Can also classify as: Cutaneous, subcutaneous, plexiform
Malignant peripheral nerve sheath tumor (MPNST): Spindle cell sarcoma arising from nerve or BPNST
Plexiform neurofibromas have ↑ 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
Koike H et al: Diffusion-weighted magnetic resonance imaging improves the accuracy of differentiation of benign from malignant peripheral nerve sheath tumors. World Neurosurg. ePub, 2021
Liu Y et al: Correlation between NF1 genotype and imaging phenotype on whole-body MRI: NF1 radiogenomics. Neurology. 94(24):e2521-31, 2020
Markham A et al: Selumetinib: first approval. Drugs. 80(9):931-7, 2020
Ahlawat S et al: Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 87(7 Suppl 1):S31-9, 2016
Tovmassian D et al: The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol. 2016:6162182, 2016
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
Sabatini C et al: Treatment of neurofibromatosis type 1. Curr Treat Options Neurol. 17(6):355, 2015
Salamon J et al: Nerve sheath tumors in neurofibromatosis type 1: assessment of whole-body metabolic tumor burden using F-18-FDG PET/CT. PLoS One. 10(12):e0143305, 2015
Kransdorf MJ et al: Neurogenic lesions. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott, Williams & Wilkins. 395-460, 2014
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
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
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
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
Van Meerbeeck SF et al: Whole body MR imaging in neurofibromatosis type 1. Eur J Radiol. 69(2):236-42, 2009
Mautner VF et al: MRI growth patterns of plexiform neurofibromas in patients with neurofibromatosis type 1. Neuroradiology. 48(3):160-5, 2006
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
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
Synonyms
Neurofibromatosis type 1 (NF1): von Recklinghausen disease
Definitions
Neurofibroma: Type of benign peripheral nerve sheath tumor (BPNST)
Can also classify as: Cutaneous, subcutaneous, plexiform
Malignant peripheral nerve sheath tumor (MPNST): Spindle cell sarcoma arising from nerve or BPNST
Plexiform neurofibromas have ↑ 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
Koike H et al: Diffusion-weighted magnetic resonance imaging improves the accuracy of differentiation of benign from malignant peripheral nerve sheath tumors. World Neurosurg. ePub, 2021
Liu Y et al: Correlation between NF1 genotype and imaging phenotype on whole-body MRI: NF1 radiogenomics. Neurology. 94(24):e2521-31, 2020
Markham A et al: Selumetinib: first approval. Drugs. 80(9):931-7, 2020
Ahlawat S et al: Current whole-body MRI applications in the neurofibromatoses: NF1, NF2, and schwannomatosis. Neurology. 87(7 Suppl 1):S31-9, 2016
Tovmassian D et al: The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol. 2016:6162182, 2016
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
Sabatini C et al: Treatment of neurofibromatosis type 1. Curr Treat Options Neurol. 17(6):355, 2015
Salamon J et al: Nerve sheath tumors in neurofibromatosis type 1: assessment of whole-body metabolic tumor burden using F-18-FDG PET/CT. PLoS One. 10(12):e0143305, 2015
Kransdorf MJ et al: Neurogenic lesions. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott, Williams & Wilkins. 395-460, 2014
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
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
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
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
Van Meerbeeck SF et al: Whole body MR imaging in neurofibromatosis type 1. Eur J Radiol. 69(2):236-42, 2009
Mautner VF et al: MRI growth patterns of plexiform neurofibromas in patients with neurofibromatosis type 1. Neuroradiology. 48(3):160-5, 2006
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
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