link
Bookmarks
Plexiform Neurofibroma of Head and Neck
Daniel E. Meltzer, MD; Luke N. Ledbetter, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe
0
3
32
0

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Plexiform neurofibroma (PNF)
              • Definitions

                • PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
                  • Often found in areas of branched nerves
                  • Pathognomonic for neurofibromatosis type 1 (NF1)

              IMAGING

              • General Features

                • Imaging Recommendations

                  • CT Findings

                    • MR Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Ehara Y et al: Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1. J Dermatol. 47(2):190-2, 2020
                                          2. Espírito Santo V et al: Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience. J Neurooncol. 147(2):459-63, 2020
                                          3. Passos J et al: Dramatic improvement of a massive plexiform neurofibroma after administration of selumetinib. Pediatr Neurol. 105:69-70, 2020
                                          4. Peyron PA et al: Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1. Forensic Sci Med Pathol. 13(4):436-440, 2017
                                          5. Latham K et al: Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 135(3):845-55, 2015
                                          6. Setabutr D et al: Neurofibromatosis of the larynx causing stridor and sleep apnea. Am J Otolaryngol. 35(5):631-5, 2014
                                          7. Jett K et al: Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 12(1):1-11, 2010
                                          8. Moharir M et al: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 37(7):1309-17, 2010
                                          9. Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
                                          10. Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
                                          11. Bredella MA et al: Value of PET in the assessment of patients with neurofibromatosis type 1. AJR Am J Roentgenol. 189(4):928-35, 2007
                                          12. Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003
                                          Related Anatomy
                                          Loading...
                                          Related Differential Diagnoses
                                          Loading...
                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Plexiform neurofibroma (PNF)
                                                      • Definitions

                                                        • PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
                                                          • Often found in areas of branched nerves
                                                          • Pathognomonic for neurofibromatosis type 1 (NF1)

                                                      IMAGING

                                                      • General Features

                                                        • Imaging Recommendations

                                                          • CT Findings

                                                            • MR Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Ehara Y et al: Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1. J Dermatol. 47(2):190-2, 2020
                                                                                  2. Espírito Santo V et al: Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience. J Neurooncol. 147(2):459-63, 2020
                                                                                  3. Passos J et al: Dramatic improvement of a massive plexiform neurofibroma after administration of selumetinib. Pediatr Neurol. 105:69-70, 2020
                                                                                  4. Peyron PA et al: Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1. Forensic Sci Med Pathol. 13(4):436-440, 2017
                                                                                  5. Latham K et al: Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 135(3):845-55, 2015
                                                                                  6. Setabutr D et al: Neurofibromatosis of the larynx causing stridor and sleep apnea. Am J Otolaryngol. 35(5):631-5, 2014
                                                                                  7. Jett K et al: Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 12(1):1-11, 2010
                                                                                  8. Moharir M et al: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 37(7):1309-17, 2010
                                                                                  9. Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
                                                                                  10. Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
                                                                                  11. Bredella MA et al: Value of PET in the assessment of patients with neurofibromatosis type 1. AJR Am J Roentgenol. 189(4):928-35, 2007
                                                                                  12. Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003