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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Plexiform neurofibroma (PNF)
Definitions
- PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
- Often found in areas of branched nerves
- Pathognomonic for neurofibromatosis type 1 (NF1)
- PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
IMAGING
General Features
Imaging Recommendations
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Ehara Y et al: Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1. J Dermatol. 47(2):190-2, 2020
- Espírito Santo V et al: Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience. J Neurooncol. 147(2):459-63, 2020
- Passos J et al: Dramatic improvement of a massive plexiform neurofibroma after administration of selumetinib. Pediatr Neurol. 105:69-70, 2020
- Peyron PA et al: Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1. Forensic Sci Med Pathol. 13(4):436-440, 2017
- Latham K et al: Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 135(3):845-55, 2015
- Setabutr D et al: Neurofibromatosis of the larynx causing stridor and sleep apnea. Am J Otolaryngol. 35(5):631-5, 2014
- Jett K et al: Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 12(1):1-11, 2010
- Moharir M et al: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 37(7):1309-17, 2010
- Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
- Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
- 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
- Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Plexiform neurofibroma (PNF)
Definitions
- PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
- Often found in areas of branched nerves
- Pathognomonic for neurofibromatosis type 1 (NF1)
- PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
IMAGING
General Features
Imaging Recommendations
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Ehara Y et al: Distribution of diffuse plexiform neurofibroma on the body surface in patients with neurofibromatosis 1. J Dermatol. 47(2):190-2, 2020
- Espírito Santo V et al: Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience. J Neurooncol. 147(2):459-63, 2020
- Passos J et al: Dramatic improvement of a massive plexiform neurofibroma after administration of selumetinib. Pediatr Neurol. 105:69-70, 2020
- Peyron PA et al: Fatal acute retropharyngeal hemorrhage in neurofibromatosis type 1. Forensic Sci Med Pathol. 13(4):436-440, 2017
- Latham K et al: Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 135(3):845-55, 2015
- Setabutr D et al: Neurofibromatosis of the larynx causing stridor and sleep apnea. Am J Otolaryngol. 35(5):631-5, 2014
- Jett K et al: Clinical and genetic aspects of neurofibromatosis 1. Genet Med. 12(1):1-11, 2010
- Moharir M et al: Utility of positron emission tomography for tumour surveillance in children with neurofibromatosis type 1. Eur J Nucl Med Mol Imaging. 37(7):1309-17, 2010
- Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
- Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
- 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
- Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003
