Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Ahead Of Print Login 
Users Online: 6005
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
  Table of Contents    
CASE REPORT  
Year : 2022  |  Volume : 65  |  Issue : 4  |  Page : 911-913
Intranasal glioma (Nasal heterotopia): A rare cause of nasal polyp in neonates


Department of Pathology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

Click here for correspondence address and email

Date of Submission18-Jan-2021
Date of Decision30-Apr-2021
Date of Acceptance02-May-2021
Date of Web Publication25-May-2022
 

   Abstract 


A 3-month-old boy presented with an intranasal polypoidal mass protruding out of the nostril which was present since birth and growing slowly. The mass was non-pulsatile and soft to firm in consistency. It did not increase in size on coughing, crying, or compression of the jugular vein. Magnetic resonance imaging and contrast-enhanced computed tomography (CT) revealed a lobulated well-circumscribed soft tissue mass in the left nasal cavity with no intracranial communication. Complete surgical excision of the mass was carried out via an intranasal endoscopic approach. Histopathological examination confirmed the diagnosis of intranasal glioma.

Keywords: Nasal glioma, nasal heterotopia, nasal polyp

How to cite this article:
Narang V, Jindal S, Garg B, Kaur H, Soni A. Intranasal glioma (Nasal heterotopia): A rare cause of nasal polyp in neonates. Indian J Pathol Microbiol 2022;65:911-3

How to cite this URL:
Narang V, Jindal S, Garg B, Kaur H, Soni A. Intranasal glioma (Nasal heterotopia): A rare cause of nasal polyp in neonates. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Nov 30];65:911-3. Available from: https://www.ijpmonline.org/text.asp?2022/65/4/911/345884





   Introduction Top


Nasal gliomas are rare congenital midline lesions composed of extracranial masses of neuroglial tissue unconnected with the brain.[1] V. Nada et al.[2] reported the incidence of congenital midline nasal masses in about 1 in 20,000–40,000 live births. However, only 5% of all congenital nasal masses are attributed to nasal gliomas. Although nasal gliomas are benign lesions, they require complete excision for a good prognosis. A near-definitive diagnosis can be made by clinical and radiological examinations. However, histopathology remains the gold standard for final diagnosis.

We, hereby, present a case of intranasal glioma in a neonate who presented with an intranasal polyp for uncommonness and diagnostic importance.


   Case Report Top


A 3-month-old boy presented with an intranasal mass in his left nasal cavity which was also protruding out of the left nostril. It was present since birth and was slowly increasing in size. History of intermittent nasal obstruction was stated. There was no history of nasal discharge or epistaxis. General physical examination was normal with the patient being active and playful. An endoscopic nasal examination was performed as the initial local examination which revealed a polypoidal lesion present in the left nasal cavity protruding through the left nostril [[Figure 1]; panel A] which was attached to the nasal septum. It was a soft to firm, non-pulsatile lesion which did not increase in size on coughing, crying, or compression of the jugular vein (Furstenberg's test). The right nasal cavity was patent. No facial deformity was apparent clinically. Contrast-enhanced computed tomography of paranasal sinuses (CECT PNS) and magnetic resonance imaging (MRI) of the brain were conducted to determine the nature and extent of the lesion. CECT PNS revealed a well-circumscribed lobulated soft tissue density mass lesion measuring approximately 1.8 cm × 1.6 cm × 0.6 cm present in the left nasal cavity with no intracranial communication and having a few calcific foci within. No evidence of bone destruction was noted. MRI brain also revealed a hyperintense mass on T2-weighted imaging in the left nasal cavity with no abnormality in the brain or ventricular system [[Figure 1]; panel B]. With this workup, trans-nasal endoscopic excision was conducted. The patient was monitored for postoperative complications including postoperative bleeding, cerebrospinal fluid leak, and infections. Histopathological examination revealed a lining of stratified squamous epithelium with subepithelial tissue showing the presence of benign glial tissue with very low proliferation activity [[Figure 1]; panel C and D]. No dural tissue or leptomeninges was identified. Thus, a final diagnosis of intranasal glioma was rendered. The patient is under regular clinical follow-up with an uneventful clinical course to date.
Figure 1: Nasal endoscopy showing a polypoidal mass in left nasal cavity (panel A); MRI T2 weighted imaging showing a hyperintense mass in left nasal cavity (panel B); Photomicrograph (hematoxylin and eosin stain; ×100) showing mature glial tissue with low proliferative index (panel C & D)

Click here to view



   Discussion Top


Nasal glioma is an uncommon cause of congenital midline masses in neonates. Schmidt coined the term glioma for such lesions in 1900.[3] However, the term glioma is a misnomer as it indicates a malignant neoplasm of the brain. Neuroglial heterotopia is a more suitable term which implies the correct nature of these lesions. It commonly affects the head and neck region and can be located in the nose, nasopharynx, orbit, palate, lip, oropharynx, and scalp. Nasal gliomas are heterotopic rests of extracranial benign neuroglial tissue unconnected with the brain. These can be of three types depending on the location: (1) intranasal gliomas (20%) involve nasal or nasopharyngeal cavity and are present as polypoidal mass; (2) extranasal gliomas (60%) most commonly occur at the bridge of the nose; (3) mixed/combined gliomas (10%) have intercommunicating intranasal and extranasal components.[4]

During the 12th week of gestation fusion of nasal capsule and fonticulus nasofrontalis leads to the formation of nasal cavity. Abnormal closure of fonticulus nasofrontalis causes the formation of ectopic extracranial rests of mature glial tissue unconnected with the brain.[5] However, a fibrous stalk can be found in approximately 15–20% of the cases linking them to intracranial spaces.[6]

Clinical presentation depends on the clinical type of nasal glioma. Intranasal gliomas present as nasal polyps attached either to the nasal septum or lateral wall of the nose and may cause symptoms of nasal obstruction as noted in our case. Extranasal gliomas present as incompressible, firm, non-pulsatile mass at the bridge of the nose and may cause facial deformities when large. Mixed/combined gliomas have intercommunicating intranasal as well as extranasal components.

