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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 20  |  Issue : 1  |  Page : 121-125
Intra-oral schwannoma: Case report and literature review

Department of Rehabilitation Science, Post-Graduation Program, Nove de Julho University UNINOVE, São Paulo - SP, Brazil

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Date of Submission24-Apr-2007
Date of Decision12-Aug-2007
Date of Acceptance19-Dec-2007


Schwannoma is a relatively uncommon, slow-growing benign tumor that is derived apparently from the Schwann cells. The tongue is the most common site, followed by the palate, floor of mouth, buccal mucosa, lips, and jaws. It can present itself at any age. Usually, this lesion is not taken into account during clinical practice and the differential diagnosis includes numerous benign neoformations based on epithelial and connective tissues. Immunohistochemical features can be useful in determining the neural differentiation. Anti-S100 protein is probably the most used antibody to identify this lesion. The schwannoma is usually a solitary lesion, and can be multiple when associated with neurofibromatosis. In the current study, authors report a case of an intraoral schwannoma situated at the vestibule with 20 years of evolution treated by complete surgical excision. The diagnosis was established based on clinical, histopathological, and immunohistochemical aspects. The patient is under clinical control, with no signs of recurrence even after four years.

Keywords: Benign tumor, neural tumors, oral lesions, Schwann cells, schwannoma, soft tissue tumors

How to cite this article:
Martins MD, Anunciato de Jesus L, Fernandes KP, Bussadori SK, Taghloubi SA, Martins MT. Intra-oral schwannoma: Case report and literature review. Indian J Dent Res 2009;20:121-5

How to cite this URL:
Martins MD, Anunciato de Jesus L, Fernandes KP, Bussadori SK, Taghloubi SA, Martins MT. Intra-oral schwannoma: Case report and literature review. Indian J Dent Res [serial online] 2009 [cited 2019 Oct 14];20:121-5. Available from:
The schwannoma, also known as neurilemmoma, neurinoma, or perineural fibroblastoma, is a rare benign neural tumor, arising from the neural sheath Schwann cells of the peripheral, cranial, or autonomic nerves. Its origin, most commonly associated with a nervous trunk, affecting the whole position throughout the course of the peripheral nervous system most of the times. The etiology is unknown, but it is postulated that the lesion arises by proliferation of Schwann cells at one point inside the perineurium. The lesion will cause the displacement and compression of the surrounding normal nerve tissue. [1],[2]

This neoplasm has predilection for the head and neck region where one-thirds of the cases are reported, however intraoral lesions are rare. [1],[2],[3] When it is found in oral structures, the tongue is reported to be the favored site. [4] In a revision of 303 solitary neural tumors, 136 lesions (45%) were located in the head and neck region and among these, only 30 patients presented lesions in oral cavity. [5] Leu and Chang [6] reviewed a series of 52 cases of schwannomas originating in the head and neck region area over an 8-year period. In seven of these cases, the lesions were located in the oral cavity, one in the hard palate, one in the soft palate, two in submasseteric, and one in the tongue, and one in lower lip.

Clinically, the benign schwannoma is a slow-growing encapsulated nodular lesion, usually solitary. Generally, it is asymptomatic, although pain and paresthesia may occur. Within its growth, the lesion dislocates the nerve. It may present itself at any age, but it is more frequent between the second and third decades of life. It is not certain if there is a predilection for women or it is equally distributed between sexes. [1],[7]

The clinical differential diagnosis could be with any other benign tumoral lesions such asfibroma, lipoma, neurofibroma, or salivary glands tumor. However, the histological differential diagnosis is made with other neural origin lesions, which could be neurofibroma and neuroma, or muscular or fibroblastic origin tumor. [5],[8]

Microscopically, the characteristic histological features for schwannomas are similar. These include complete tumor encapsulation and composition consisting of alternating regions of hypocellularity, and hypocellularity known as Antoni A and Antoni B, respectively. An immunohistochemical examination of the tumor may show positive results with S100 antigen. [9],[10],[11]

Radiological examinations such as a computed tomography (CT) scan with contrast and magnetic resonance imaging (MRI) may be performed to show the extension of the tumor. [12]

Conservative surgical removal is the treatment of choice, with wide excision not advocated. Schwannoma shows no reccurence if completely excised. [9] The prognosis is good and malignant transformation of benign schwannoma has been controversial, although a few isolated cases have been documented. [11]

The objective of this study is to report a clinical case of benign schwannoma located at the vestibule of the left maxilla, whose diagnosis was established upon clinical, histological, and imunohistochemical findings and to present a review of the literature.

