Facial Plexiform Neurofibromatosis Type I

Abstract

Introduction 

Plexiform neurofibroma is a benign tumor of peripheral nerves arising from a proliferation of all neural elements. Clinically, it presents as a subcutaneous mass which feels like a "bag of worms".

Case Report 

A 23-years-old male with a swelling over left side of the face large enough to involve whole one side of the face. There was tenderness and the swelling had a very peculiar consistency, soft in most of the areas with few firm nodular areas.

Operative procedure: surgery was done in two stages. Initially subtotal excision was done and 3 months later re-explored to excise the recurrent disease. Complete excision was not possible.

Discussion

Plexiform neurofibroma (PNF) occurs due to overgrowth of neural tissue in the subcutaneous region. Surgical management remains the mainstay of treatment but functional disturbances are almost inevitable while resecting tumors involving the head and neck region. Resection and de bulking of invasive PNF is however associated with a high rate of recurrence. One of the limiting factors is vascularity of these lesions and their abnormal propensity to bleed.

Conclusion 

Although benign, plexiform neurofibromas can cause pain, disfigurement and functional changes and more importantly, may turn malignant. Surgery should be attempted considering psychological benefits.