Neuroimaging including computed tomography (CT) and magnetic resonance imaging (MRI) plays an important role in the diagnosis of nasal gliomas by determining the nature of the mass along with a possible intracranial connection. On CT, nasal gliomas are usually isodense to brain tissue. It also helps in the documentation of any associated bony defects. However, MRI remains the modality of choice as it permits optimal visualization of any extracranial extension which is seen in the encephalocele.[7] On T1-weighted imaging, nasal glioma appears isointense to hypointense relative to the gray matter and hyperintense on T2-weighted imaging.[8] Nasal endoscopic examination is equally important as neuroimaging to decide the type of surgery.

The differential diagnoses of congenital midline masses are encephalocele, nasal dermoid, hemangioma, nasolacrimal cyst, and lipoma.[9] The most important of all remains encephalocele wherein neural tissue is herniated through a skull defect which can be established clinically as well as on imaging. A change in size occurs in encephalocele on compression of the internal jugular vein (Furstenberg's sign). However, nasal gliomas show no change in size because of absent patent intracranial connection. On CECT, nasal gliomas are mostly nonenhancing while encephalocele shows contrast enhancement on compression of the internal jugular vein.[10]

Histopathological examination reveals the presence of mature neuroglial tissue with fibrovascular tissue and foci of calcification.[6] Presence of choroid plexus, ependyma, or leptomeninges warrants the diagnosis of encephalocele. On immunohistochemistry, nasal gliomas express strong positivity for S-100 and glial fibrillary acidic protein (GFAP).

Complete surgical excision is the treatment of choice for nasal gliomas.[2] However, the approach depends on clinical type and intracranial extension. Intranasal glioma with no intracranial extension can be entirely removed by intranasal endoscopic surgery and holds minimal postoperative complications. Intranasal gliomas with intracranial connection though require frontal craniotomy involving a multispecialty team with a high possibility of postoperative complications like cerebrospinal fluid leak and meningitis. Surgical excision through an external approach with the closure of primary defects remains the treatment of choice for extranasal gliomas. Although benign incomplete excision may lead to recurrence with a recurrence rate of 4–10%.

Our case was an intranasal glioma with no intracranial connection which was excised through an intranasal endoscopic approach. The patient had no immediate postsurgical complication and is on a regular clinical follow-up.


   Conclusion Top


Nasal glioma (nasal heterotopia) is a rare cause of congenital midline lesions. Although benign, an expert clinical outlook along with neuroimaging are required to plan the mode of surgery and to reduce the postsurgical complications. A multispecialty team approach may be required depending upon the clinical type and extent of the lesion. A near definite diagnosis can be made by clinical and radiological findings but the final diagnosis can only be made on histopathology.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Yeoh GPS, Bale PM, Silva M. Nasal cerebral heterotopia: The so-called nasal glioma or s sequestrated encephalocele and its variants. Pediatr. Pathol 1989;9:531-49.  Back to cited text no. 1
    
2.
Nada V, Dejan V, Dragan D, Ljiljana J. Nasal glioma. Arch Oncol 2006;14:57-9.  Back to cited text no. 2
    
3.
Schmidt MB. Uberseltene spaltbildungen im Bereiche des mittleren Stirnforsatzes. Virchows Arch Pathol Anat 1900;162:340-70.  Back to cited text no. 3
    
4.
Lowe LH, Booth TN, Joglar JM, Rollins NK. Midface anomalies in children. Radiographics 2000;20:907-22.  Back to cited text no. 4
    
5.
Krishna LG, Uppoor R, Rao KN, Harish K. Heterotopic central nervous tissue-Nasal glioma -A case report. Ind J Radiol Imag 2005;15:4:511-6.  Back to cited text no. 5
    
6.
Patterson K, Kapur S, Chandra RS. ''Nasal gliomas'' and related brain heterotopias: A pathologist's perspective. Pediatr Pathol 1986;5:353-62.  Back to cited text no. 6
    
7.
Hedlund G. Congenital frontonasal masses: Developmental anatomy, malformations, and MR imaging. Pediatr Radiol 2006;36:647-62; quiz 726-7.  Back to cited text no. 7
    
8.
Singhal SK, Virk RS, Dass A, Amanjit BS. Neonatal nasal glioma: A case report. Int J Otorhinol Gol 2006;4:2.  Back to cited text no. 8
    
9.
Clarós P, Bandos R, Clarós A Jr, Gilea I, Clarós A, Real M. Nasal gliomas: Main features, management and report of five cases. Int J Pediatr Otorhinolaryngol 1998;46:15-20.  Back to cited text no. 9
    
10.
Ajose-Popoola O, Lin HW, Silvera VM, Teot LA, Madsen JR, Meara JG, et al. Nasal glioma: Prenatal diagnosis and multidisciplinary surgical approach. Skull Base Rep 2011;1:83-8.  Back to cited text no. 10
    

Top
Correspondence Address:
Ankita Soni
Dayanand Medical College and Hospital, Tagore Nagar, Ludhiana - 141 001, Punjab
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpm.ijpm_57_21

Rights and Permissions


    Figures

  [Figure 1]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed616    
    Printed47    
    Emailed0    
    PDF Downloaded33    
    Comments [Add]    

Recommend this journal