Angiofibroma

Angiofibroma (AGF) is a descriptive term for a wide range of benign skin or mucous membrane (i.e. the outer membrane lining body cavities such as the mouth and nose) lesions in which individuals have: AGF lesions share common macroscopic (i.e. gross) and microscopic appearances.

Grossly, AGF lesions consist of multiple papules, one or more skin-colored to erythematous, dome-shaped nodules, or usually just a single tumor.

Microscopically, they consist of spindle-shaped and stellate-shaped cells centered around dilated and thin-walled blood vessels in a background of coarse bundles of collagen (i.e. the main fibrous component of connective tissue).

Some of these cutaneous AGF lesions occur in individuals with one or more of 3 different genetic diseases: tuberous sclerosis, multiple endocrine neoplasia type 1, and Birt-Hogg-Dube syndrome.

They develop in up to 8% of the general adult population and occur as 1 to 3[5] pink to red,[4] dome-shaped papules in the central areas of the face, nose, and/or lips.

[17] These lesions are nodules that occur on the buccal mucosa (i.e. mucous membranes lining the cheeks and back of the lips) or lateral tongue.

Oral fibromas are not neoplasms; they are hyperplastic (i.e. overgrowth) reactions of fibrous tissue to local trauma or chronic irritation.

They commonly arise in the nasopharynx (i.e. upper part of the throat that lies behind the nose) and typically have attachments to the sphenopalatine foramen, clivus, and/or root of the pterygoid processes of the sphenoid bone.

[26] These tumors are edematous (i.e. abnormally swollen with fluid), highly vascular, spindle-shaped cell lesions with a variable amount of fibrous stroma.

Acral angifibromeae occur primarily in areas close to the nails of fingers and toes (~80% of cases)[28] or, less commonly, palms of the hands or soles of the feet.