Case of the Season: Diffuse Neurofibroma

Imaging appearance of Isolated diffuse neurofibroma of nipple areolar area: a case report

“…Clinically they are skin-colored dome-shaped or pedunculated papules that display the pathognomonic “button hole” sign. It is the subtype most familiar to radiologists because its imaging appearance has been well documented [ 2 - 4 ]. Plexiform neurofibromas diffusely involve single or multiple nerve branches [ 2 ].…”

“…They are usually single lesions, although It is suggested that 10% of patients with diffuse neurofibromas have NF1 [ 2 ]. Unlike other types of neurofibroma, which have a mass like pattern of growth, diffuse neurofibroma is a variably size and ill-defined lesion which diffusely infiltrate the dermis and subcutaneous tissues, which appear like a thickening of those [ 4 ]. It has been reported to occur in the head and neck regions of children and young adults.…”

“…Neurofibroma tissue is isointense or mildly hyperintense in relation to muscle on T1-weighted images and mildly or markedly hyperintense to muscle on T2 weighted images. Prominent internal vascularity and homogeneous enhancement following intravenous gadolinium administration are mostly found [ 4 - 7 ]. On histopathological examination, diffuse neurofibromas are ill-defined tumors that diffusely infiltrate the dermis and subcutaneous tissue, that spreads along connective tissue septa and surrounds rather than destroys adjacent normal structures.…”