link
Bookmarks
Neurofibroma
Stephanie A. Bernard, MD; Catherine C. Roberts, MD; B. J. Manaster, MD, PhD, FACR
To access 4,300 diagnoses written by the world's leading experts in radiology.Try it free - 15 days
0
9
3
1

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

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

              • von Recklinghausen disease = NF1
            • Definitions

              • BPNST with combination of Schwann and other neoplastic tissue between normal nerve axons
              • 3 types of neurofibromas: localized, plexiform, and diffuse

            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. Bergqvist C et al: Neurofibromatosis 1 French national guidelines based on an extensive literature review since 1966. Orphanet J Rare Dis. 15(1):37, 2020
                                          2. Broski SM et al: Extraneural perineurioma: CT and MRI imaging characteristics. Skeletal Radiol. 49(1):109-14, 2020
                                          3. Ahlawat S et al: Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 10;93(11):e1076-84, 2019
                                          4. Miller DT et al: Health supervision for children with neurofibromatosis type 1. Pediatrics. 143(5), 2019
                                          5. Saade-Lemus S et al: Whole-body magnetic resonance imaging of pediatric cancer predisposition syndromes: special considerations, challenges and perspective. Pediatr Radiol. 49(11):1506-15, 2019
                                          6. Chong KL et al: Dystrophic scoliosis in neurofibromatosis and rib-head resection: A Case Report. Malays Orthop J. 11(3):59-62, 2017
                                          7. Ahlawat S et al: Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 87(7 Suppl 1):S31-9, 2016
                                          8. Broski SM et al: Evaluation of (18)F-FDG PET and MRI in differentiating benign and malignant peripheral nerve sheath tumors. Skeletal Radiol. 45(8):1097-105, 2016
                                          9. 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. 39(9):1234-41, 2015
                                          10. Nacey NC et al: Intraneural perineurioma of the sciatic nerve: an under-recognized nerve neoplasm with characteristic MRI findings. Skeletal Radiol. 43(3):375-9, 2014
                                          11. Ravi AK et al: Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol. 43(12):1773-8, 2014
                                          12. Patel NB et al: Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol. 199(1):W99-106, 2012
                                          13. 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
                                          14. Hassell DS et al: Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol. 190(3):582-8, 2008
                                          15. Weiss SW et al: Benign tumors of peripheral nerves. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 835-53, 872-8, 888-92, 2008
                                          16. 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
                                          17. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 335, 349-51, 2006
                                          18. Cooper JA: Plexiform neurofibromatosis involving the colon. Radiographics. 25(2):454, 2005
                                          19. Huang GS et al: On the AJR viewbox. Diffuse neurofibroma of the arm: MR characteristics. AJR Am J Roentgenol. 184(5):1711-2, 2005
                                          20. Huang GS et al: Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol. 34(5):303-6, 2005
                                          21. 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
                                          22. 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
                                          23. Jee WH et al: Extraaxial neurofibromas versus neurilemmomas: discrimination with MRI. AJR Am J Roentgenol. 183(3):629-33, 2004
                                          24. Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
                                          25. Wilkinson LM et al: Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 24 Suppl 1:S237-42, 2004
                                          26. Paksoy Y et al: Solitary juxtacortical neurofibroma of the humerus. Skeletal Radiol. 31(2):112-5, 2002
                                          27. Fortman BJ et al: Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics. 21(3):601-12, 2001
                                          28. 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
                                          29. Beggs I: Sonographic appearances of nerve tumors. J Clin Ultrasound. 27(7):363-8, 1999
                                          30. Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
                                          31. 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
                                          32. 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
                                          33. Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
                                          Related Anatomy
                                          Loading...
                                          Related Differential Diagnoses
                                          Loading...
                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

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

                                                      • von Recklinghausen disease = NF1
                                                    • Definitions

                                                      • BPNST with combination of Schwann and other neoplastic tissue between normal nerve axons
                                                      • 3 types of neurofibromas: localized, plexiform, and diffuse

                                                    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. Bergqvist C et al: Neurofibromatosis 1 French national guidelines based on an extensive literature review since 1966. Orphanet J Rare Dis. 15(1):37, 2020
                                                                                  2. Broski SM et al: Extraneural perineurioma: CT and MRI imaging characteristics. Skeletal Radiol. 49(1):109-14, 2020
                                                                                  3. Ahlawat S et al: Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 10;93(11):e1076-84, 2019
                                                                                  4. Miller DT et al: Health supervision for children with neurofibromatosis type 1. Pediatrics. 143(5), 2019
                                                                                  5. Saade-Lemus S et al: Whole-body magnetic resonance imaging of pediatric cancer predisposition syndromes: special considerations, challenges and perspective. Pediatr Radiol. 49(11):1506-15, 2019
                                                                                  6. Chong KL et al: Dystrophic scoliosis in neurofibromatosis and rib-head resection: A Case Report. Malays Orthop J. 11(3):59-62, 2017
                                                                                  7. Ahlawat S et al: Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 87(7 Suppl 1):S31-9, 2016
                                                                                  8. Broski SM et al: Evaluation of (18)F-FDG PET and MRI in differentiating benign and malignant peripheral nerve sheath tumors. Skeletal Radiol. 45(8):1097-105, 2016
                                                                                  9. 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. 39(9):1234-41, 2015
                                                                                  10. Nacey NC et al: Intraneural perineurioma of the sciatic nerve: an under-recognized nerve neoplasm with characteristic MRI findings. Skeletal Radiol. 43(3):375-9, 2014
                                                                                  11. Ravi AK et al: Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol. 43(12):1773-8, 2014
                                                                                  12. Patel NB et al: Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol. 199(1):W99-106, 2012
                                                                                  13. 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
                                                                                  14. Hassell DS et al: Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol. 190(3):582-8, 2008
                                                                                  15. Weiss SW et al: Benign tumors of peripheral nerves. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 835-53, 872-8, 888-92, 2008
                                                                                  16. 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
                                                                                  17. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 335, 349-51, 2006
                                                                                  18. Cooper JA: Plexiform neurofibromatosis involving the colon. Radiographics. 25(2):454, 2005
                                                                                  19. Huang GS et al: On the AJR viewbox. Diffuse neurofibroma of the arm: MR characteristics. AJR Am J Roentgenol. 184(5):1711-2, 2005
                                                                                  20. Huang GS et al: Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol. 34(5):303-6, 2005
                                                                                  21. 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
                                                                                  22. 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
                                                                                  23. Jee WH et al: Extraaxial neurofibromas versus neurilemmomas: discrimination with MRI. AJR Am J Roentgenol. 183(3):629-33, 2004
                                                                                  24. Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
                                                                                  25. Wilkinson LM et al: Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 24 Suppl 1:S237-42, 2004
                                                                                  26. Paksoy Y et al: Solitary juxtacortical neurofibroma of the humerus. Skeletal Radiol. 31(2):112-5, 2002
                                                                                  27. Fortman BJ et al: Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics. 21(3):601-12, 2001
                                                                                  28. 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
                                                                                  29. Beggs I: Sonographic appearances of nerve tumors. J Clin Ultrasound. 27(7):363-8, 1999
                                                                                  30. Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
                                                                                  31. 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
                                                                                  32. 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
                                                                                  33. Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991