Two cases of plexiform neurofibromatosis at unusual sites

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Case Report

Author Details : Khushboo Modasia, Aishni Shah, Shah Patel, Rita Vora*

Volume : 5, Issue : 3, Year : 2019

Article Page : 258-260

https://doi.org/10.18231/j.ijced.2019.055

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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.

Keywords: Plexiform neurofibroma, Tumour, Nerves.

How to cite : Modasia K, Shah A, Patel S, Vora R, Two cases of plexiform neurofibromatosis at unusual sites. IP Indian J Clin Exp Dermatol 2019;5(3):258-260

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