• plexiform neurofibromas
  • One half to two thirds arises from neurofibromas, often of plexiform neurofibromas or in the setting of neurofibromatosis type I, which occur frequently on the head or neck. (jcancer.org)
  • Gene profile analysis demonstrates overexpression of fibroblast growth factor, epidermal growth factor, and platelet-derived growth factor in plexiform neurofibromas in patients with NF1. (clinicaltrials.gov)
  • A phase I trial of pirfenidone in children and young adults with NF1 and plexiform neurofibromas was completed, and has established the phase II dose (the dose resulting in a mean drug exposure [AUC] not more than 1 standard deviation below the mean drug exposure [AUC] in adults who received pirfenidone at the dose level demonstrating activity in fibrosing conditions). (clinicaltrials.gov)
  • MPNST
  • Malignant peripheral nerve sheath tumor (MPNST), also known as "Malignant schwannoma", "Neurofibrosarcoma", or "Neurosarcoma", is derived from Schwann cells or pluripotent cells of the neural crest [ 1 - 5 ]. (jcancer.org)
  • MPNST is generally characterized by alternating hypo- and hyper-cell areas or a diffuse growth pattern of spindle-shaped cells which are asymmetrical and fusiform with wavy or comma-shaped hyperchromatic nuclei, arranged in palisades or spiral shapes [ 1 - 3 , 7 , 8 , 18 , 19 ]. (jcancer.org)
  • MPNST also has glandular structures [ 1 , 2 , 6 , 10 - 12 , 16 ], and foci of neuroendocrine differentiation are often seen in glandular MPNST [ 1 ]. (jcancer.org)
  • The lifetime risk of patients with NF-1 developing MPNST has been estimated at 8-13%, while those with only MPNST have a 0.001% in the general population. (wikipedia.org)
  • In a 10-year institutional review for the treatment of chemotherapy for MPNST in NF1, which followed the cases of 1 per 2,500 in 3,300 live births, chemotherapy did not seem to reduce mortality, and its effectiveness should be questioned. (wikipedia.org)
  • patients
  • The p53 (a tumor suppressor gene in the normal population) genome on 17p in neurofibrosarcoma patients is mutated, increasing the probability of cancer. (wikipedia.org)