Schwannoma of the Nasal Septum

Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2016) xxx, xxx–xxx

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CASE REPORT Schwannoma of the nasal septum

Abdullah Karatas a,*, Isil Taylan Cebi a, Mehti Salviz b, Ayhan Kocak c, Tuba Selcuk c a Haseki Training and Research Hospital, ENT Clinic, Fatih, Istanbul, Turkey b Yeni Yuzyil University, Faculty of Medicine, ENT Clinic, GOP, Istanbul, Turkey c Haseki Training and Research Hospital, Radiology Clinic, Fatih, Istanbul, Turkey

Received 24 February 2016; accepted 2 June 2016

KEYWORDS Abstract Schwannomas are benign and slow growing tumors originating from the Schwann cells Nasal septum; of peripheral nerve sheath. Schwannomas of sinonasal origin are rare (4%) however septal schwan- Schwann cell; nomas are much more rarer. We presented a 31 year old female patient. At physical examination a Schwannoma pale gray, smooth polypoid lesion obstructing the right nasal cavity was detected. Midfacial degloving and endoscopic approach were combined for surgical treatment. The tumor was originating from posteromedial area of the septal nasal cartilage, close to the bony cartilaginous junction. Post- operative histological examination of the specimen showed a benign tumoral growth consisting of spindle shaped cells and immunohistochemical staining of the tumor proved septal schwannoma. Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/ by-nc-nd/4.0/).

1. Introduction 2. Case presentation

Schwannomas are benign and slow growing tumors originat- 31 year old caucasian female patient presented to Haseki ing from the Schwann cells of peripheral nerve sheath. Training and Research Hospital in June 2015. The main symp- Twenty-ﬁve to forty-ﬁve percent of the extracranial schwanno- toms were nasal obstruction, headache, facial pain, anosmia mas are located at the head and neck region.1 Among these and intermittent bloody nasal discharge. Nasal obstruction most common ones are the vestibular schwannomas of internal started eight months ago and increased in last six months, acoustic meatus. Schwannomas of sinonasal origin are rare resulting in headaches, facial pain, anosmia and intermittent (4%) however septal schwannomas are much more rarer.2 bloody nasal discharge eventually. There was no special condi- tion in family history, also no comorbid diseases and trauma history. At physical examination a pale gray, smooth polypoid lesion obstructing the right nasal cavity was detected. Endo- * Corresponding author at: Haseki Training and Research Hospital, scopic examination of the left nasal cavity revealed a septal ENT Clinic, Millet Caddesi, Fatih, Istanbul, Turkey. Mobile: +90 532 perforation of 5 Â 10 mm at the posterior nasal septum, 623 02 18 26; fax: +90 212 529 44 00. through which the tumor passed from right to the left nasal E-mail addresses: [email protected] (A. Karatas), drisiltaylan@- cavity. hotmail.com (I.T. Cebi), [email protected] (M. Salviz), dr.ayhan- Computed tomography (CT) (Brilliance CT, Philips, Ams- [email protected] (A. Kocak), [email protected] (T. Selcuk). terdam, the Netherlands) of the paranasal sinuses revealed a Peer review under responsibility of Egyptian Society of Ear, Nose, soft tissue mass expanding and remodeling the superior and Throat and Allied Sciences. http://dx.doi.org/10.1016/j.ejenta.2016.06.001 2090-0740 Ó 2016 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Please cite this article in press as: Karatas A et al. Schwannoma of the nasal septum. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j. ejenta.2016.06.001 2 A. Karatas et al.

Figure 1 Paranasal sinus CT sections; A – coronal B – axial.

Figure 2 Paranasal sinus MRI sections; A – T1A coronal, B – T1A coronal contrast enhanced, C – T1A axial contrast enhanced.

inferior conchae and the medial wall of the maxillary sinu¨ s, cavity did not invade the surrounding soft tissues and origi- leading to the obstruction of right nasal cavity (Fig. 1A and B). nated from the posteromedial area of the septal nasal cartilage, T1-hypointense and T2-hyperintense mass lesion of close to the bony cartilaginous junction. Nasal septal perfora- 47 Â 17 mm at right nasal cavity showing postcontrast tion was observed right anterior to this site. Perpendicular enhancement was detected by the magnetic resonance imaging plate of the ethmoid bone and septal cartilage near the tumor (MRI) (Philips Achieva 1.5T, Philips Medical Systems, Best, were excised along with the tumor. The Netherlands) of the paranasal sinuses (Figs. 2A–C and Postoperative histological examination of the specimen 3A and B). showed a benign tumoral growth consisting of spindle shaped Diagnostic incisional biopsy was performed and with Ki- cells with round nuclei, arranged in interlacing fascicles nested 67, immunohistochemical nuclear staining was 5%. These in histiocyte groups. Immunohistochemical staining for differ- histopathological ﬁndings suggested a benign mesenchymal ential diagnosis of the nerve sheath tumors demonstrated tumor growth. vimentin(+), CD68(+) (in histiocytes), CD34(À), SMA(À), Midfacial degloving and endoscopic approach were com- Desmin(À), S-100(+), EMA(À), PANC(À) hence the diagno- bined for surgical treatment. The tumor in the right nasal sis was conﬁrmed as schwannoma (Fig. 4).

