Eur Arch Otorhinolaryngol (2000) 257 : 402–405
HEAD AND NECK ONCOLOGY
Marco Berlucchi · Cesare Piazza · Laura Blanzuoli ·
Giuseppe Battaglia · Piero Nicolai
Schwannoma of the nasal septum:
a case report with review of the literature
Received: 11 June 1999 / Accepted: 10 February 2000
Abstract Schwannomas are neurogenic neoplasms rarely
found in the sinonasal tract, where localization to the
nasal septum is exceedingly rare (only 11 cases have been
described in the western literature). We report the case of
a 29-year-old white male with a schwannoma completely
filling the left nasal fossa and arising from the bony part
of the septum. A computer tomography (CT) scan and a
biopsy suggestive of benign schwannoma were obtained
before the lesion was removed by a degloving approach.
The preoperative diagnosis of nasal septum schwannoma
was confirmed. The patient is asymptomatic and without
endoscopic evidence of recurrence 7 years after surgery.
A review of the literature with particular emphasis on the
clinical presentation, histological features, differential di-
agnosis and therapeutic options for such a rare lesion is
included.
Key words Nasal neoplasms · Septal schwannoma ·
Midface degloving
Introduction
Schwannoma is a neurogenic tumour that arises from the
sheath of myelinated nerves. Only 4% of the lesions in-
volve nasal and paranasal cavities [10], and the nasal sep-
tum is one of the rarest localizations of schwannoma. We
report the 12th case of nasal septum schwannoma, to-
gether with a review of the literature focusing on the clin-
ical presentation, histology, differential diagnosis and
therapy of this rare disease.
P. Nicolai () · M. Berlucchi · C. Piazza
Department of Otolaryngology, University of Brescia,
Piazza Spedali Civili 1, 25123 Brescia, Italy
e-mail: nicolai@master.cci.unibs.it
Tel.: +39-030-3995319; Fax: +39-030-395212
L. Blanzuoli
Institute of Pathology, University of Brescia, Brescia, Italy
G. Battaglia
Institute of Radiology, University of Brescia, Brescia, Italy
© Springer-Verlag 2000
Case report
A 29-year-old white male was admitted to the Department of Oto-
laryngology of the University of Brescia in May 1992 with a
4-week history of progressive nasal obstruction and epistaxis. He
had no history of anosmia, facial pain or allergies. Family history
was otherwise unremarkable. Anterior and posterior rhinoscopy
showed a smooth, dark-red, bleeding, polypoid lesion completely
filling the left nasal fossa and extending through the choana into
the nasopharynx. A rigid endoscopy, performed after application
of cotonoid pledgets soaked in 1% xylocaine with 1:200,000 epi-
nephrine all around the mass, suggested an origin from the perpen-
dicular plate of the ethmoid and revealed only an indirect contact
between the lesion and the cribriform plate.
A computer tomography (CT) scan of the paranasal sinuses
(2 mm-thick sections) in the axial and coronal planes showed a
mass with non-homogeneous enhancement after contrast medium
administration that involved the nasal septum and compressed the
left inferior and middle turbinate, which were partially eroded
(Fig. 1 a, b). The lesion encroached on the left nasal bone, the
frontal process of the maxilla and the lacrimal bone. The left max-
illary sinus was partially filled by retained secretions, whereas the
other paranasal sinuses were normally aerated. The lesion did not
involve the skull base, nor was there any sign of ethmoid septa re-
absorption. A biopsy was suggestive of benign schwannoma. Since
neither café-au-lait spots nor multiple cutaneous neurofibromas
suggesting Von Recklinghausen’s disease were evident, other lab-
oratory tests and radiological examinations were not considered
necessary.
A medial maxillectomy with preservation of the lacrimal path-
ways through a degloving approach was performed to expose the
lesion. The latter measured 4 × 3 × 1.5 cm and originated in the
area of the perpendicular plate of the ethmoid. En bloc excision in-
cluded a large portion of the adjacent nasal septum; frozen sections
confirmed the radical nature of the resection.
Histologically, the lesion was composed of spindle-shaped
cells arranged in bundles, sometimes resembling a palisade. Re-
gressive areas composed of cells with large, multilobar nuclei and
blood vessels with hyalin thickening of the adventitia were evi-
dent. No mitotic figures were present (Fig. 2). Immunohistochemi-
cal studies were positive for S-100 protein and vimentin and nega-
tive for desmin and smooth-muscle actin. The diagnosis of benign
schwannoma was confirmed.
The patient was free of disease at the endoscopic evaluation
7 years after surgery; CT of the paranasal sinuses confirmed the
finding.

