Schwannoma of nasal septum: A rare case report with literature review
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Transcript of 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.
O. Belgharia, 700056 Kolk
74174040.-mail address: bhaskarmitra
er review under responsibili
roat and Allied Sciences.
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90-0740 ª 2012 Egyptian So
tp://dx.doi.org/10.1016/j.ejen
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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,
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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.
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.
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-
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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