Figure 3 Paranasal sinus MRI sections; A – T2A axial, B – T2A saggital.

3. Discussion were evaluated by a multicentered study and inverted papil- loma, osteoma and juvenila nasopharyngeal angiofibroma were found to be the most common benign tumors. Nasal Schwannomas are mostly benign tumors deriving from the 7 Schwann cells in periferic nerve sheath. The incidence of schwannoma was seen only at seven patients. malign schwannomas are 2%.3 Schwannomas may arise from Schwannomas, perineuromas, neurofibromas, myxomas peripheral motor, sensorial, sympathetic nerves and also from and granular cell tumors are called as peripheral nerve sheath cranial nerve sheath. tumors which develop from schwann cells, perineural cells and Twenty-five to forty-five percent of the schwannomas are neural fibroblasts. Differential diagnosis of these tumors are 1 made by immunohistochemical staining. S-100 protein expres- located at head and neck region. The incidence of schwanno- 8 mas at sinonasal area is 4% and mostly at ethmoid sinuses fol- sion is diagnostic for schwannoma, diagnosis was confirmed lowed by maxillary sinuses, nasal cavity and sphenoid sinuses.3 likewise in our case. Schwannomas of nasal septum are extremely rare and there The location and size of the lesion defines the surgical have been approximately 20 patients in western literature.4 approach. In our case midfacial degloving was preferred due Usually schwannomas arise from large peripheral nerve to the size of the tumor and better cosmetic results. After the fibers such as vestibulocochlear nerve, vagal nerve and cervical total excision of schwannoma, posterior nasal cavity was sympathetic trunk.4,5 However in our case due to the localiza- inspected for tumor residue by endoscopic approach. Preoper- tion of the tumor; it may be considered to derive from small ative complaints of our patient has ceased in two months after somatosensorial nerve fibers of nasal septum (e.g. nasopalatine the surgery. The follow up is done every two months by nasal or nasociliary nerves). endoscopy. No recurrence is detected up to date. Since eight Slowly increasing nasal obstruction and related symptoms such as nasal/postnasal discharge, intermittent epistaxis and headache are of primary importance in patient history. Large nasal tumors may cause cosmetic problems. In our case nasal obstruction, headache and bloody nasal discharge were the main symptoms. Unilateral tumoral mass in the right nasal cavity was detected by physical examination and imaging (CT and MRI). No destruction was seen in nearby bony tissues. Remodeling of maxillary sinu¨ s medial wall implicated a benign lesion, no orbital or intracranial extension was detected. Fasci- cular sign, seen at MRI, implies a lesion of neurogenic origin. It is characterized by multiple small ring-like structures with peripheral hyperintensity at T2 intense MRI images. The lesion in our case did not exhibit fascicular sign. MRI is espe- cially valuable for evaluation of the intracranial extension of schwannomas derived from nasoethmoid roof.6 Unilateral nasal obstruction is the most common symptom in patients with either benign or malignant tumors of the sino- Figure 4 Positive immunohistochemical staining for S-100 nasal region. In Italy 931 patients with benign nasal masses protein.

Please cite this article in press as: Karatas A et al. Schwannoma of the nasal septum. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j. ejenta.2016.06.001 4 A. Karatas et al. month follow up duration is not adequate we continue the 3. Frosch MP, Anthony DC, De Girolami U, et al. Patologia: Bases bimonthly follow-ups. Patolo´gicasdas Doenças. 7th ed. Rio de Janeiro: Elsevier; 2005, 1479–1480. 4. Cadd B, Offiah C, Alusi G. A surprising cause of unilateral nasal Conflict of interest obstruction and epistaxis: nasal septal schwannoma. J Surg Case Rep.. 2014;21:2014. There is no conflict of interest. 5. D’Alessandro G, Di Giovanni M, Iannizzi L, et al. Epidemiology of primary intracranial tumors in the Valle d’Aosta (Italy) during the Source of funding 6-year period 1986–1991. Neuroepidemiology. 1995;14(3):139–146. 6. Fujiyoshi F, Kajiya Y, Nakajo M. CT and MR imaging of nasoethmoid schwannoma with intracranial extension. Am J There are no financial relations. Roentgenol.. 1997;169(6):1754–1755. 7. Nicolai P, Castelnuova P. Benign tumors of the sinonasal tract. In: References Flint PW, Haughey BH, eds. Cummings Otolaryngology. 6th ed. Canada: Saunders an imprint of Elsevier Inc.; 2015:740–752. 8. Azani AB, Bishop JA, Thompson LD. Sinonasal tract neurofi- 1. Wada A, Matsuda H, Matsuoka K, et al. A case of schwannoma on broma: a clinicopathologic series of 12 cases with a review of the the nasal septum. Auris Nasus Larynx.. 2001;28(2):173–175. literature. Head Neck Pathol. 2015 Sep;9(3):323–333. 2. Luchi GER, Magalha˜es MR, Lanzelotti SM, et al. Nasal schwannoma. Braz J Otorhinolaryngol.. 2006;72(5):714.

Please cite this article in press as: Karatas A et al. Schwannoma of the nasal septum. Egypt J Ear Nose Throat Allied Sci (2016), http://dx.doi.org/10.1016/j. ejenta.2016.06.001