a Between 25% and 45% of such tumours have been ob-
b ing 160 cases of schwannoma of the paranasal and nasal
Fig. 1 a, b Computer tomography scan after contrast administra-
tion: a axial and b coronal scans. In the left nasal fossa, a polypoid
lesion adherent to the nasal septum with a non-homogeneous en-
hancement is evident; the inferior and middle turbinate are later-
ally displaced and partially eroded. Stenosis of the ostio-meatal
complex has caused retention of inflammatory secretions in the left
maxillary sinus
Fig. 2 Regressive areas of the lesion composed of spindle-shaped
cells with large and multilobated nuclei and evident nucleoli. No
mitoses are evident (hematoxylin-eosin, OM × 20)
403
Discussion
A schwannoma is a tumour arising from Schwann cells
and was first described as a distinct pathological entity by
Verocay in 1908 [27]. However, only in 1942 was the
term schwannoma proposed by Del Rio-Hortega [8] to in-
dicate lesions arising from Schwann cells. Other terms
used to designate the tumour, such as neurinoma, neu-
roma, neurolemmoma, neurilemmoma, perineural fibrob-
lastoma, peripheral glioma and schwannoglioma, are
nowadays considered inaccurate and obsolete, since they
indicate lesions containing all elements of a nerve [16,
22].
served in the head and neck region, where the vestibular
nerve is the most frequent site of origin. The scalp, face,
oral cavity, tongue, soft palate, pharynx and parapharyn-
geal space, larynx, trachea, parotid gland, middle ear, in-
ternal and external auditory meatus, and neck are less fre-
quently involved [19]. The first description of a schwan-
noma involving the nasal cavity dates back to 1926 [25].
Only 4% of head and neck schwannomas originate from
the sinonasal tract [9, 10, 13, 28], where the ethmoid,
maxillary sinus, nasal fossa and sphenoid sinus are in-
volved in decreasing order [3, 22]. No case arising from
the frontal sinus has been documented [7]. Involvement of
more than one sinus is a not uncommon finding [28].
Higo et al. [14] reviewed the world literature, collect-
cavities. They reported an age distribution ranging from
6 to 78 years, with most of the patients between 25 and
55 years of age, without any peculiar prevalence of race
or sex [12, 21]. The lesion is almost always solitary, but
instances of multiple schwannomas have been documented,
particularly in Von Recklinghausen’s disease [18].
Nasal septum schwannoma was first described by Bog-
dasarian and Stout in 1943 [4], and 10 additional cases
with adequate clinical–pathological information were
subsequently reported in the western literature (Table 1)
[5, 6, 11, 15, 18, 19, 20, 24, 26]. Sympathetic nerves to the
septal blood vessels, parasympathetic nerves to septal mu-
cous glands, and sensory nerves to the nasal septum (na-
sopalatine nerve and the anterior and posterior ethmoidal
nerves) have been suggested as structures of origin for a
schwannoma of this region [19]. However, at the time of
surgery the nerve of origin is usually not identifiable [22].
Symptoms and signs depend on the site of origin and
the extent of the lesion. They include unilateral nasal ob-
struction, epistaxis, mucopurulent rhinorrhea, anosmia,
facial swelling, proptosis and pain when the maxillary si-
nus is involved [16, 17, 21]. Extension into the sphenoid
sinus can lead to diplopia (secondary to deficits of III, IV
and VI cranial nerves), deep retro-orbital pain and occipi-
tal, frontal and bitemporal headache [28]. As documented
by previous reports [16, 19] and also by our observations,
septal schwannoma commonly displays a polypoid ap-
pearance without any distinctive features, so differential
diagnosis includes a broad spectrum of lesions ranging
404
Table 1 Schwannoma of the nasal septum: review of the western literature. NED no evidence of disease
Author(s) Year Age/sex Symptoms Site Treatment Follow-up
Bogdasarian 1943 42/M Nasal obstruction Nasal septum at Endonasal excision –
and Stout [4] Kiesselbach’s area
Sooy [24] 1950 3 mo/F Nasal obstruction Superior part of Endonasal excision NED 31 mo
the nasal septum
Johnson and 1959 31/F Epistaxis Adherent to the lateral Endonasal excision –
Lineback [15] wall and to the septum
Dutt [11] 1969 20/M Nasal obstruction, Attachment to the Piecemeal excision –
watery rhinorrhea, anosmia, lateral wall and septum
occasional sneezing
Thomas [26] 1977 21/F Nasal obstruction, right Anterior part of the Lateral rhinotomy –
mucopurulent epiphora and perpendicular plate
proptosis, facial distortion of the ethmoid
Perzin et al. [20] 1982 59/M Nasal obstruction Nasal septum Endonasal excision NED 6 yr
Bonfils et al. [5] 1989 55/F – Posterior part of the Lateral rhinotomy NED 6 yr
nasal septum
Pasic and 1990 36/M Epistaxis, nasal obstruction Nasal septum Endonasal excision NED 18 mo
Makielski [19]
Butugan et al. [6] 1993 25/F Epistaxis, nasal ostruction, Nasal septum Midface degloving NED 16 mo
hyposmia
52/M Epistaxis, nasal discharge Nasal septum Surgery (the technique NED 4 yr
is not specified)
Oi et al. [18] 1993 71/M Epistaxis, rhinorrhea, Nasal septum Combined trans-palatal NED 2 yr
nasal obstruction and trans-maxillary
approach
Present case 29/M Nasal obstruction, epistaxis Nasal septum Midface degloving NED 7 yr

from angiomatous polyps to malignant tumours such as
melanoma and olfactory neuroblastoma.
