 Site created by Dr Sampurna Roy MD
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| Normal Anatomy of the Nerve |
Surgical-Pathology.com
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Syn: Nasal Glioma ; Glial Heterotopias.
Nasal gliomas or heterotopia are nonhereditary congenital malformations composed of heterotopic neuroglial tissue.
Encephaloceles and
glial heterotopias are related tumour -like lesions. Encephaloceles are
usually associated with bony defects unlike glial heterotopias.
Site:
Occur in the subcutaneous tissue (60%) or may be intranasal in location
(30%).
Both external and intranasal components may be present.
Rarely may occur on the scalp, either as multiple subcutaneous nodules or as
a single midline parietal nodule.
Clinical
presentation:
The lesion
presents as reddish blue, firm nodule on the bridge of nose, usually at
birth or in early infancy.
Histopathology:
Image Link: click
Unencapsulated, multilobulated lesion composed of islands of neural and
fibrovascular tissue.
The neural tissue is composed of astrocytes set in a neurofibrillary stroma.
Neurons are usually absent.
Old cases show prominent stromal sclerosis.
Some cases show cystic change and rarely focal calcification.
Immunohistochemistry:
Glial
fibrillary acidic protein (GFAP), S-100 protein and
vimentin : Positive
The nodules do not contain meningeal covering (EMA is negative).
Complete surgical excision of nasal glial heterotopias is curative
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Reactive and hamartomatous lesions:
Fibrolipomatous hamartoma of nerve
Schwannoma (neurilemmoma)
Dermal nerve sheath myxoma
Malignant peripheral nerve sheath tumour Merkel cell (neuroendocrine) carcinoma Malignant primitive neuroectodermal tumour Miscellaneous neuroectodermal tumours presenting in soft tissue |
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MESOTHELIOMA OF ATRIOVENTRICULAR NODE |