Fig. 1.58 Neurofibroma. A A central "circumscribed" area has cellular and hypocellular areas. There is nerve tissue noted at the periphery. B "Wavy" nuclei with bundles of collagen separated by a myxoid, degenerated stroma.

all ages may be affected, although patients tend to be older with an equal gender distribution.


The tumour arises from the ophthalmic or maxillary branches of the trigeminal (5th) nerve and is most commonly located in the maxillary and ethmoid sinuses and/or nasal cavity {288}. Plexiform neurofibroma may occur in the sinonasal area where it is found in the maxillary sinus {817}, usually associated with NF1.

Clinical features

Symptoms include epistaxis, rhinor-rhoea, swelling, mass, obstruction, and pain {61,2018}.


The tumour is firm, glistening, grey-tan, fusiform, and sometimes polypoid, in a submucosal location with an intact surface epithelium {61,1095}.


Neurofibromas are generally submucosal paucicellular lesions. They are composed of spindled cells with wavy, dark-staining nuclei and scanty cytoplasm, in a background of wavy collagen fibres, myxoid stroma and mast cells. The centre of the lesion usually shows residual neurites.


The tumour is diffusely immunoreactive for S100 protein, but the proportion of positive cells is lower than that in schwannoma. CD34 stains the admixed fibroblasts.

Genetic susceptibility

Sinonasal neurofibromas are generally not associated with NF1 {1091,1095, 2018}.

Prognosis and predictive factors

Neurofibromas are benign and have a very low recurrence rate. A small percentage of cases may undergo malignant transformation.

Meningioma Definition

A benign neoplasm of meningothelial cells.

ICD-O code



Primary extracranial (ectopic, extracal-varial) meningiomas of the sinonasal tract are rare, comprising <0.5% of non-

Fig. 1.59 Meningioma. A Meningothelial growth and "whorling". Note the squamous mucosa intimately associated with the meningioma. B "Whorling" of cytologi-cally bland meningeal cells is prominent in this meningioma.

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