CT clearly depicts the relationship of the lesion to the
surrounding bony structures; erosion is more common
with large schwannomas. The lesions usually have a mot-
tled central lucency with peripheral intensification on
contrast-enhanced CT scans. The heterogeneous appear-
ance is related to areas of increased vascularity with adja-
cent non-enhancing cystic or necrotic regions [28].
Magnetic resonance imaging (MR) is superior to CT in
differentiating a tumour from inflammatory changes and
normal tissues; furthermore, intracranial extension can be
better delineated. Schwannomas have an intermediate
signal intensity on T1-weighted images, whereas a T2-
weighted signal varies from intermediate intensity (highly
cellular lesion) to non-homogeneously high intensity
(cystic and stromal lesions). A more uniform enhance-
ment pattern after gadolinium administration has been ob-
served [10, 23, 28]. In the present case, a CT scan with
contrast medium clearly ruled out the involvement of vital
structures (i.e. skull base, orbit, carotid artery); therefore,
MR would not have added any information relevant to the
therapeutic planning.
Macroscopically, schwannomas appear as gelatinous
or cystic, well encapsulated masses. Enlargement of
schwannomas can lead to areas of cystic degeneration as
the tumours outgrow their blood supply. Other common
regressive changes are necrosis, lipidization and forma-
tion of angiomatous clusters of blood vessels with focal
thrombosis. The changes justify the adjectives sometimes
used for such tumours (i.e. cystic, ancient, pleomorphic).
The nasal septum schwannoma reported here was consid-
ered to be an ancient schwannoma because of the nuclear
characteristics and the presence of extensive hyalinization
[1].
As described by Antoni in 1920 [2], the lesion can dis-
play two distinct histological patterns: Antoni type A (fas-
ciculated, with high cellular density) and type B (reticular,
with low cellular density). The former presents areas
composed of compact spindle cells with twisted nuclei,
indistinct cytoplasmic borders and occasional intranuclear
vacuoles. The cells are arranged in short bundles or inter-
lacing fascicles. They may show nuclear palisading,
whorling of cells and Verocay bodies (compact groups of
parallel spindle-shaped nuclei). Antoni type B schwanno-
mas show a degenerative pattern and are composed of
spindle-shaped cells running in a haphazard manner and
more loosely arranged in a fibrillar myxoid-like stroma.
However, the distinction is considered to have only an
academic interest [9, 19].
Differentiation between schwannoma and neuro-
fibroma can be particularly intriguing because of the
presence of overlapping histological features. Basically,
schwannomas are often solitary and tender, with degener-
ative changes. Neurofibromas are more frequently multi-
ple and associated with Von Recklinghausen’s disease;
they are usually non-tender and less commonly present re-
gressive changes. Neurofibromas are not encapsulated
and are formed by a combined proliferation of all the ele-
ments of a peripheral nerve: axons, Schwann cells, fibro-
blasts and probably perineural cells. Malignant transfor-
mation of a schwannoma is an exceedingly rare event, but

the risk increases in patients with Von Recklinghausen’s
disease, in whom the incidence of malignant transforma-
tion is about 10–15% [6].
The treatment of choice for nasal septum schwannoma
is surgery. Different approaches such as lateral rhinotomy
with external ethmoidectomy, the Caldwell-Luc approach,
midface degloving or endonasal endoscopic resection have
been employed in relation to tumour extension [6]. In our
case, an external approach was chosen because of the con-
siderable size of the tumour and because at the time the
patient was treated, endoscopic surgery was reserved for
the management of inflammatory diseases. However,
schwannoma of the nasal septum nowadays can be ade-
quately resected endoscopically, as reported by Klossek et
al. [17] of a case involving the maxillary sinus.
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