Schwannoma of nasal septum: A rare case report with literature review

5
Schwannoma of nasal septum: A rare case report with literature review Bhaskar Mitra a, * , Sharmistha Debnath b , Biswanath Paul a , Mallika Pal a , Tapan Jyoti Banerjee c , Tarak Nath Saha a a Department of Pathology, Midnapore Medical College & Hospital, Paschim Medinipur, West Bengal, India b Department of Oncopathology, Medical College Kolkata, West Bengal, India c Institute of Child Health, Kolkata, West Bengal, India Received 27 June 2012; accepted 10 August 2012 Available online 7 September 2012 KEYWORDS Schwannoma; Nasal septum; Nerve sheath tumor Abstract Schwannomas are benign tumors of nerve sheath and quite uncommon in the nasal sep- tum. In contrast to the earlier reports in the literature, a confounding case of a nasal septal schwan- noma presenting as a symptomatic growth on the left side of nasal septum in a 24-year-old man is discussed here. ª 2012 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. All rights reserved. 1. Introduction Schwannomas (neurilemmomas or neurinomas or perineural fibroblastoma) are benign encapsulated nerve sheath neo- plasms composed of Schwann cells first described by Verocay in 1908. 1 Stout (1935) coined the term neurilemmoma believing that this tumor arose from cells of sheath of Schwann (neuri- lemmoma) which may also develop in any part of the body. This tumour most frequently originating from the acoustic nerve in the head and neck region, 45% of all schwannomas occur in body and 25% as extracranial schwannomas. 2 It has also been observed in the neck, pharynx, larynx, scalp, face, oral cavity, middle ear, and internal auditory canal. However, involvement of the nasal cavity, paranasal sinuses and espe- cially the nasal septum is rare. Incidence is 1 in 3000 schwan- nomas. 3 Tumors arising from nasal septum are extremely rare with only 16 cases having been reported in the literature. 4–11 A case of neurilemmoma of nasal septum was first described by Bogdasanian and Stout. 5 2. Case details A 24-year-old man was referred to our department with a 6- month history of progressive left-sided nasal obstruction, rhin- orrhoea, epistaxis. There was no history of anosmia, facial pain, headache and recent nasal trauma. Patient was neither suffering from any comorbid diseases nor reported any such family history. Anterior rhinoscopy revealed a large polypoid mass almost completely filling the left nasal cavity. The polyp was firm in consistency and appeared to be covered by normal * Corresponding author. Address: 54/2/G, Feeder Road, P.O. Belgharia, 700056 Kolkata, West Bengal, India. Tel.: +91 9874174040. E-mail address: [email protected] (B. Mitra). Peer review under responsibility of Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2012) 13, 121125 Egyptian Society of Ear, Nose, Throat and Allied Sciences Egyptian Journal of Ear, Nose, Throat and Allied Sciences www.ejentas.com 2090-0740 ª 2012 Egyptian Society of Ear, Nose, Throat and Allied Sciences. Production and hosting by Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.ejenta.2012.08.002

Transcript of Schwannoma of nasal septum: A rare case report with literature review

Page 1: Schwannoma of nasal septum: A rare case report with literature review

Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2012) 13, 121–125

Egyptian Society of Ear, Nose, Throat and Allied Sciences

Egyptian Journal of Ear, Nose, Throat and Allied

Sciences

www.ejentas.com

Schwannoma of nasal septum: A rare case report

with literature review

Bhaskar Mitraa,*, Sharmistha Debnath

b, Biswanath Paul

a, Mallika Pal

a,

Tapan Jyoti Banerjeec, Tarak Nath Saha

a

a Department of Pathology, Midnapore Medical College & Hospital, Paschim Medinipur, West Bengal, Indiab Department of Oncopathology, Medical College Kolkata, West Bengal, Indiac Institute of Child Health, Kolkata, West Bengal, India

Received 27 June 2012; accepted 10 August 2012Available online 7 September 2012

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KEYWORDS

Schwannoma;

Nasal septum;

Nerve sheath tumor

Corresponding author.

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Abstract Schwannomas are benign tumors of nerve sheath and quite uncommon in the nasal sep-

tum. In contrast to the earlier reports in the literature, a confounding case of a nasal septal schwan-

noma presenting as a symptomatic growth on the left side of nasal septum in a 24-year-old man is

discussed here.ª 2012 Egyptian Society of Ear, Nose, Throat and Allied Sciences.

Production and hosting by Elsevier B.V. All rights reserved.

