Recurrent diffuse neurofibroma of nose associated with neurofibromatosis type 1: A rare case report with review of literature

Abstract

Diffuse neurofibroma is an unusual variant of neurofibroma with the head and neck being the common sites of involvement. It is benign in nature and spreads superficially and has many ectatic blood vessels. Histologically it is similar to conventional neurofibromas except for a few peculiar distinguishing features. We report a case of a 14-year-old boy who presented with a diffuse recurrent painless swelling over the dorsum of the nose with the clinical stigmata of neurofibromatosis. Microscopy revealed a diagnosis of diffuse neurofibroma with a few foci showing differentiation towards Meissner's type of tactile corpuscles. It is important to recognize this entity as it has a tendency to recur, yet hardly ever become malignant and is almost always associated with neurofibromatosis type 1.

Spatiotemporal Loss of NF1 in Schwann Cell Lineage Leads to Different Types of Cutaneous Neurofibroma Susceptible to Modification by the Hippo Pathway

“…pNF is congenital but cNF rarely appears before puberty. When a cNF is observed on a relatively large area of skin but still confined in the dermis and it is perfectly covered by skin pigmentation, it is called a diffuse cutaneous neurofibroma (diffuse cNF) (6, 7). In this case, the skin is much thicker, the predominant body location is the head and neck, upper arms and the pigment are localized in the dermis.…”

A diffuse traumatic neuroma in the palate: a case report

“…However, the absence of inflammatory cells in a normal or loose fibrous connective tissue background can lead to the diagnosis of a mucosal neuroma. Neurofibromas and traumatic neuromas both have fibrous connective tissue and non-encapsulated lesions, but neurofibromas have mast cells and nuclei with a wavy or serpiginous prolife, and do not contain the abundant, haphazardly arranged axons that are unique to traumatic neuromas [ 8 ]. Although palisading neuromas and traumatic neuromas both form nerve bundles [ 9 ], the absence of inflammatory cells and fibrous connective tissue and the existence of spindle cells showing palisading arrangement and generally circumscribed margin is more indicative of a palisading neuroma.…”