Bookmarks
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
- 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
- Broski SM et al: Extraneural perineurioma: CT and MRI imaging characteristics. Skeletal Radiol. 49(1):109-14, 2020
- Ahlawat S et al: Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 10;93(11):e1076-84, 2019
- Miller DT et al: Health supervision for children with neurofibromatosis type 1. Pediatrics. 143(5), 2019
- 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
- Chong KL et al: Dystrophic scoliosis in neurofibromatosis and rib-head resection: A Case Report. Malays Orthop J. 11(3):59-62, 2017
- Ahlawat S et al: Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 87(7 Suppl 1):S31-9, 2016
- 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
- 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
- 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
- Ravi AK et al: Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol. 43(12):1773-8, 2014
- Patel NB et al: Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol. 199(1):W99-106, 2012
- 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
- Hassell DS et al: Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol. 190(3):582-8, 2008
- 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
- 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
- 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
- Cooper JA: Plexiform neurofibromatosis involving the colon. Radiographics. 25(2):454, 2005
- Huang GS et al: On the AJR viewbox. Diffuse neurofibroma of the arm: MR characteristics. AJR Am J Roentgenol. 184(5):1711-2, 2005
- Huang GS et al: Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol. 34(5):303-6, 2005
- 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
- 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
- Jee WH et al: Extraaxial neurofibromas versus neurilemmomas: discrimination with MRI. AJR Am J Roentgenol. 183(3):629-33, 2004
- Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
- Wilkinson LM et al: Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 24 Suppl 1:S237-42, 2004
- Paksoy Y et al: Solitary juxtacortical neurofibroma of the humerus. Skeletal Radiol. 31(2):112-5, 2002
- Fortman BJ et al: Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics. 21(3):601-12, 2001
- 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
- Beggs I: Sonographic appearances of nerve tumors. J Clin Ultrasound. 27(7):363-8, 1999
- Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
- 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
- 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
- Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
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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
- 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
- Broski SM et al: Extraneural perineurioma: CT and MRI imaging characteristics. Skeletal Radiol. 49(1):109-14, 2020
- Ahlawat S et al: Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 10;93(11):e1076-84, 2019
- Miller DT et al: Health supervision for children with neurofibromatosis type 1. Pediatrics. 143(5), 2019
- 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
- Chong KL et al: Dystrophic scoliosis in neurofibromatosis and rib-head resection: A Case Report. Malays Orthop J. 11(3):59-62, 2017
- Ahlawat S et al: Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 87(7 Suppl 1):S31-9, 2016
- 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
- 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
- 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
- Ravi AK et al: Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum. Skeletal Radiol. 43(12):1773-8, 2014
- Patel NB et al: Musculoskeletal manifestations of neurofibromatosis type 1. AJR Am J Roentgenol. 199(1):W99-106, 2012
- 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
- Hassell DS et al: Imaging appearance of diffuse neurofibroma. AJR Am J Roentgenol. 190(3):582-8, 2008
- 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
- 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
- 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
- Cooper JA: Plexiform neurofibromatosis involving the colon. Radiographics. 25(2):454, 2005
- Huang GS et al: On the AJR viewbox. Diffuse neurofibroma of the arm: MR characteristics. AJR Am J Roentgenol. 184(5):1711-2, 2005
- Huang GS et al: Solitary intraosseous neurofibroma of the tibia. Skeletal Radiol. 34(5):303-6, 2005
- 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
- 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
- Jee WH et al: Extraaxial neurofibromas versus neurilemmomas: discrimination with MRI. AJR Am J Roentgenol. 183(3):629-33, 2004
- Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
- Wilkinson LM et al: Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 24 Suppl 1:S237-42, 2004
- Paksoy Y et al: Solitary juxtacortical neurofibroma of the humerus. Skeletal Radiol. 31(2):112-5, 2002
- Fortman BJ et al: Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics. 21(3):601-12, 2001
- 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
- Beggs I: Sonographic appearances of nerve tumors. J Clin Ultrasound. 27(7):363-8, 1999
- Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
- 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
- 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
- Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
