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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
- Neurofibroma: Type of BPNST
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
- 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
- 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
- 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
- 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
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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
- Neurofibroma: Type of BPNST
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
- 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
- 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
- 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
- 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
