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Plexiform Neurofibroma
Arthur B. Meyers, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Neurofibromatosis type 1 (NF1)
              • Benign peripheral nerve sheath tumor (BPNST)
              • Malignant peripheral nerve sheath tumor (MPNST)
            • Synonyms

              • NF1: von Recklinghausen disease
            • Definitions

              • Neurofibroma: Type of BPNST
                • 3 neurofibroma subtypes: Localized, diffuse, plexiform
              • MPNST: Spindle cell sarcoma arising from nerve or BPNST
                • Plexiform neurofibromas at ↑ risk of MPNST
                • Associated with NF1 in 25-70% of cases

            IMAGING

            • General Features

              • Radiographic Findings

                • Ultrasonographic Findings

                  • CT Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Reporting Tips

                                          Selected References

                                          1. Chirindel A et al: 18F-FDG PET/CT qualitative and quantitative evaluation in neurofibromatosis type 1 patients for detection of malignant transformation: comparison of early to delayed imaging with and without liver activity normalization. J Nucl Med. 56(3):379-85, 2015
                                          2. Salamon J et al: Nerve sheath tumors in neurofibromatosis type 1: assessment of bhole-body metabolic tumor burden using F-18-FDG PET/CT. PLoS One. 10(12):e0143305, 2015
                                          3. Kransdorf MJ et al: Neurogenic lesions. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Philadelphia: Lippincott, Williams & Wilkins. 395-460, 2014
                                          4. Delgado J et al: MRI features of plexiform neurofibromas involving the liver and pancreas in children with neurofibromatosis type 1. Clin Radiol. 69(6):e280-4, 2014
                                          5. Nguyen R et al: Benign whole body tumor volume is a risk factor for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. J Neurooncol. 116(2):307-13, 2014
                                          6. Salamon J et al: Evaluation of intratumoural heterogeneity on ¹⁸F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 40(5):685-92, 2013
                                          7. Wasa J et al: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                          8. Van Meerbeeck SF et al: Whole body MR imaging in neurofibromatosis type 1. Eur J Radiol. 69(2):236-42, 2009
                                          9. Mautner VF et al: MRI growth patterns of plexiform neurofibromas in patients with neurofibromatosis type 1. Neuroradiology. 48(3):160-5, 2006
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Neurofibromatosis type 1 (NF1)
                                                      • Benign peripheral nerve sheath tumor (BPNST)
                                                      • Malignant peripheral nerve sheath tumor (MPNST)
                                                    • Synonyms

                                                      • NF1: von Recklinghausen disease
                                                    • Definitions

                                                      • Neurofibroma: Type of BPNST
                                                        • 3 neurofibroma subtypes: Localized, diffuse, plexiform
                                                      • MPNST: Spindle cell sarcoma arising from nerve or BPNST
                                                        • Plexiform neurofibromas at ↑ risk of MPNST
                                                        • Associated with NF1 in 25-70% of cases

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • Ultrasonographic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Reporting Tips

                                                                                  Selected References

                                                                                  1. Chirindel A et al: 18F-FDG PET/CT qualitative and quantitative evaluation in neurofibromatosis type 1 patients for detection of malignant transformation: comparison of early to delayed imaging with and without liver activity normalization. J Nucl Med. 56(3):379-85, 2015
                                                                                  2. Salamon J et al: Nerve sheath tumors in neurofibromatosis type 1: assessment of bhole-body metabolic tumor burden using F-18-FDG PET/CT. PLoS One. 10(12):e0143305, 2015
                                                                                  3. Kransdorf MJ et al: Neurogenic lesions. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Philadelphia: Lippincott, Williams & Wilkins. 395-460, 2014
                                                                                  4. Delgado J et al: MRI features of plexiform neurofibromas involving the liver and pancreas in children with neurofibromatosis type 1. Clin Radiol. 69(6):e280-4, 2014
                                                                                  5. Nguyen R et al: Benign whole body tumor volume is a risk factor for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. J Neurooncol. 116(2):307-13, 2014
                                                                                  6. Salamon J et al: Evaluation of intratumoural heterogeneity on ¹⁸F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 40(5):685-92, 2013
                                                                                  7. Wasa J et al: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                                                                  8. Van Meerbeeck SF et al: Whole body MR imaging in neurofibromatosis type 1. Eur J Radiol. 69(2):236-42, 2009
                                                                                  9. Mautner VF et al: MRI growth patterns of plexiform neurofibromas in patients with neurofibromatosis type 1. Neuroradiology. 48(3):160-5, 2006