












































International Journal of Cancer Therapy and Oncology
www.ijcto.org

Copyright © Sherwani et al. ISSN 2330-4049

Nasal glioma with psammomatous calcification-
An unusual presentation

Rana K Sherwani1, Kafil Akhtar1*, Prasenjit S Ray1, Sayed S Ahmad2

1Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, India.
2Department of Oral Maxillo-facial Surgery, Sir Ziauddin Dental College, Aligarh Muslim University, Aligarh, India.

Received January 14, 2014; Revised March 18, 2014; Accepted March 20, 2014; Published Online April 09, 2014

Case Report

Abstract
Congenital midline swellings of nose are encountered rarely, and nasal gliomas constitute about 5% of such lesions. Various
theories have been suggested to explain the pathogenesis. Imaging preferably by MRI is mandated to study the extent and to
rule out intracranial extension. Clinically, these masses are firm and incompressible. Histologically, they are made up of astro-
cytes and neuroglial cells, embedded in fibrous and vascular connective tissue. The mainstay of treatment is conservative sur gi-
cal excision because nasal gliomas are slow-growing, rarely recurrent, and have no malignant potential. We present a case of
congenital extranasal glioma with psammomatous calcification and without any intracranial extension in an eighteen month old
boy.

Keywords: Extranasal Glioma; Child; Psammomatous Calcification

Introduction
The developmental anomalies of the nose encompass a di-
verse group of conditions, which include nasal dermoids,
gliomas, encephaloceles, nasal clefts, proboscis lateralis, pol-
yrrhinia, nasopharyngeal teratoma, and epignathus.1 The
most common are nasal dermoids, nasal gliomas and nasal
(meningo) encephaloceles, which are clinically significant
because some of them might have an effective or potential
connection with the central nervous system (CNS).

Nasal gliomas are one form of the congenital midline nasal
masses that usually present at birth. They are rare, benign,
congenital tumours, which arise from abnormal embryonic
development. They are nonencapsulated CNS masses of
neurogenic origin, which have lost their intracranial con-
nections, and present as an obvious external or intranasal
mass at birth, without associated surgical symptoms.2, 3 The
term "nasal glioma" is a confusing misnomer as it implies a
neoplastic condition with malignant potential, which it is
not. Around 15 to 20 % of nasal gliomas have a fibrous stalk

connecting to the central nervous system. It should be dif-
ferentiated from glioma, which is a malignant tumor of the
brain. In addition, it should be differentiated from a primary
encephalocele, which is herniation of the cranial contents
through a bone defect in the skull, through which it retains
an intact connection with the central nervous system.4, 5 An
accurate diagnosis permits proper management and prevents
potentially life-threatening intracranial complications. Se-
vere complications such as meningitis or a brain abscess can
be avoided if the lesions are removed at an early stage. We
report a case of extranasal glioma with psammomatous calci-
fications in eighteen months old boy.

Case Report

An eighteen month old boy presented with a swelling over
his nasal bridge. The swelling had been present since birth
but did not cause any nasal obstruction. There was no histo-
ry of epistaxis or any other nasal discharge. He was born at
full-term and had a normal vaginal delivery. His elder sibling
had no congenital abnormalities and the family history was
unremarkable. On physical examination, the boy had a 3 × 3
cm superficial purple, soft to firm mass with a smooth sur-
face over his nasal bridge. It was neither tender nor pulsatile.
There was no intranasal mass. Clinically, the mass was diag-
nosed as nasal dermoid/hemangioma.

*Corresponding author: Dr. Kafil Akhtar; Department of
Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim
University, Aligarh, India.

Cite this article as:
Sherwani RK, Akhtar K, Ray PS, Ahmad SS. Nasal glioma with
psammomatous calcification- An unusual presentation. Int J
Cancer Ther Oncol 2014; 2(2):02027. DOI: 10.14319/ijcto.0202.7

http://ijcto.org/index.php/IJCTO
http://dx.doi.org/10.14319/ijcto.0202.7


2 Sherwani et al.: Nasal glioma with psammomatous calcification International Journal of Cancer Therapy and Oncology
www.ijcto.org

Copyright © Sherwani et al. ISSN 2330-4049

Magnetic resonance imaging (MRI) of the brain showed a
well-defined soft tissue attenuation lesion, at the root of the
nose without any underlying bony defect or intracranial
extension, measuring 2.9 × 2.9 × 2.5 cm. (Figure 1)The lesion
showed mild heterogenous enhancement with evidence of
concentric calcifications within the lesion.

FIG. 1: MRI of the brain shows a well-defined soft tissue attenuation
lesion, at the root of the nose without any underlying bony defect or
intracranial extension, measuring 2.9 × 2.9 × 2.5cm.

The tumour was excised externally under general anaesthe-
sia, using a left lateral elliptical skin incision. The margin
was taken down to the cartilaginous pyramid. Grossly, it
appeared as a lobulated, well-circumscribed, and unencapsu-
lated whitish pink firm mass. Microscopic sections demon-
strated the presence of glial tissue with a fibrillary back-
ground and interspersed fibrovascular stroma with psam-
momatous calcifications. (Figure 2 and 3). No meningeal or
dural tissue was identified. The glial nature of the tumour
was confirmed by a positive reaction for S100 protein (Figure
4), and a definitive diagnosis of nasal glioma was made.

FIG. 2: Photomicrograph shows glial tissue with a fibrillary back-
ground and interspersed fibrovascular stroma. Haematoxylin and
Eosin × 10

FIG. 3: Photomicrograph shows glial tissue with a fibrillary back-
ground and interspersed fibrovascular stroma with psammomatous
calcifications. Haematoxylin and Eosin × 40

The postoperative recovery was uneventful and the cosmetic
result was satisfactory. Follow-ups performed over the last
12 months using brain MRI showed no residual parts or re-
currence of the glioma.

