Central Annals of Otolaryngology and Rhinology

Case Report *Corresponding author

İbrahim Murat Afat, Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Marmara Infraorbital Schwannomas: University, Istanbul, Turkey, Tel: 0090 216 421; Email:

Review of the Literature and Submitted: 10 October 2018 Accepted: 07 November 2018 Presentation of a Rare Case Published: 09 November 2018 ISSN: 2379-948X Ibrahim Murat Afat* Copyright © 2018 Afat Department of Oral and Maxillofacial Surgery, Marmara University, Turkey OPEN ACCESS Abstract Keywords Schwannomas are well-differentiated, benign tumours arising from the Schwann cells of the • Schwannoma nerve sheath. Schwannomas arising from the (ION) are very rare, and very • Infraorbital nerve few cases are reported in literature. The Review of the Literature covered all English literature between 1944 and January 2018. The keywords used in the search were ‘schwannoma’ or ‘neurinoma’ or ‘neurilemmoma’ and ‘infraorbital’ or ‘infra-orbital’. As a result, 10 cases of ION and branch case reports and 4 cases of malignant peripheral nerve sheath tumours with ION involvement were examined in full text. A rare case of a schwannoma arising from the medial superior labial branch of the ION and its treatment are presented.

INTRODUCTION Schwannomas or neurilemmomas are well-differentiated provide data on age, gender, location, presenting symptoms, size, benign tumours arising from the Schwann cells of the nerve andCASE treatment PRESENTATION methods. sheath with well-defined borders [1,2]. A 28-year-old male patient presented with a progressively The first description of this tumor was by Verocay in 1910,the enlarging, painless but tender-to-touch swelling over the right first trigeminal nerve schwannoma was reported by Smith in cheek. Physical examination showed a firm, subcutaneous 1835, who described a tumor of the gasserian ganglion [3]. 3-cm lump in the right infraorbital region that was not fixed to butmost of these originate from the lower cranial nerve and the skin or underlying (Figure1a). The nasolabial fold was Schwannomas are common in the head and neck region, shallowed. The skin was pinchable over the lesion. There was no proptosis, and vision was normal. There was no subjective sympathetic nervous system [4]. Schwannomas from the upper sensory deficit as compared with the left side, and cervical lymph cranial nerves, such as the trigeminal nerve and its branches, are nodes were not palpable. There was no significant family history lessThe common. infraorbital nerve (ION) is the continuation of the of other illnesses. There were no other similar lesions in the trunkIntra-oral or limbs. examination showed no swelling over the anterior maxillary nerve after it has entered the , through the infraorbital fissure, traversing the infraorbital canal to reach the . The maxillary anterior teeth were vital, firm, nontender face innervates the midface maxillary region. Most of the ION on percussion, and had no cavities. territory is supplied by the nasal and superior labial branches, Computed tomography (CT) showed a well-defined expansile which divide within the infraorbital canal. The nasal nerves hypodense lesion arising from the right maxillary region above comprise the external nasal nerve, which terminates in the ala the root of the maxillary anterior teeth. The overall clinical of the nose and the internal nasal nerve, which terminates in presentation was suggestive of a benign subcutaneous lesion, the nasal vestibule. The superior labial nerve has a medial and and the differential diagnosis included fibroma, lipoma, dermoid a lateral sub-branch, which terminates in the superior lip [5]. cyst, epidermoid cyst, and neurogenic tumour (Figure 1b). Schwannomas arising from the ION are very rare, and few cases The magnetic resonance imaging (MRI) scans showed well- are reported in the literature [2]. defined, heterogeneous oval mass of 3,5 × 3,0 × 2,3 cm within the Here we describe a case of a schwannoma of the ION, including buccal sub-cutaneous tissue with no orbital, or a review of the literature from 1944 to January 2018, and we underlying bone involvement. (Figure 1c, d). Cite this article: Afat IM (2018) Infraorbital Schwannomas: Review of the Literature and Presentation of a Rare Case. Ann Otolaryngol Rhinol 5(4): 1217. Afat (2018) Email: [email protected] Central