1. Introduction

Schwannomas (neurilemmomas or neurinomas or perineural

fibroblastoma) are benign encapsulated nerve sheath neo-plasms composed of Schwann cells first described by Verocayin 1908.1 Stout (1935) coined the term neurilemmoma believing

that this tumor arose from cells of sheath of Schwann (neuri-lemmoma) which may also develop in any part of the body.This tumour most frequently originating from the acousticnerve in the head and neck region, 45% of all schwannomas

: 54/2/G, Feeder Road,

st Bengal, India. Tel.: +91

il.com (B. Mitra).

yptian Society of Ear, Nose,

g by Elsevier

ar, Nose, Throat and Allied Scien

8.002

occur in body and 25% as extracranial schwannomas.2 It hasalso been observed in the neck, pharynx, larynx, scalp, face,oral cavity, middle ear, and internal auditory canal. However,

involvement of the nasal cavity, paranasal sinuses and espe-cially the nasal septum is rare. Incidence is 1 in 3000 schwan-nomas.3 Tumors arising from nasal septum are extremely rare

with only 16 cases having been reported in the literature.4–11 Acase of neurilemmoma of nasal septum was first described byBogdasanian and Stout.5

2. Case details

A 24-year-old man was referred to our department with a 6-

month history of progressive left-sided nasal obstruction, rhin-orrhoea, epistaxis. There was no history of anosmia, facialpain, headache and recent nasal trauma. Patient was neither

suffering from any comorbid diseases nor reported any suchfamily history. Anterior rhinoscopy revealed a large polypoidmass almost completely filling the left nasal cavity. The polypwas firm in consistency and appeared to be covered by normal

ces. Production and hosting by Elsevier B.V. All rights reserved.

Page 2: Schwannoma of nasal septum: A rare case report with literature review

Figure 1 CT scan image of the nasal septal mass.

Figure 2 Photomicrograph showing low power schwannoma lying just beneath the septal cartilage[A–D], with the presence of Verrucay

Bodies[C] and surrounding mucosal glands[D]. H&E 10·.

122 B. Mitra et al.

Page 3: Schwannoma of nasal septum: A rare case report with literature review

Figure 3 Photomicrograph shows both high cellular density and palisading pattern of the tumor cells [Antoni A][B &D], lower cellular

density of the tumor cells with a loose stroma [Antoni B][A&C]. Verrucay Bodies are present [B] without any evidence of ancient or

malignant changes.

Schwannoma of nasal septum: A rare case report with literature review 123

nasal mucosa. It bleeds on touch. The attachment of the polyp

was difficult to determine. The nasal septum was slightly devi-ated to opposite side and right nasal cavity was clear. A CTscan of the paranasal sinuses was advised to determine the nat-

ure and extent of the polyp. This showed a homogeneous massmeasuring 30 · 16 · 15 mm, lying within the midportion of theleft nasal cavity, arising from the left side of nasal septum

(Fig. 1). The lesion was well defined with smooth marginsand without calcification (Fig. 2). The paranasal sinuses wereclear. A contrast study was not performed due to the absenceof bony destruction. The patient agreed and consented to un-

dergo removal of the polyp under general anesthesia. The pol-ypoid mass was attached to middle part of the left side of thenasal septum, opposite the anterior end of the middle turbi-

nate. The mass was excised completely along with a cuff of sep-tal mucoperichondrium. Histopathological examination showsboth spindle shaped schwann cell rich area with nuclear pali-

sading(Antoni A) and schwann cell poor loose myxoidareas(Antoni B). Verocay Bodies are present without any evi-dence of ancient or malignant changes. The histopathologicaldiagnosis was benign schwannoma (Figs. 2 and 3). Immuno-

histochemistry shows tumor cells are strongly positive for S-100 protein (Fig. 4) confirming the diagnosis and distinguishesneurilemmoma from neurofibroma The patient was under fol-

low up for six months and there was no recurrence at sixmonths.

3. Discussion

The neural origin of the schwannomas is considered to be fromperipheral motor, sensory, sympathetic and cranial nerve

sheath.4,7 The optic and olfactory cranial nerves are not poten-tial sites of the origin, since they lack sheaths that contain

schwann cells and since nasal schwannomas are sometimes re-

moved without loss of original nerve functions, it is usually dif-ficult to determine the neural origin. Nasal schwannomas arepresumed to be arising from the sheath of the ophthalmic

and maxillary branches of the trigeminal nerve and autonomicganglia.12 Schwannomas of the sinonasal tract are very infre-quent, representing less than 4% of the schwannomas of the

head and neck.9 In this location they have been reported in pa-tients between the ages of 6 years and 78 years. There is no sexor racial predilection.8 The ethmoidal sinus is most commonlyinvolved, followed by the maxillary sinus, nasal fossa and

