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Neurofibroma
Catherine C. Roberts, MD; B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Neurofibroma (NF)
            • Neurofibromatosis type 1 (NF1)
          • Definitions

            • Benign peripheral nerve sheath tumor with neoplastic tissue interspersed with normal nerve bundles

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Schaefer IM et al: Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. ePub, 2015
                                        2. Ravi AK et al: Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol. 43(12):1773-8, 2014
                                        3. Patel NB et al: Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol. 199(1):W99-106, 2012
                                        4. Wasa J, Nishida Y, Tsukushi S, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                        5. Hassell DS et al: Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol. 190(3):582-8, 2008
                                        6. Weiss SW et al: Benign tumors of peripheral nerves. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 835-53, 872-8, 888-92, 2008
                                        7. 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
                                        8. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 335, 349-51, 2006
                                        9. Cooper JA: Plexiform neurofibromatosis involving the colon. Radiographics. 25(2):454, 2005
                                        10. Huang GS et al: On the AJR viewbox. Diffuse neurofibroma of the arm: MR characteristics. AJR Am J Roentgenol. 184(5):1711-2, 2005
                                        11. Huang GS et al: Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol. 34(5):303-6, 2005
                                        12. Levy AD et al: From the archives of the AFIP: abdominal neoplasms in patients with neurofibromatosis type 1: radiologic-pathologic correlation. Radiographics. 25(2):455-80, 2005
                                        13. Lim R et al: Superficial neurofibroma: a lesion with unique MRI characteristics in patients with neurofibromatosis type 1. AJR Am J Roentgenol. 184(3):962-8, 2005
                                        14. Jee WH et al: Extraaxial neurofibromas versus neurilemmomas: discrimination with MRI. AJR Am J Roentgenol. 183(3):629-33, 2004
                                        15. Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
                                        16. Wilkinson LM et al: Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 24 Suppl 1:S237-42, 2004
                                        17. Paksoy Y et al: Solitary juxtacortical neurofibroma of the humerus. Skeletal Radiol. 31(2):112-5, 2002
                                        18. Fortman BJ et al: Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics. 21(3):601-12, 2001
                                        19. Lin J et al: Cross-sectional imaging of peripheral nerve sheath tumors: characteristic signs on CT, MR imaging, and sonography. AJR Am J Roentgenol. 176(1):75-82, 2001
                                        20. Beggs I: Sonographic appearances of nerve tumors. J Clin Ultrasound. 27(7):363-8, 1999
                                        21. Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
                                        22. Ogose A et al: Tumors of peripheral nerves: correlation of symptoms, clinical signs, imaging features, and histologic diagnosis. Skeletal Radiol. 28(4):183-8, 1999
                                        23. Sullivan TP et al: Case report 828: Plexiform neurofibroma of the tibial nerve invading the medial and lateral gastrocnemius muscles and plantaris muscle. Skeletal Radiol. 23(2):149-52, 1994
                                        24. Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Neurofibroma (NF)
                                                  • Neurofibromatosis type 1 (NF1)
                                                • Definitions

                                                  • Benign peripheral nerve sheath tumor with neoplastic tissue interspersed with normal nerve bundles

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Nuclear Medicine Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Schaefer IM et al: Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. ePub, 2015
                                                                              2. Ravi AK et al: Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol. 43(12):1773-8, 2014
                                                                              3. Patel NB et al: Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol. 199(1):W99-106, 2012
                                                                              4. Wasa J, Nishida Y, Tsukushi S, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                                                              5. Hassell DS et al: Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol. 190(3):582-8, 2008
                                                                              6. Weiss SW et al: Benign tumors of peripheral nerves. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 835-53, 872-8, 888-92, 2008
                                                                              7. 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
                                                                              8. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 335, 349-51, 2006
                                                                              9. Cooper JA: Plexiform neurofibromatosis involving the colon. Radiographics. 25(2):454, 2005
                                                                              10. Huang GS et al: On the AJR viewbox. Diffuse neurofibroma of the arm: MR characteristics. AJR Am J Roentgenol. 184(5):1711-2, 2005
                                                                              11. Huang GS et al: Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol. 34(5):303-6, 2005
                                                                              12. Levy AD et al: From the archives of the AFIP: abdominal neoplasms in patients with neurofibromatosis type 1: radiologic-pathologic correlation. Radiographics. 25(2):455-80, 2005
                                                                              13. Lim R et al: Superficial neurofibroma: a lesion with unique MRI characteristics in patients with neurofibromatosis type 1. AJR Am J Roentgenol. 184(3):962-8, 2005
                                                                              14. Jee WH et al: Extraaxial neurofibromas versus neurilemmomas: discrimination with MRI. AJR Am J Roentgenol. 183(3):629-33, 2004
                                                                              15. Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
                                                                              16. Wilkinson LM et al: Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 24 Suppl 1:S237-42, 2004
                                                                              17. Paksoy Y et al: Solitary juxtacortical neurofibroma of the humerus. Skeletal Radiol. 31(2):112-5, 2002
                                                                              18. Fortman BJ et al: Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics. 21(3):601-12, 2001
                                                                              19. Lin J et al: Cross-sectional imaging of peripheral nerve sheath tumors: characteristic signs on CT, MR imaging, and sonography. AJR Am J Roentgenol. 176(1):75-82, 2001
                                                                              20. Beggs I: Sonographic appearances of nerve tumors. J Clin Ultrasound. 27(7):363-8, 1999
                                                                              21. Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
                                                                              22. Ogose A et al: Tumors of peripheral nerves: correlation of symptoms, clinical signs, imaging features, and histologic diagnosis. Skeletal Radiol. 28(4):183-8, 1999
                                                                              23. Sullivan TP et al: Case report 828: Plexiform neurofibroma of the tibial nerve invading the medial and lateral gastrocnemius muscles and plantaris muscle. Skeletal Radiol. 23(2):149-52, 1994
                                                                              24. Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991