Plexiform Neurofibroma

Definition

Neurofibromas, one of the manifestations of neurofibromatosis type 1 and type 2 (with difference depending on the location of the tumors), are benign neoplastic peripheral nerve sheath tumors that result in variable tumor shapes and unpredictable growth patterns (Packer et al. 2002). Plexiform neurofibromas involve multiple nerves. Initially, the neurofibromas grow along the neuron from which they arose, but cause distortions of the neuron’s cellular functions and structure, and result in inflating the neuron and often spreading diffusely into dermis, soft tissue, and surrounding vascular tissues (Gottfried et al. 2006). They can present at any time in life, are usually slow growing, and may or may not be surgically excised. The decision to surgically resect the tumor is dependent on clinical symptoms and on how intertwined the neurofibroma tumor is with the nerve and blood vessel structures. These tumors, though benign, can cause pain and significant functional...