sphenoid sinus.9,12,13 Localization to the nasal septum is exceed-ingly rare. Septal schwannomas arise from the autonomic orsensory nerves within the nasal septum. There is no apparent

site predilection on the septum.Symptoms are non-specific and are the result of the mass ef-

fect or tumor necrosis. Patients may present with nasal

obstruction, rhinorrhoea, or recurrent epistaxis, as for this pa-tient. Nasal obstruction is the most common clinical symptomfollowed by epistaxis.4,8,14 Facial swelling and pain are associ-ated with paranasal sinus involvement. These lesions rarely un-

dergo malignant transformation.Computerized tomography(CT) delineates an image of the

soft tissue tumor and simultaneously outlines the skeletal mar-

gins well enough to rule out invasion and demonstrated centrallucency and peripheral enhancement after contrast administra-tion in case of schwannomas because peripheral neovascular

areas of the tumor are enhanced in contrast with nonenhanc-ing necrotic or cystic regions.15 Although magnetic resonanceimaging (MRI) is superior in defining soft tissue tumors, CT

offers better resolution of bony invasion. However, as benignschwannoma can erode bone by pressure, bony erosion isnot a criterion for malignancy. 16 With either approach, finaldiagnosis rests solely on the histologic examination. Micro-

Page 4: Schwannoma of nasal septum: A rare case report with literature review

Figure 4 Immunohistochemistry shows tumor cells are strong

positive for S-100 protein.

124 B. Mitra et al.

scopically, schwannomas can exhibit two architectural pat-terns, Antoni type A and Antoni type B, in different propor-tions. Antoni type A area is composed of an organized

compact cellular stroma with elongated spindle cells. Parallelrows of palisading nuclei can be seen in this highly differenti-ated tissue. Antoni type B area is composed of disorganized

loose myxoid stroma with few spindle cells. Additionally,schwannomas usually show intense immunostaining for S100protein (particularly Antoni A areas),17 which may help to dis-tinguish peripheral nerve sheath neoplasm from other tumors.

The nasal septum appears to be the most common locationfor this lesion. The main differential diagnoses of nasal masseswith similar MRI/CT findings as schwannoma include nasal

polyp, malignant peripheral nerve sheath tumors (MPNST),myxoma & fibromyxoma, Lobular Capillary hemangioma(LCH) and sarcoma. Nasal polyps represent hyperplasia of

the mucosa in response to chronic inflammation, usually fromchronic sinusitis. Nasal polyps usually have high signal inten-sity on T2-weighted images, which helps to distinguish themfrom tumors. Myxoma & fibromyxoma are benign neoplasm

of uncertain histogenesis with characteristic histologic appear-ance. When relatively a greater amount of stromal collagen ifpresent, the term fibromyxoma (or myxofibroma) is used. His-

tologically these tumor show scanty, loose cellular prolifera-tion containing spindle-shaped or stellate-appearing cellsembedded in abundant mucinous stroma. This tumor can

manifest as a well-marginated mass but may also show localinfiltration and local invasion.17 In patients with a known his-tory of NF, a nasal mass with such MRI/CT findings provides

a clue for this diagnosis of schwannoma,18 which requires con-firmation by histology. MPNS tumors show features of malig-nancy, high mitotic count and with or without heterologouselements which will be lacking in schwannomas. Other sarco-

mas similarly show higher pleomorphism with absence of neu-ral differentiation. LCH is most often found in the anteriorportion of the nasal septum.19,20 Histologically LCH is charac-

terized by submucous vascular proliferation arranged in lob-ules or clusters composed of central capillaries and smallerramifying tributaries.

The only treatment for schwannoma is wide local excisionthrough an approach allowing adequate exposure as schwan-noma are generally radioresistant. 4,8 But in treating benign

schwannoma, functional and cosmetic considerations shouldbe taken into account. Recently, the technique of endoscopicnasal surgery has rapidly developed and transnasal endoscopic

excision of benign tumors of the nose, paranasal sinuses andnasal septum has been successful.14,17 The tumor mass in ourcase was limited to the septal mucosa and successfully excised

by transnasal endoscopic approach. A single schwannomadoes not recur when completely excised, but intracranial exten-sion of a nasal schwannoma has been reported.21,22

4. Conclusion

We report a 24-year-old male patient with nasal septal schwan-

noma which was successfully treated by transnasal endoscopicexcision. Although schwannoma of the nasal septum is extre-mely rare, the possibility of their existence should be realized

and included in the differential diagnosis of any nasal mass.

Funding

The source of funding was Institutional funds of MidnaporeMedical College & Hospital, Paschim Medinipur.

Acknowledgement

All the staffs of department of pathology, Midnapore MedicalCollege & Hospital, Paschim Medinipur.

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