   Case Report Top

A 44-year-old white female patient presented to the dentistry service for evaluation of a swelling in the vestibule of the left maxilla that had been present for 20 years. Although the patient had noticed swelling, she left it unexamined because there was no pain. The medical history was noncontributory. On extraoral examination, a discreet facial swelling was noted with elevation in the upper lip. On intraoral examination, a submucosal nodular lesion was observed, located in the left maxillary vestibule from the left maxillary lateral incisor to the left maxillary second premolar, it was covered with normal appearing mucosa and measuring approximately 3 x 2 cm [Figure 1].

The lesion was moderately firm and was not fixed to the surrounding tissues. There was no pain on touch. From these data the provisional diagnosis was established as some benign soft-tissue neoplasm process or minor salivary gland tumor. Incisional biopsy was done under local anesthesia, followed by histopathological examination. Microscopic findings revealed a mucous surface covered with unremarkable stratified squamous keratinized epithelium. In lamina propria, a benign mesenchymal neoplasm composed of dense irregularly arranged cells, elongated spindle tumor cells circumscribed by a thin well-defined fibrous connective tissue capsule [Figure 2].

The tumor cells had a fusiform aspect, with no precise cytoplasmatic limits and were organized forming arrangements of Antoni type B [Figure 3],[Figure 4].

Numerous blood vessels were able to be observed. Rare areas with Antoni type A aspect, forming Verocay bodies, were observed. The immunohistochemical examination for S-100 protein was done and showed intense positivity in the tumoral cells [Figure 5].

Based on the clinical behavior, histological, and immunohistochemical aspects, the final diagnosis was of schwannoma. After this diagnosis the lesion was totally excised under local anesthesia. Healing was uneventful. In the light of the pathologic diagnosis, a thorough physical examination was made to rule out multiple tumors of neural origin. The results of this examination were negative. The patient is under clinical control, with no signs of recurrence even after four years [Figure 6].

   Discussion Top

Oral schwannoma is a rare solitary, slow growing, generally asymptomatic neural tumor that can present itself at any age. However it is more common between the second and third decades of life. [1],[3] William et al. [13] findings showed that in 83% of the cases studied by them the schwannomas presented in males, while for Lucas [14] there was a greater predilection for females, and for Hatziotis and Asprides [1] ; Enzinger and Weiss [15] there was an equal distribution between both sexes.

In most of the cases, between 25% and 45%, extracranial schwannoma occurs in the head and neck region. In the oral cavity the lesion in usually presented in soft tissue, more commonly the tongue, followed by the palate, buccal mucosa, and may have clinical aspects similar to other benign lesions like mucocele, fibromas, lipomas, and benign salivary gland tumors. [1],[6],[7],[16] In some cases the tumor could be intraosseous, being more frequent in the mandible where it may cause bone expansion, pain, and paresthesia. In these cases differential clinical diagnosis of cysts and odontogenic tumors are commonly formulated. [17]

Gallo et al. [18] reported on 157 cases, where 45.2% of the cases involved the tongue and 13.3% involved the cheek. Gupta et al. [5] on 136 cases of schwannoma in the head and neck that consisted of 60 cases in the neck, ten cases in the parotid gland, nine cases in the cheek, eight cases in the tongue, and eight cases in the pharynx. Kun et al. [19] reported in their study that 18 out of 49 cases were in the neck and 11 in the tongue.

Wright and Jackson [7] reported 146 cases of schwannoma of the oral cavity soft tissue. Of those, 52% involved the tongue, 19.86% the buccal or vestibular mucosa, 8.9% the soft palate, and the remainder 19.24% were in the gingivae and lip.

In the present case the schwannoma presented as a slow growing, circumscribed swelling without any particular features to distinguish it from other benign soft-tissue lesions. Thus, the provisional diagnosis after clinical examination was of benign neoplasm of mesenchymal origin or minor salivary gland neoplasm. At the beginning, no specific hypothesis was formulated concerning schwannoma because it's a rare oral lesion, specifically at the vestibule. When solitary slow-growing lesions are found, excisional biopsy is suggested as a diagnostic procedure. But in this case an incisional biopsy was chosen, initially, to establish the diagnosis. Some malignant glandular lesions could present slow-growing aspects like those of benign processes. According to Wright and Jackson, [7] the differential diagnosis of schwannomas included some lesions such as lipomas, hemangiomas, eosnophilic granuloma, epidermoid and dermoid cysts, epithelial hyperplasia, granular cell tumor, leyomioma, and lymphangioma.

