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Year : 2014  |  Volume : 25  |  Issue : 2  |  Page : 263-265
Infraorbital nerve schwannoma: A rare cause of upper jaw swelling

1 Department of ENT, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India

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Date of Submission17-Dec-2012
Date of Decision25-Feb-2013
Date of Acceptance05-Apr-2013
Date of Web Publication4-Jul-2014


Introduction: Schwannoma of the infraorbital nerve is a rare entity, with lesser than a dozen cases reported in literature and only one from India.
Report: This article reports a 23-year-old male presenting with a painless swelling in the cheek, which was eventually diagnosed as infraorbital nerve schwannoma. He underwent a complete excision of the tumor via a Caldwell Luc approach and continues to be disease free on 3 year follow-up.
Conclusion: Despite its rarity, infraorbital nerve schwannomas should be considered in the differential diagnosis, of upper jaw swelling. We recommend the Caldwell Luc approach as safe, effective and cosmetically acceptable, for anteriorly based infraorbital schwannomas, and review literature on this unusual entity

Keywords: Caldwell luc approach, infraorbital nerve schwannoma, upper jaw swelling

How to cite this article:
Naina P, Masih D, Mathews SS. Infraorbital nerve schwannoma: A rare cause of upper jaw swelling . Indian J Dent Res 2014;25:263-5

How to cite this URL:
Naina P, Masih D, Mathews SS. Infraorbital nerve schwannoma: A rare cause of upper jaw swelling . Indian J Dent Res [serial online] 2014 [cited 2022 Aug 13];25:263-5. Available from:
Schwannomas are benign tumors that arise from schwann cells present in nerve sheaths and grow very slowly in close relationship with the nerve of origin. More than one-third of all schwannomas occur in the head and neck. Most of these originate from the lower cranial nerve and sympathetic nervous system. Schwannomas from the upper cranial nerves such as trigeminal nerve and its branches are less common. Schwannomas of the infraorbital nerve are rare with only 11 cases reported in the literature so far, which includes one case from India. [1],[2],[3],[4],[5]

   Case report Top

A 23-year-old male presented with a 6 month history of progressively enlarging, painless swelling over the right cheek with no nasal, oral or visual complaints. There was no history of trauma or previous surgery. Physical examination showed a 5 cm × 3 cm, firm swelling over the right cheek with no features of inflammation. Computed tomography (CT) of the paranasal sinuses showed a well-defined expansile hypodense lesion arising from the right maxillary region, above the root of teeth with expansion and remodeling of walls of the right maxillary sinus. The possibility of dentigerous cyst, odontogenic keratocyst, and ameloblastoma were considered.

Considering the size and location of the tumor, it was planned to approach it through a right Caldwell Luc approach. Intra-operatively, the lesion was found to be large, expansile and circumscribed with areas of solid and cystic consistency, filling the entire maxillary sinus and was seen to be arising from the infraorbital nerve [Figure 1]. The anterior wall of the maxilla was thinned out due to pressure from the mass. The lesion was completely excised and subjected to the histopathological and immunohistochemical examination.
Figure 1: Intra-operative picture showing large circumscribed lesion in right maxillary sinus

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Histopathological examination of the tan colored specimen excised, revealed tumor composed of fascicles of monomorphic spindle shaped cells with pointed basophilic nuclei and poorly defined eosinophilic cytoplasm. Focal palisading of tumor cells was present [Figure 2]. There was diffuse nuclear immunopositivity to S-100 protein indicative of a schwannoma [Figure 3]. This tumor originated from the infraorbital branch of the maxillary nerve - A type E tumor in accordance to the classification of trigeminal nerve schwannoma by Yoshida and Kawase. [6] The patient had an uneventful recovery and continues to be free of symptoms, 3 years after surgery.
Figure 2: Photomicrograph showing tumors composed of fascicles of monomorphic spindle shaped cells with pointed basophilic nuclei and poorly defined eosinophilic cytoplasm (H and E, ×200)

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Figure 3: Photomicrograph of tumor cells showing intense immunoreactivity for S-100 protein, ×100

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   Discussion Top

Schwannomas are common benign tumors, with 25-45% occurring in the head and neck. In the head and neck, schwannomas are more commonly seen in the lower cranial nerves as compared to upper cranial nerves. Surgical resection is the main stay of treatment.

Microscopically, these tumors show a biphasic pattern of tumor cells with areas of closely packed spindle cells having fusiform nuclei and eosinophilic cytoplasm (Antoni A) admixed with looser myxoid tissue having ovoid cells (Antoni B). At places, these tumor cells are arranged in palisades and this arrangement of cells is referred to as Verocay bodies. Long standing lesions may show areas of degeneration such as hyalinization, stromal hemorrhage, cystic change, and calcification. Immunohistochemical markers are routinely used as an adjunct in the diagnosis of schwannomas. The S-100 protein, a protein expressed in the peripheral schwann cells is used not only in differentiating schwannomas from tumors of non-neural origin, but also in separating schwannomas from neurofibromas. Schwannomas show diffuse intense immunoreactivity with more uniform and pronounced staining as compared to neurofibromas.