Surgical excision of the lesion was planned and executed under local anaesthesia. After intraoral vestibular incision at the time of exploration, the lesion was well-encapsulated and was freed from surrounding tissue by blunt dissection. The lesion was found to emanate from the nerve trunk of the peripheral branch of the ION (Figure 2a, b). labial branch of the ION. The mass was excised by sacrificing the involved medial superior Gross examination showed an oval-shaped, well- circumscribed tumour, apparently encapsulated, with a homogeneous surface, which was yellow-gray in color and of medium consistency. Figure 2 Intraoperative view a,b: shows a well-circumscribed, The patient reported paraesthesia localized over the area of encapsulated, pink mass that attached to the infraorbital nerve branch. distribution of the medial superior labial branch of the ION after surgery. Histopathological examination (hematoxylin and eosin staining) of the tan-colored specimen excised revealed a tumour composed of fascicles of monomorphic spindle- eosinophilic cytoplasm (Figure 3a). There was diffuse nuclear immunopositivityshaped cells with topointed S‑100 basophilicprotein, indicative nuclei and of apoorly schwannoma defined (Figure 3b). Diagnosis of a schwannoma, arising from the medial superior labial branch of the ION, was established. The patient had an Figure 3 uneventful recovery and continues to be free of recurrence one year after surgery. b: Intense and Histopathological diffuse reactivity findings. with anti-S- a: 100 Hematoxylin protein antibody. and eosin staining, original magnification×10, shows dense spindle shaped cells. Review of the Literature Library of Medicine (Medline PubMed). The search covered all The literature searches were performed using the National literature between 1944 to January 2018. Keywords used in the search were ‘schwannoma’ or ‘neurinoma’ or ‘neurilemmoma’

studies or reports in humans. No animal studies were included. and ‘infraorbital’ or ‘infra-orbital’. The search was confined to The search as; (Infra-orbital [All Fields] OR Infraorbital[All Fields]) AND (“neurilemmoma”[All Fields] OR “neurinoma”[All Fields] OR “schwannoma”[All Fields])yielded 29 results and 19 of them related to the subject. As a result, 10 cases of ION and branch case reports [1-4, 6-11] (Table 1), and 4cases of malignant peripheral nerve sheath tumours with ION involvement [12-15] (Table 2) were examined in full text. Five case reports published between 1961-1979 that

(Table 3). were not have abstract were identified from their title [16-20] DISCUSSION Head and neck tumours of neurogenic origin are relatively rare and constitute a heterogeneous group of neoplasms with vastly different histologic features, behaviours, and prognoses. A schwannoma is an ectodermal benign neoplasm arising from Schwann cells present in nerve sheaths [4]. These cells are neural crest-derived glial cells, providing myelin insulation to peripheral nervous system axons [21]. Of the 12 cranial nerves, only the olfactory and optic nerves do not develop schwannomas because they lack Schwann cells Figure 1 a: Preoperative photograph of the patient b: Computed in their sheaths. Schwannomas may be intracranial (central tomography scan. c,d: Coronal and Axial view magnetic resonance schwannomas) or located in the soft tissues (peripheral imaging scans of the facial area. schwannomas) [3].

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Table 1: Summarizes the features of the previously reported infraorbital nerve schwannomas. Author, year of Follow up Dura- Size of Surgical Ap- No. publica- Journal Age Sex Clinical Features Area Involved and Re- tion tumuor proach tion and currence region Paresthesia over the Right infraorbital Endoscopic Cham- region, extended endonasal Clinical 2 months pagnea with chewing. exoph- intracrani- 5.6 x3.1x approach, 1 Neurology and 48 male 6 years no recur- et.al 2014 thalmia,right cheek, upward difficulty gaze ally centeredon 6 cm endoscopic Neurosurgery rence. Canada - pterygopalatine maxillary ocular diplopia. fossa. meatotomy. Paresthesiadeficit and vertical over the bin Pterygopalatine Weber Fergus- Indian Journal of Sasindran right infraorbital re- fossa, extended son incision, 1 months Otolaryngology 6 2 et.al 2008 22 male gion. Diminished vision into infratem- n/a transantral ap- no recur- and Head & Neck months India poral fossa and proach partial rence. Surgery nasal obstruction. maxillectomy. Boneand corneal erosion reflex of the and infraorbital canal and floor of the orbit Clarençon - Journal of fe- - 3 et.al 2009 45 n/a Infraorbital canal n/a ber Fergusson n/a Neuroradiology male lar globe and inferior France incision.Modified We rectusorbital musclefloor. Left were ocu displaced upwards. Kok et.al Right nasolabial 2 years Annals of Plastic Right nasolabial subcu- 3 2.3x 2.1x Sublabial in- 4 2013 Singa- 12 male subcutaneous no recur- Surgery taneous lump months 1.7 cm traoral incision. pore lump rence 3 years Nainaet.al Indian Journal of Painless swelling over 6 Right maxillary Caldwell Luc 5 26 male 5 x3 cm no recur- 2014 India Dental Research right cheek. months sinus approach. rence Slow growing sub-cu- Journal of Natural Sub-cutaneous Kumar taneous nodular swell- 6 Science, Biology 40 male 1 year tissue of leftch- Skin incision. n/a 2015 India ing and mid paresthe- and Medicine eek. sia over left cheek. 4,5 × 3,0 Painless, slowly pro- × 2,3 cm Mora- gressive mass above Anterior and 4 months Cirugía y Subdermal ap- 7 Ríos 2014 32 male the left lacrimal sac. 4 years inferior to the left no recur- Cirujanos cm proach. México Hypoesthesia of the globe 1,9 × 1,1 rence. left cheek. Right nasal fossa, Weber Fergus- No hypoesthesia. maxillary sinus, son incision Right exophthalmos Karkas with subciliary 7 months with bulging of the 1.5 8 et.al 2008 Head & Neck 14 male pterygopala- extension fol- no recur- cheek and . months; 4,3 cm France tineorbital fossa, floor, and lowed by maxil- rence. Headache and nasal 5,95 × infratemporal lozygomatic obstruction. fossa. osteotomy. Painless, slowly grow- Eyelid crease ing non-tender, freely Garg et.al Indian Journal of fe- Right inferior incision fol- 9 35 mobile mass in the 4 years n/a 2008 India Ophthalmology male orbit lowed by inferi- inferior orbit no visual 2,5 × 2,0 or orbitotomy. acuity, no diplopia × 1,2 cm Bulging and tender- Tezer et.al 12 months Auris Nasus fe- ness in left inferior 6 Left inferior Subciliary inci- 10 2006 Tur- 16 norecur- Larynx male eyelid, eyeball was months eyelid. sion. key rence. pushed superiorly. 3 × 4 cm Abbreviation: NA: Data not available

