Two cases of plexiform neurofibromatosis at unusual sites


Article PDF :

Veiw Full Text PDF

Article type :

Case Report

Author :

Khushboo Modasia, Aishni Shah, Shah Patel, Rita Vora

Volume :

5

Issue :

3

Abstract :

Plexiform neurofibromatosis (PNF) is a potentially devastating type of neurofibromatosis, but is rare to occur. PNFs are benign tumours that originate from nerve sheath cells, visceral or subcutaneous peripheral nerves that can involve multiple fascicles and present as large tumours attached to major nerves. They are associated with NF-1 lesions in majority of cases and present with bulging and deforming masses involving skin folds and connective tissue most commonly at sites of trigeminal nerve distribution, and may also present as isolated lesions. There are high chances of malignant transformation in these lesions and thus should not be left untreated. Due to paucity of options available for treatment, surgical debulking remains the most important management. Being an autosomal dominant disorder, genetic counseling is necessary. We are presenting two cases of PNFs, occurring at unusual sites like sole of foot and forearm.

Keyword :

 Plexiform neurofibroma, Tumour, Nerves.