The term malignant schwannomas is used for malignant variants of schwannoma, which are included in the group of malignant peripheral nerve sheath tumors. Only one case of malignant schwannoma of the infraorbital nerve has been reported so far. [7] The diagnosis may be suggested on the basis of rapid growth or extensive nerve involvement, but can be confirmed only by histopathological diagnosis by features such as diffuse cellular atypia, p53 positivity, and high MIB1 staining.

Imaging modalities such as CT and magnetic resonance imaging (MRI) are used in pre-operative analysis. On MRI, schwannomas show low to intermediate signal on T1W1 images and high signal intensity on T2W1 with intense post-contrast enhancement. [8] Though MRI is the investigation of choice in delineating the tumor from the surrounding structures, it is not routinely carried out in all sinonasal schwannomas. A contrast enhanced CT scan could provide information that is comparable to MRI though the latter is much superior in demonstrating the internal characteristics and the relationship of the tumor. [9]

Surgical excision is the treatment of choice. The approach to surgical excision for an infraorbital schwannoma depends on the location and size of the tumor. Conservative approaches such as the Caldwell Luc approach can be used in case of very limited and anteriorly localized tumors. They have the advantage of minimal morbidity and no facial scarring, but are hampered by limited superior and lateral tumor exposure. Large extensive lesions usually require a combination of approaches. Various approaches have been described such as the transfacial, Lefort I maxillary osteotomy approach, osteoplastic maxillotomy approach, midfacial degloving, and limited transzygomatic or limited rhytidectomy approach. [3] The transfacial approaches are suited for more medial based tumors with infratemporal extension laterally, but are disadvantaged by the associated facial scarring. The bilateral sublabial incision followed by Lefort I maxillary osteotomy approach; although, offering good exposure is limited by morbidity coupled with it. It requires maxillary bone suspension and intermaxillary blockage for 3 weeks. [10] The limited transzygomatic or limited rhytidectomy approach is more cosmetically acceptable and usually indicated for lesions involving upper infratemporal fossa tumors with medial extension into the orbit. [11] It is however hindered by limited anterior exposure. The midfacial degloving approach is suitable for medially based tumors. For larger extensive tumors, approaches such as the transfacial, limited rhytidectomy, bilateral sublabial incision followed by Lefort I maxillary osteotomy and the osteoplastic maxillotomy approach provide a panoramic view of the facial structures, but are associated with significant morbidity and cosmetic disfigurement.

   Conclusion Top

Schwannoma of the infra orbital nerve presenting as an upper jaw swelling is a rare entity. Only 11 cases have been reported in literature so far, with only 1 case from India. Despite its rarity schwannoma of the infraorbital nerve should be considered in the differential diagnosis of a painless slow growing cheek swelling. The Caldwell Luc approach is a safe, effective, and cosmetically acceptable approach for removal of infraorbital schwannoma and is highly recommended for anterior tumors. An appropriate diagnostic work up includes appropriate imaging and pre-surgical analysis to decide on the best surgical approach.

   References Top

1.Tezer MS, Ozcan M, Han O, Unal A, Ozlugedik S. Schwannoma originating from the infraorbital nerve: A case report. Auris Nasus Larynx 2006;33:343-5.  Back to cited text no. 1
2.Pereira LA, Choo YB. Schwannoma of the infraorbital nerve. Ear Nose Throat J 1979;58:236-9.  Back to cited text no. 2
3.Karkas AA, Schmerber SA, Bettega GV, Reyt EP, Righini CA. Osteoplastic maxillotomy approach for infraorbital nerve schwannoma, a case report. Head Neck 2008;30:401-4.  Back to cited text no. 3
4.Garg R, Dhawan A, Gupta N, D'souza P. A rare case of benign isolated schwannoma in the inferior orbit. Indian J Ophthalmol 2008;56:514-5.  Back to cited text no. 4
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5.Clarençon F, Jafari A, Lefevre M, Périé S, Angelard B, Marsault C, et al. Infraorbital nerve schwannoma. J Neuroradiol 2009;36:301-3.  Back to cited text no. 5
6.Yoshida K, Kawase T. Trigeminal neurinomas extending into multiple fossae: Surgical methods and review of the literature. J Neurosurg 1999;91:202-11.  Back to cited text no. 6
7.Sierszen ´ W, Stankiewicz C. Malignant schwannoma of infraorbital nerve. Otolaryngol Pol 2003;57:573-6.  Back to cited text no. 7
8.Abe T, Kawamura N, Homma H, Sasaki K, Izumiyama H, Matsumoto K. MRI of orbital schwannomas. Neuroradiology 2000;42:466-8.  Back to cited text no. 8
9.Anil G, Tan TY. CT and MRI evaluation of nerve sheath tumors of the cervical vagus nerve. AJR Am J Roentgenol 2011;197:195-201.  Back to cited text no. 9
10.Négrier B, Hadjean T, Brette MD, Klap P, Fabre A, Hadjean E, et al. Approach to the pterygomaxillary region by the Lefort I osteotomy. Ann Otolaryngol Chir Cervicofac 1985;102:487-91.  Back to cited text no. 10
11.Obwegeser HL. Temporal approach to the TMJ, the orbit, and the retromaxillary-infracranial region. Head Neck Surg 1985;7:185-99.  Back to cited text no. 11

Correspondence Address:
P Naina
Department of ENT, Christian Medical College, Vellore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9290.135940

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  [Figure 1], [Figure 2], [Figure 3]

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