The vestibular nerve is the most frequent site of origin in the The ION, an entirely sensory nerve, is the terminal branch of head and neck region schwannomas [22]. . Extracranial cranial the maxillary nerve, the second division of the trigeminal nerve nerve V (trigeminal) involvement appears to be less frequent [24]. than cranial nerves IX, X, XI, andXII [23]. Previously reported ION schwannomas, depending on the Trigeminal schwannomas may originate from any part of the site of the nerve trunk or the branch from which it arises,located nerve roots or branches. However, those from the extracranial withinthe lower part of the orbit [1, 6-11] or the maxillary sinus [2, branches of the nerves are less frequent [10]. 8], or may present as a subcutaneous mass in the malar region [3,

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Table 2: Summarizes the features of the previously reported malignant peripheral nerve sheath tumours with infraorbital nerve involvement. Author and Age/ Clinical Infraorbital nerve Tereat- No. Diagnosis year of publi- Journal Area Involved Survival Sex Features involvement ment cation Malignant epithe- Residual tumor was Journal Progressive Both V3 cranial Surgery lioid cra- Fisher and Den- 41 present at the cut Died after 7 1 of Neuro- left facial nerve and the distal and radio- nial nerve nis 2006 Canada male end of the infraor- years Oncology weakness VII’th nerve. therapy. sheath bital nerve, tumor Radical Malignant resection periph- Crani- Ulcerative Process of the max- eral nerve omaxil- illa involving the neck dis- sheath D’Addino et.al lofac 78 Died after 18 2 skin lesion, Origin of tumor section,modified tumor, 2016 Trauma male months inabilityinfiltrated to and superior maxilla chemo- malignant Recon- open the eye bone. therapy schwan- struction via doxo- noma rubicin First Malignant chemo- schwan- Otolaryn- 2 year English Ab- therapy, 3 noma of Stankiewic golo- disease-free stract, (lang: Pol) in second infraorbital 2003Sierszeń and giaPolska survival. surgical nerve excision Ra- diotherapy Malignant Extended through was not periph- 53 male English the orbit and the complete Morujo et.al Neuro- Involving the infra- Died after 4 eral nerve Abstract, (lang: base of the , and radical 2014 Spain cirugia orbital nerve 10months sheath tu- Span) progressing intrac- surgical mour ranially. resection was im- possible.

