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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Neurofibromatosis type 1 (NF1)
- Plexiform neurofibroma (PNF)
Definitions
- PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
- Often found in areas of branched nerves
- Pathognomonic for 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
- 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. Epub ahead of print, 2010
- Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
- Laffan EE et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 2. Tumors of fibroblastic/myofibroblastic, so-called fibrohistiocytic, muscular, lymphomatous, neurogenic, hair matrix, and uncertain origin. Radiographics. 29(4):e36, 2009
- Larizza L et al: Developmental abnormalities and cancer predisposition in neurofibromatosis type 1. Curr Mol Med. 9(5):634-53, 2009
- Parsons CM et al: Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 18(1):175-96, x, 2009
- Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
- Fadda MT et al: Intra-parotid facial nerve multiple plexiform neurofibroma in patient with NF1. Int J Pediatr Otorhinolaryngol. 72(5):553-7, 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
- Ferner RE: Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 6(4):340-51, 2007
- Lee MJ et al: Recent developments in neurofibromatosis type 1. Curr Opin Neurol. 20(2):135-41, 2007
- Son JM et al: Varying degrees of FDG uptake in multiple benign neurofibromas on PET/CT. Br J Radiol. 80(957):e222-6, 2007
- Overdiek A et al: Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas. Brain Dev. 28(5):275-80, 2006
- Pacelli J et al: Brachial plexopathy due to malignant peripheral nerve sheath tumor in neurofibromatosis type 1: case report and subject review. Muscle Nerve. 33(5):697-700, 2006
- 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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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Neurofibromatosis type 1 (NF1)
- Plexiform neurofibroma (PNF)
Definitions
- PNF: Architecturally complex neurofibroma involving multiple nerve fascicles
- Often found in areas of branched nerves
- Pathognomonic for 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
- 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. Epub ahead of print, 2010
- Brems H et al: Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1. Lancet Oncol. 10(5):508-15, 2009
- Laffan EE et al: Pediatric soft-tissue tumors and pseudotumors: MR imaging features with pathologic correlation: part 2. Tumors of fibroblastic/myofibroblastic, so-called fibrohistiocytic, muscular, lymphomatous, neurogenic, hair matrix, and uncertain origin. Radiographics. 29(4):e36, 2009
- Larizza L et al: Developmental abnormalities and cancer predisposition in neurofibromatosis type 1. Curr Mol Med. 9(5):634-53, 2009
- Parsons CM et al: Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 18(1):175-96, x, 2009
- Slam KD et al: Surgical considerations for the familial cancer syndrome, neurofibromatosis 1: a comprehensive review. Am Surg. 75(2):120-8, 2009
- Fadda MT et al: Intra-parotid facial nerve multiple plexiform neurofibroma in patient with NF1. Int J Pediatr Otorhinolaryngol. 72(5):553-7, 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
- Ferner RE: Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. 6(4):340-51, 2007
- Lee MJ et al: Recent developments in neurofibromatosis type 1. Curr Opin Neurol. 20(2):135-41, 2007
- Son JM et al: Varying degrees of FDG uptake in multiple benign neurofibromas on PET/CT. Br J Radiol. 80(957):e222-6, 2007
- Overdiek A et al: Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas. Brain Dev. 28(5):275-80, 2006
- Pacelli J et al: Brachial plexopathy due to malignant peripheral nerve sheath tumor in neurofibromatosis type 1: case report and subject review. Muscle Nerve. 33(5):697-700, 2006
- Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003
