Giant Plexiform Neurofibroma of the Thigh: A Case Report

Abstract

Neurofibromas are known to manifest most frequently as localized lesions, less frequently as a diffuse form, and rarely as a plexiform variety We report the clinical and imaging features of a young male patient with biopsy proven plexiform neurofibroma (PNF). A 23 years old male, followed for neurofibromatosis type 1, plexiform lesions of the left lower limb progressively increasing in volume with scrotal extension. The MRI showed thickening of the cutaneous and subcutaneous soft tissues on the medial and posterior aspect of the left thigh, extending to the perineo-scrotal region. The role of imaging is important for a variety of reasons, including delineating the extent of involvement and effect on adjacent structures, exposing associated anomalies and last but not least, for predicting possible malignant transformation. MRI is the reference standard modality for evaluating neural tissues and also for delineating the parent nerve in cases of tumors of neural origin. Therapy of plexiform neurofibromas is usually surgical, aiming at resecting deforming masses and cancerous tissue when malignant transformation occurs.