

innervation and function. Head Neck. 2019 Jul;41(7):2450–66.

12. Liu HL, Yu SY, Li GKH, Wei WI. Extracranial head and neck schwannomas: a study of the

nerve of origin. Eur Arch Otorhinolaryngol. 2011 Sep;268(9):1343–7.

13. Ahn D, Lee GJ, Sohn JH, Jeong JY. Fine‐needle aspiration cytology versus core‐needle

biopsy for the diagnosis of extracranial head and neck schwannoma. Head Neck. 2018

Dec;40(12):2695–700.

14. Ijichi K, Murakami S. Surgical treatment of parapharyngeal space tumors: A report of 29

cases. Oncol Lett. 2017 Sep;14(3):3249–54.

15. Ozdemir O, Ozsoy MH, Kurt C, Coskunol E, Calli I. Schwannomas of the hand and wrist:

long-term results and review of the literature. J Orthop Surg Hong Kong. 2005 Dec;13(3):267–

72.

16. Kim SH, Kim NH, Kim KR, Lee JH, Choi HS. Schwannoma in head and neck: preoperative

imaging study and intracapsular enucleation for functional nerve preservation. Yonsei Med J.

2010 Nov;51(6):938–42.
17. Ziadi A, Saliba I. Malignant peripheral nerve sheath tumor of intracranial nerve: a case

series review. Auris Nasus Larynx. 2010 Oct;37(5):539–45.

18. Tucker T, Wolkenstein P, Revuz J, Zeller J, Friedman JM. Association between benign and

malignant peripheral nerve sheath tumors in NF1. Neurology. 2005 Jul 26;65(2):205–11.

19. Pasmant E, Masliah-Planchon J, Lévy P, Laurendeau I, Ortonne N, Parfait B, et al.

Identification of genes potentially involved in the increased risk of malignancy in NF1-

microdeleted patients. Mol Med Camb Mass. 2011;17(1–2):79–87.