PLEXIFORM NEUROFIBROMA
Neurofibroma is a benign nerve sheath tumor arising in the peripheral nervous system. It may be sporadic or associated with Neurofibromatosis 1. Based on the growth pattern, it is subdivided into localized, plexiform, and diffuse types. Plexiform Neurofibroma develops in early childhood and are virtually diagnostic of NF1. They can be found in superficial as well deep locations converting the involved large nerve trunks and nerve roots into a thickened tortuous mass resembling 'a bag of worms.' They can cause massive enlargement of the entire extremity, hypertrophy of the long bones, and redundancy and hyperpigmentation of skin. PF may display increased cellularity as well as nuclear atypia. Such lesions are at a greater risk of malignant transformation.