The histological findings of the present case are similar to those reported previously, consisting of a thin fibrous capsule and a tumoral proliferation formed by two types of tissue arrangements: Antoni type A and type B. The Antoni type A tissue is characterized by Schwann cells that are closely packed, forming bundles or arranged in rows with elongated, palisaded nuclei. Free bands of amorphous substance between the rows of nuclei constitute the so-called Verocay bodies which under electron microscope, appear to be composed of thin cytoplasmic processes with small amounts of collagen and basal laminar material showing frequent redoubling. [2] While in the Antoni B tissue it has less number of cells and less organization, where the fusiform cells are widely separated, dispersed in a loose and random fashion with a network of delicate reticulated fibers. The vasculature is generally not prominent, but in more older tumors some changes occur, like inflammation, fibrosis, and nuclear atypia. [14],[15],[19],[20] In the present case, the histopathologic analysis revealed a majority of Antoni B pattern for the whole specimen. Considering the nonorganization of the tumoral Schwann cells forming the Verocay bodies, that are classical structures of this lesion, immunohistochemical research for S100 was done which was intensively positive. According to the literature survey, immunostaining analysis is essential in the diagnosis of this neoplasm. Most parts of the tumoral cells in Schwannoma show immunohistochemical positive reaction for S100 protein. [15],[20],[21],[22] According to Chrysomali et al. [20] the tumoral cells with Antoni A show greater intensity scores compared to Antoni B tumor pattern.

Microscopically, both schwannoma and the neurofibroma contain elongated cells with irregular nuclei lying between bundles of collagen fibers. They differ histologically and histogenetically, the schwannoma is derived from the Schwann cells and the neurofibroma from the fibroblasts of the perineurium. Neurofibroma is unencapsulated, consisting of a mixture of Schwann cells, perineurial cells, and endoneurial fibroblasts. [8],[15],[20]

From the immunohistochemical viewpoint, all neural origin tumors show positivity for S100 protein, but immunohistochemical examination could assist in lesion differentiation. Chrysomaly et al. [20] observed an intense positive reaction to S100 in schwannoma and palisaded encapsulated neuroma. Intensive reaction to CD57 is observed in traumatic neuroma, while capsular epithelial membrane antigen (EMA), and CD34 stainings are observed in schwannoma.

Passador-Santos et al. [22] studied S100 protein, EMA, laminin, fibronectin, and collagens I and II in neural benign neoplasms. They found that schwannoma cells were intensively positive for S100 regardless of the growth pattern. EMA was seen only in the capsule staining perineurial cells and laminin stained basement membrane around tumor cells. Fibronectin and collagens I and III were expressed, especially in the capsule. In neurofibroma, the cells expressed S100 whereas EMA was expressed only in a few scattered cells. Laminin and fibronectin showed a diffuse positivity. Collagens I and III showed a fibrilar arrangement with diffuse positivity.

The treatment for benign schwannoma consists of total surgical lesion removal. According to Asaumi et al[23] , ultrasonography, CT, and MRI may be helpful diagnostic and treatment tools, for the estimation of tumor margins, the lesion composition, and the determination of whether there is infiltration to surrounding structures or not. The recurrences as well as the malignant transformation are rare events.

The presence of schwannoma calls for the careful search for nerve tumors in other parts of the body, although in most cases none may be found. The differentiation of schwannoma from neurofibroma is essential, because an apparently solitary neurofibroma may be a manifestation of neurofibromatosis. [7],[13]

   Conclusion Top

The schwannoma represents a lesion not often encountered in clinical practice. The submucosal forms of this lesion are usually indistinguishable from other benign neoplasms that are also usually seen in the same region. The final diagnosis should be done after histopathological exam and in some cases after immunohistochemical analysis. The therapeutical conduct is the total removal of the lesion.

