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Plexiform Neurofibroma of Head and Neck
Luke N. Ledbetter, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Neurofibromatosis type 1 (NF1)
                • Plexiform neurofibroma (PNF)
              • Definitions

                • PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
                  • Often found in areas of branched nerves
                  • Pathognomonic for 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. Latham K et al: Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 135(3):845-55, 2015
                                          2. Setabutr D et al: Neurofibromatosis of the larynx causing stridor and sleep apnea. Am J Otolaryngol. 35(5):631-5, 2014
                                          3. Jett K et al: Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 12(1):1-11, 2010
                                          4. Moharir M et al: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. Epub ahead of print, 2010
                                          5. Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
                                          6. Laffan EE et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 2. Tumors of fibroblastic/myofibroblastic, so-called fibrohistiocytic, muscular, lymphomatous, neurogenic, hair matrix, and uncertain origin. Radiographics. 29(4):e36, 2009
                                          7. Larizza L et al: Developmental abnormalities and cancer predisposition in neurofibromatosis type 1. Curr Mol Med. 9(5):634-53, 2009
                                          8. Parsons CM et al: Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 18(1):175-96, x, 2009
                                          9. Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
                                          10. Fadda MT et al: Intra-parotid facial nerve multiple plexiform neurofibroma in patient with NF1. Int J Pediatr Otorhinolaryngol. 72(5):553-7, 2008
                                          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. Ferner RE: Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 6(4):340-51, 2007
                                          13. Lee MJ et al: Recent developments in neurofibromatosis type 1. Curr Opin Neurol. 20(2):135-41, 2007
                                          14. Son JM et al: Varying degrees of FDG uptake in multiple benign neurofibromas on PET/CT. Br J Radiol. 80(957):e222-6, 2007
                                          15. Overdiek A et al: Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas. Brain Dev. 28(5):275-80, 2006
                                          16. Pacelli J et al: Brachial plexopathy due to malignant peripheral nerve sheath tumor in neurofibromatosis type 1: case report and subject review. Muscle Nerve. 33(5):697-700, 2006
                                          17. 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
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Neurofibromatosis type 1 (NF1)
                                                        • Plexiform neurofibroma (PNF)
                                                      • Definitions

                                                        • PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
                                                          • Often found in areas of branched nerves
                                                          • Pathognomonic for 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. Latham K et al: Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 135(3):845-55, 2015
                                                                                  2. Setabutr D et al: Neurofibromatosis of the larynx causing stridor and sleep apnea. Am J Otolaryngol. 35(5):631-5, 2014
                                                                                  3. Jett K et al: Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 12(1):1-11, 2010
                                                                                  4. Moharir M et al: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. Epub ahead of print, 2010
                                                                                  5. Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
                                                                                  6. Laffan EE et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 2. Tumors of fibroblastic/myofibroblastic, so-called fibrohistiocytic, muscular, lymphomatous, neurogenic, hair matrix, and uncertain origin. Radiographics. 29(4):e36, 2009
                                                                                  7. Larizza L et al: Developmental abnormalities and cancer predisposition in neurofibromatosis type 1. Curr Mol Med. 9(5):634-53, 2009
                                                                                  8. Parsons CM et al: Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 18(1):175-96, x, 2009
                                                                                  9. Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
                                                                                  10. Fadda MT et al: Intra-parotid facial nerve multiple plexiform neurofibroma in patient with NF1. Int J Pediatr Otorhinolaryngol. 72(5):553-7, 2008
                                                                                  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. Ferner RE: Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 6(4):340-51, 2007
                                                                                  13. Lee MJ et al: Recent developments in neurofibromatosis type 1. Curr Opin Neurol. 20(2):135-41, 2007
                                                                                  14. Son JM et al: Varying degrees of FDG uptake in multiple benign neurofibromas on PET/CT. Br J Radiol. 80(957):e222-6, 2007
                                                                                  15. Overdiek A et al: Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas. Brain Dev. 28(5):275-80, 2006
                                                                                  16. Pacelli J et al: Brachial plexopathy due to malignant peripheral nerve sheath tumor in neurofibromatosis type 1: case report and subject review. Muscle Nerve. 33(5):697-700, 2006
                                                                                  17. Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003