Table 3: Summarizes the features of the previously reported case reports that were not have. No. Author and year of publication Journal Reasons for exclusion 1 Emerick 1965 Laryngoscope No abstract 2 Kahl 1973 Zeitschrift Fur Neurologie No abstract in Eng. 3 Artenie and Muntenescu 1961 Rumanian Medical Review No abstract in Eng. 4 Batiunin 1962 VestnikOtorinolaringologii No abstract in Eng. 5 Pereira and Choo 1979 Ear, Nose & Throat No abstract

4]. Lesions arising from the ION grow to involve the surrounding The mean age of the previously reported ION schwannoma structures, such asthe paranasal sinuses [2, 8], the nasal cavity cases was 29. The male/female ratio was 7:3. [8], the infratemporal fossa [8, 10], and the Schwannomas typically appear as isodense or slightly [1, 8, 10]. In our case, the schwannoma was located in the right hyperdense and are uniformly enhancing on CT. MRI imaging cheek. usually reveals low signal intensity on T1-weighted images and Although a schwannoma is a painless lesion, the pressure of high signal intensity on T2-weighted images. In our patient, the tumour on an adjacent nerve may cause paraesthesia and tendernes. Degenerative changes can be seen in some older the literature [9], presenting as a homogeneous infraorbital tumours [25]. tomographic findings were very similar to those reported in parenchyma without apparent cyst formation. Swelling in the cheek, paraesthesia, nasal atresia, bone tumour of well-defined margins that is isodense to the cerebral resorption, downward transversion of the palate, tenderness, Unlike intracranial trigeminal nerve schwannomas, which are exophthalmos, and diplopia were common symptoms in previously reported ION schwannoma case reports. However, prognosis, patients presenting with extracranial schwannomas in our case, a well-localized, painless infraorbital subcutaneous tendoften to characterized have good prognoses. by difficulty in complete removal and poor swelling was observed without any ocular lesions or the The treatment of extracranial head and neck schwannomas involvement of the underlying osseous skeleton. is exclusively surgical, and its surgical approach is dictated by