   References Top

1.Hatziotis JC, Asprides H. Neurilemmoma (schwannoma) of the oral cavity. Oral Surg 1967;24:510-26.  Back to cited text no. 1    
2.Zachariades N, Mezitis M, Vairaktaris E, Triantafyllou D, Skoura-Kafoussia C, Konsolaki-Agouridaki E, et al . Benign neurogenic tumors of the oral cavity. Int J Oral Maxillofac Surg 1987;16:70-6.  Back to cited text no. 2  [PUBMED]  
3.Takeda Y. Neurilemmoma in maxillary alveolar bone: Report of a case. Br J Oral Maxillofac Surg 1991;29:208-10.  Back to cited text no. 3  [PUBMED]  
4.Pahwa R, Khurana N, Chaturvedi KU, Raj A. Neurilemmoma of tongue. Indian J Otolatyngol Head Neck Surg 2003;55:193-4.  Back to cited text no. 4    
5.Das Gupta TK, Brasfield RD, Strong EW, Hadju SI. Benign solitary schwannomas (neurilemmomas). Cancer 1969;24:355-66.  Back to cited text no. 5    
6.Leu Y-S, Chang KC. Extracranial head and neck schwannomas: A review of 8 years experience. Acta Otolaryngol 2002;122:435-7.  Back to cited text no. 6    
7.Wright BA, Jackson D. Neural tumors of the oral cavity: A review of the spectrum of benign and malignant oral tumors of the oral cavity and jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1980;49:509-22.  Back to cited text no. 7    
8.Requena L, Sangueza OP. Benign neoplasms with neural differentiation: A review. Am J Dermatopathol 1995;17:75-96.  Back to cited text no. 8    
9.Lopez JI, Ballestin C. Intraoral schwannoma: A clinicopathologic and immunohistochemical study of nine cases. Arch Anat Cytol Pathol 1993;41:18-23.  Back to cited text no. 9    
10.Pfeifle R, Baur DA, Paulino A, Helman J. Schwannoma of the tongue: Report of two cases. J Oral Maxillofac Surg 2001;59:802-4.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Yang SW, Lin CY. Schwannoma of the upper lip: Case report and literature review. Am J Otolaryngol 2003;24:351-4.   Back to cited text no. 11  [PUBMED]  [FULLTEXT]
12.Anil HT, Chandre Gowda BV, Lakshmi S, Niveditha SR. Schwannoma of the palatine tonsil. J Laryngol Otol 2005;119:570-2.  Back to cited text no. 12    
13.William HK, Cannell H, Silvester K, Williams DM. Neurilemmoma of the head and neck. Br J Oral Maxillofac Surg 1993;31:32-5.  Back to cited text no. 13    
14.Lucas RB. Pathology of tumors of the oral tissue. New York: Churchill Livingstone; 1984.  Back to cited text no. 14    
15.Enzinger IM, Weiss SW. 3 rd ed. Soft tissue tumours. St Louis: MO Mosby; 1995. p. 821-50.  Back to cited text no. 15    
16.Colreavy MP, Lacy PD, Hughes J, Bouchier-Hayes D, Brennan P, O'Dwyer, et al . Head and neck schwannomas: A 10 years review. J Laryngol Otol 2000;114:119-24.  Back to cited text no. 16    
17.Llewelyn J, Sugar AW. Neurilemmoma of the mandible: Report of a case. Br J Oral Maxillofac Surg 1989;27:512-6.  Back to cited text no. 17  [PUBMED]  
18.Gallo WJ, Moss M, Shapiro DN, Gaul JV. Neurilemoma: Review of the literature and report of five cases. J Oral Surg 1977;35:235-6.  Back to cited text no. 18  [PUBMED]  
19.Kun Z, Qi DY, Zhang KH. A comparison between the clinical behavior of neurilemomas in the head and neck and oral and maxillofacial region. J Oral Maxillofac Surg 1993;51:769-71.  Back to cited text no. 19  [PUBMED]  
20.Chrysomali E, Papanicolaou SI, Dekker NP, Regezi JA. Benign neural tumors of the oral cavity: A comparative immunohistochemical study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:381-90.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]
21.Stefanson K, Wollmann R, Jerkovic M. S-100 protein in soft-tissue tumors derived from Schwann cells and melanocytes. Am J Pathol 1982;106:261-8.  Back to cited text no. 21    
22.Passador-Santos F, Turatti E, Gorisch MR, Santos E, Sousa SC. Estudo imuno-histoquνmico de tumores de origem neural na cavidade oral. RPG Rev Pós Grad 2002;9:277.   Back to cited text no. 22    
23.Asaumi JI, Konouchi H, Kishi K. Schwannoma of the upper lip: Ultrasound, CT and MRT findings. J Oral Maxillofac Surg 2000;58:1173-5.  Back to cited text no. 23    

Correspondence Address:
Manoela Domingues Martins
Department of Rehabilitation Science, Post-Graduation Program, Nove de Julho University UNINOVE, São Paulo - SP
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9290.49059

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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