FIG. 4: Photomicrograph shows glial tissue with a positive reaction
for S100 protein. S-100 × 10

Discussion

The term nasal glioma has been used to describe a congenital
benign tumor of the nasal region containing neural tissue.
Schmidt was the first scientist to describe the comprehensive
nature of the nasal glioma in 1900. However, the term he
used is a misnomer.3, 5 Nasal gliomas are not true neoplasms;
they originate from ectopic glial tissue left extracranially
following abnormal closure of the nasal and frontal bone
during embryonic development.1, 4, 6 Therefore, some authors
recommend using the term ‘glial heterotopia’ instead.3 Nasal
gliomas generally present at birth, rarely in adults, as a mass
without associated nasal symptoms. They have a 3:1 male
predominance, with no familial or hereditary predisposition



Volume 2 • Number 2 • 2014 International Journal of Cancer Therapy and Oncology 3
www.ijcto.org

Copyright © Sherwani et al. ISSN 2330-4049

and no malignant potential.3, 7 The tumour growth rate is
consistent with the patient’s body growth. There are in-
tranasal (30%), extranasal (60%), or combined (10%) types of
nasal gliomas.6 Our case was of an extranasal glioma.

Most nasal gliomas are diagnosed at birth or during early
childhood, with relative peaks of occurrence between 5 and
10 years of age. Although the majority of patients present
with signs and symptoms during the first year of life, a later
presentation may be due to a specialist’s failure to recognize
a subclinical lesion in childhood or because of a trauma.2, 5
Unlike dermoids, they do not necessarily occur in the mid-
line, or attach to sinuses or skin. Gliomas form an uncom-
pressible mass that does not increase in size on the Valsalva
testing and does not transilluminate. Extranasal gliomas are
usually located at the glabella level, but they may be present
laterally. Intranasal gliomas are associated most frequently
with the middle turbinate or higher structures, and may
mimic nasal polyps. Combined intra/extranasal gliomas have
a typical dumbbell shape with a connecting band. Fifteen
percents of gliomas are connected with the dura, either
through the foramen cecum or through the fonticulus. Pa-
tients may present with a unilateral nasal obstruction, uni-
lateral nasal mass, epistaxis, or cerebrospinal rhinorrhea.4, 5

As gliomas are nonencapsulated accumulations of glial cells
situated outside the CNS, the possible theories of develop-
ment include the following: 1) sequestration of glial tissue of
the olfactory bulb entrapped during cribriform plate fusion;
2) ectopic neural tissue cells; 3) pinched encephalocele; and
4) inappropriate closure of the anterior neuropore (fonticulus
frontalis), with the failure of mesoderm to enter the region,
resulting in an inadequate bone formation.7, 8

Histologically, nasal gliomas are composed of astrocytes and
neuroglial cells, embedded in fibrous and vascular connec-
tive tissue.5 They have no true capsule and mitosis is rarely
noted. Multinucleated or gemistocytic astrocytes may be
present but it is rare to find neurons. The presence of abun-
dant neurons raises the possibility of an encephalocoele. The
glial nature of the cells can be further confirmed by im-
munohistochemical demonstration of S100 protein and
GFAP. These two proteins can identify neurological cells
with high specificity, and help to distinguish nasal gliomas
from other tumours such as meningiomas and granular cell
tumours.5, 8 In our case, both S100 and GFAP staining were
positive. Hence, a definitive diagnosis of nasal glioma was
made.

The clinical differential diagnosis includes several various
congenital and acquired disorders, which could be manifest-
ed by nasal masses. Nasal glioma should be differentiated
from several common or important lesions, among which:
1) Nasal dermoids, which are epithelial-lined cavities or si-
nus tracts with variable numbers of skin appendages, in-
cluding hair follicles, sebaceous glands, and eccrine glands.

They constitute the most common congenital nasal anomaly.
2) Encephaloceles point to the herniation of neural tissue
through defects in the skull. They may contain meninges
(meningocele) or brain matter and meninges (encepha-
lomeningocele), or they may communicate with a ventricle
(encephalomeningocystocele). Encephaloceles have an eti-
ology similar to that of gliomas. Twenty percents of all en-
cephaloceles occur in the cranium. Of those, 15 % are nasal.
3) Hemangioma, which are the most frequent benign vascu-
lar tumors in infancy.9-11

The diagnosis is corroborated by CT scan, which shows a
bony defect. MR imaging is obligatory in patients with sus-
pected glioma to determine possible intracranial extension of
the nasal cavity lesion and the brain.12, 13

The treatment of choice of nasal gliomas is complete surgical
excision.1 Gliomas are benign but incomplete excision results
in a 4 % to 10 % recurrence rate. The approach depends up-
on the location and extent of the lesion and levels of exper-
tise available. When facilities are available, intranasal endo-
scopic surgery is considered most appropriate approach for
the removal of intranasal glioma having no intracranial ex-
tension as this approach allows precise excision with mini-
mal trauma to the surrounding tissues.9 If however intracra-
nial extension is evident, than frontal craniotomy, multidis-
ciplinary team approach may be required in specialized neu-
rosurgical or craniofacial centers to ensure complete and safe
excision of the lesions.10

Conclusion

Nasal gliomas are rare congenital anomalies. It is mandatory
to rule out intracranial extension by cross-sectional imaging,
preferably by MRI before performing any invasive proce-
dure. Conservative surgical excision is the mainstay of the
management of an extranasal glioma, as it is slow-growing,
rarely recurrent, and has no malignant potential.

Conflict of interest

The authors declare that they have no conflicts of interest.
The authors alone are responsible for the content and writ-
ing of the paper.

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