Midline Nasal Mass in Infancy: A Nasal Glioma Case Report

Abstract

Congenital midline nasal masses are rare anomalies that occur in about one in 20,000-40,000 live births. The most common are dermoid/epidermoid tumors, nasal cerebral heterotopias (nasal gliomas), and nasal encephaloceles; some have an actual or potential central nervous system connection. Nasal gliomas are CNS masses of neurogenic origin which have lost their intracranial connections and present as an obvious external or intranasal mass at birth without associated surgical symptoms. Careful evaluation is required to confirm the diagnosis and appropriate management. The interpretation of CT and MR images can be difficult but is useful in differentiating nasal gliomas from other congenital nasal masses. The presence of a fibrous stalk may be associated with cranial defects and CSF leak. Excisional biopsy allows histopathologic diagnosis and is the definitive treatment. They are benign lesions, and recurrences are rare, so conservative cosmetic surgical techniques should be chosen for gliomas where there is no proven intracranial extension. The authors report an illustrative nasal glioma case in a one-year-old male infant with extranasal and intranasal components, and discuss the therapeutic options.

Nasal glioma with psammomatous calcification- An unusual presentation

“…They have a 3:1 male predominance, with no familial or hereditary predisposition and no malignant potential. 3,7 The tumour growth rate is consistent with the patient's body growth. There are intranasal (30%), extranasal (60%), or combined (10%) types of nasal gliomas.…”

“…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 and no malignant potential.…”

“…4,5 As gliomas are nonencapsulated accumulations of glial cells situated outside the CNS, the possible theories of development 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 connective tissue. 5 They have no true capsule and mitosis is rarely noted.…”

“…They are nonencapsulated CNS masses of neurogenic origin, which have lost their intracranial connections, 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.…”

Extranasal nasal glioma

“…The presence of a fibrous stalk is associated with CSF leaks and defects in the bony cranium. 4 Although rare, the ophthalmologist needs to be aware that CMNMs require high-resolution neuroimaging, prior to surgical intervention, to exclude bony defects and to delineate the lesion looking for both intracranial extension and connection, [5][6][7] …”