Ann Otolaryngol Rhinol 5(4): 1217 (2018) 4/6 Afat (2018) Email: [email protected] Central the size, extent, and anatomical location of the tumour. Various an infraorbital nerve schwannoma. Clin Neurol Neurosurg. 2014; 119: surgical incisions have been described for tumours in this 106-109. location. 2. Naina P, D Masih, SS Mathews, Infraorbital nerve schwannoma: a rare cause of upper swelling. Indian J Dent Res. 2014; 25: 263-265. Approaches used in previous reports of ION schwannomas include; intra-oral vestibular incision [3]or incision placed in the 3. Kok YO, Yeo MS, Nallathamby V, Lee SJ. Infraorbital nerve schwannoma natural skin crease of the nasolabial fold [4] for lesions localized presenting as an upper lip mass in an adolescent boy. Ann Plast Surg. in the buccal space, the Caldwell-Luc approach for tumours within 2013; 71: 196-197. the maxillary sinus [2],and the endoscopic endonasal approach 4. Kumar N. Infra-orbital nerve schwannoma: Report and review. J Nat for tumours centered on the pterygopalatine fossa [1], subcilliary Sci Biol Med. 2015; 6: 267-271. [11], eyelid crease incision [7],or the subdermal approach [9] for 5. Nderitu JM, Butt F, Saidi H. Variant Anatomy of the Nasal and intraorbital schwannomas. Larger lesions involving the greater Labial Branches of the Infraorbital Nerve. Craniomaxillofac Trauma areas of the mid-face were treated via Weber Furgusson incisions Reconstr. 2016; 9: 294-296. combined with ostectomies [6, 8, 10]. We preferred in our case 6. Clarencon F. Infraorbital nerve schwannoma. J Neuroradiol. 2009; an intra-oral approach which is a safe, effective, and cosmetically 360: 301-303. acceptable method. 7. Garg R, Dhawan A, Gupta N, D’souza P. A rare case of benign isolated Torossian et al. reported that of 15 extracranial cephalic schwannoma in the inferior orbit. Indian J Ophthalmol. 2008; 56: 514- schwannomas treated with a nerve-sparingtechnique, which 515. consisted of enucleation after opening the epineurium using an 8. Karkas AA. Osteoplastic maxillotomy approach for infraorbital nerve operating microscope without interruption of the continuity schwannoma, a case report. Head Neck. 2008; 30: 401-404. patients were operated on without a microscope [26]. We chose 9. Mora-Rios LE. Infraorbital schwannoma. Case report. Cir Cir. 2014; of the nerve, they observed only two recurrences: the first two 82: 76-80. which the tumour originated, in hope of eliminating the risk of 10. Sasindran V. Giant tumour of the pterygopalatine fossa. Indian J recurrenceto sacrifice theand small malignant and relatively transformation. insignificant part of the nerve, Otolaryngol Head Neck Surg, 2008; 60: 248-251. The term ‘malignant schwannoma’ is used for malignant 11. Tezer MS. Schwannoma originating from the infraorbital nerve: a case report. Auris Nasus Larynx. 2006; 33: 343-345. schwannoma variants, which are included in the group of malignant peripheral nerve sheath tumours. Malignant 12. D’Addino JL. Malignant Peripheral Nerve Sheath Tumor of the transformation of schwannomas in head and neck region is rare Infraorbital Nerve. Craniomaxillofac Trauma Reconstr. 2016; 9: 170- [27]. 174. Our literature review yielded only 4 cases of malignant 13. Gonzalez-Orus Alvarez-Morujo R. Malignant peripheral nerve sheath tumour of the infra-orbital nerve]. Neurocirugia (Astur). 2014; 25: peripheral nerve sheath tumours with ION involvement [12- 240-243. 15]. Malignancy may be suggested on the basis of rapid growth 14. Fisher, B.J. and K.E. Dennis, Malignant epithelioid cranial nerve sheath histopathological diagnosis by features such as diffuse cellular tumor: case report of a radiation response. J Neurooncol, 2006; 78: p. 173-177. atypia,or extensive p53 positivity, nerve involvement and high MIB1 but staining can be confirmed[2]. only by 15. Despite its rarity, an ION schwannoma may be considered a nerve]. Otolaryngologia polska. The Polish otol. 2003; 57: 573-576. possible diagnosis in the case of an indolent infraorbital mass. Sierszeń WC. Stankiewicz. Malignant schwannoma of infraorbital Multislice CT and MRI are useful for making a positive diagnosis 16. Artenie VM, Muntenescu. On a case of right infraorbital invading schwannoma. Rum Med Rev.1961. 5: 84-86. at this unusual location for schwannoma, and can also serve as a guide during surgical removal of the mass [6]. 17. Batiunin LT. On neurinoma of the infraorbital nerve. Vestn Otorinolaringol. 1962; 24: 99-101. CONCLUSION 18. Emerick CW. Neurilemmoma of the infraorbital nerve. Laryngoscope; Schwannoma of the ION, especially in the extraosseous 1965; 75: 820-822. location, presenting as upper jaw swelling, is a rare entity, 19. Kahl RI. Neurinoma of the infraorbital nerve. Z Neurol. 1973; 204: 155-158. schwannoma have been reported in English literature, and the 20. Pereira LA, Choo YB. Schwannoma of the infraorbital nerve. Ear Nose intraoraloften making vestibular a clinical incision diagnosis was attempted difficult. inOnly only 10 1 case.cases Despite of ION Throat J. 1979; 58: 236-239. its rarity, a schwannoma of the ION should be considered in the differential diagnosis of a painless, slow-growing cheek swelling. 21. Handschel J, Heikaus S, Depprich R, Kübler NR, Yekta SS, Smeets R, et An intra-oral approach is a safe, effective, and cosmetically al. Intraoral schwannoma: review of the literature and presentation of a rare case. Cranio. 2012; 30: 150-153. acceptable method for removal of an infraorbital schwannoma without incurring external scars, and it is highly recommended 22. Berlucchi M, Piazza C, Blanzuoli L, Battaglia G, Nicolai P. Schwannoma for anteriorly located tumours. of the nasal septum: a case report with review of the literature. Eur Arch Otorhinolaryngol. 2000; 257: 402-405. REFERENCES 23. Yang SW, Lin CY. Schwannoma of the upper lip: case report and 1. Champagne PO, Desrosiers M, Moumdjian R. Endoscopic resection of literature review. Am J Otolaryngol, 2003; 24: 351-354.

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24. Hu KS, Kwak J, Koh KS, Abe S, Fontaine C, Kim HJ. Topographic 26. Torossian JM, Beziat JL, Abou Chebel N, Devouassoux-Shisheboran M, distribution area of the infraorbital nerve. Surg Radiol Anat. 2007; 29: Fischer G. Extracranial cephalic schwannomas: a series of 15 patients. 383-388. J Craniofac Surg. 1999; 10: 389-394. 25. Moradzadeh M. Schwannoma in the midline of hard palate: a case 27. Kun Z, Qi DY, Zhang KH. A comparison between the clinical behavior report and review of literature. J Dent Res Dent Clin Dent Prospects. of neurilemmomas in the neck and oral and maxillofacial region. J Oral 2014; 8: 114-117. Maxillofac Surg. 1993; 51: 769-771.

Cite this article Afat IM (2018) Infraorbital Schwannomas: Review of the Literature and Presentation of a Rare Case. Ann Otolaryngol Rhinol 5(4): 1217.

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