Well-circumscribed, round/oval scalp mass in skin, spinal, or peripheral nerve roots; CN rarely if ever
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Setrajcic Dragos V et al: Two novel NF1 pathogenic variants causing the creation of a new splice site in patients with neurofibromatosis type I. Front Genet. 10:762, 2019
Agrawal K et al: Incidental Neurofibroma on 18F-Fluorocholine PET/MR. Clin Nucl Med. 40(9):e455-6, 2015
Kakkar C et al: Telltale signs of peripheral neurogenic tumors on magnetic resonance imaging. Indian J Radiol Imaging. 25(4):453-8, 2015
Riccardi VM: Ketotifen suppression of NF1 neurofibroma growth over 30 years. Am J Med Genet A. 167(7):1570-7, 2015
Abramowicz A et al: Neurofibromin in neurofibromatosis type 1 - mutations in NF1gene as a cause of disease. Dev Period Med. 18(3):297-306, 2014
Ahlawat S et al: Magnetic resonance neurography of peripheral nerve tumors and tumorlike conditions. Neuroimaging Clin N Am. 24(1):171-92, 2014
Hirbe AC et al: Neurofibromatosis type 1: a multidisciplinary approach to care. Lancet Neurol. 13(8):834-43, 2014
Hirbe AC et al: BRAFV600E mutation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. Neuro Oncol. 16(3):466-7, 2014
Evans HL: Sporadic superficial diffuse neurofibromas with repeated local recurrence over many years and a tendency toward malignant change: a report of 3 cases. Am J Surg Pathol. 37(7):987-94, 2013
Canelas MM et al: Lipomatous neurofibroma associated with segmental neurofibromatosis. J Cutan Pathol. 37(6):705-6, 2010
Dimou J et al: Recurrent malignant peripheral nerve sheath tumour in a patient with neurofibromatosis Type 1: a case report. J Clin Neurosci. 16(9):1221-3, 2009
Geller M et al: Plexiform neurofibroma in the ear canal of a patient with type I neurofibromatosis. Braz J Otorhinolaryngol. 75(1):158, 2009
Gerber PA et al: Neurofibromatosis. Eur J Med Res. 14(3):102-5, 2009
Greig AV et al: Giant hemifacial plexiform neurofibroma arising from trigeminal ganglion. J Craniofac Surg. 20(3):833-6, 2009
Jhas S et al: An intracranial leiomyosarcoma in a child with neurofibromatosis type 1. Can J Neurol Sci. 36(4):491-5, 2009
Jokinen CH et al: Atypical neurofibroma of the skin and subcutaneous tissue: clinicopathologic analysis of 11 cases. J Cutan Pathol. 37(1):35-42, 2009
Le LQ et al: Cell of origin and microenvironment contribution for NF1-associated dermal neurofibromas. Cell Stem Cell. 4(5):453-63, 2009
Lu-Emerson C et al: The Neurofibromatoses. Part 1: NF1. Rev Neurol Dis. 6(2):E47-53, 2009
Mantripragada KK et al: Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array. Genes Chromosomes Cancer. 48(10):897-907, 2009
Morales J et al: Glaucoma and globe enlargement associated with neurofibromatosis type 1. Ophthalmology. 116(9):1725-30, 2009
Morris ZS et al: The neurofibroma cell of origin: SKPs expand the playing field. Cell Stem Cell. 4(5):371-2, 2009
Parrinello S et al: Neurofibroma development in NF1--insights into tumour initiation. Trends Cell Biol. 19(8):395-403, 2009
Parsons CM et al: Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 18(1):175-96, x, 2009
Rallis E et al: Giant plexiform neurofibroma in a patient with neurofibromatosis type I. Dermatol Online J. 15(5):7, 2009
Riccardi VM: Diagnostic and management considerations posed by multiple café au lait spots. Arch Dermatol. 145(8):929-30, 2009
Santaolalla F et al: Severe exophthalmos in trigeminal plexiform neurofibroma involving the orbit and the infratemporal fossa. J Clin Neurosci. 16(7):970-2, 2009
Scheithauer BW et al: Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol. 33(3):325-38, 2009
Sehgal VN et al: Evaluation of plexiform neurofibroma in neurofibromatosis type 1 in 18 family members of 3 generations: ultrasonography and magnetic resonance imaging a diagnostic supplement. Int J Dermatol. 48(3):275-9, 2009
Sehgal VN et al: Plexiform neurofibromas in neurofibromatosis type 1. Int J Dermatol. 48(9):971-4, 2009
Thomas C et al: Cutaneous malignant peripheral nerve sheath tumors. J Cutan Pathol. 36(8):896-900, 2009
Yang L et al: An unusual presentation of a solitary benign giant neurofibroma. Case report. J Neurosurg Spine. 11(1):49-52, 2009
MacCollin M et al: Diagnostic criteria for schwannomatosis. Neurology. 64(11):1838-45, 2005
Tucker T et al: Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology. 65(2):205-11, 2005
Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
Friedrich RE et al: Growth type of plexiform neurofibromas in NF1 determined on magnetic resonance images. Anticancer Res. 23(2A):949-52, 2003
Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003
Woodruff JM et al: Neurofibroma. In Kleihues P et al: Tumours of the Nervous System. Lyon: IARC Press. 167-8, 2000
Gutmann DH et al: The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA. 278(1):51-7, 1997
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
Abbreviations
Neurofibroma (NF)
Definitions
Plexiform NF (PNF): Infiltrative tumor
Exclusively in neurofibromatosis type 1 (NF1) patients
Poorly delineated, worm-like, soft tissue mass infiltrating orbit (CNI) (most commonly), scalp, or parotid gland (CNVII)
Well-circumscribed, round/oval scalp mass in skin, spinal, or peripheral nerve roots; CN rarely if ever
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Setrajcic Dragos V et al: Two novel NF1 pathogenic variants causing the creation of a new splice site in patients with neurofibromatosis type I. Front Genet. 10:762, 2019
Agrawal K et al: Incidental Neurofibroma on 18F-Fluorocholine PET/MR. Clin Nucl Med. 40(9):e455-6, 2015
Kakkar C et al: Telltale signs of peripheral neurogenic tumors on magnetic resonance imaging. Indian J Radiol Imaging. 25(4):453-8, 2015
Riccardi VM: Ketotifen suppression of NF1 neurofibroma growth over 30 years. Am J Med Genet A. 167(7):1570-7, 2015
Abramowicz A et al: Neurofibromin in neurofibromatosis type 1 - mutations in NF1gene as a cause of disease. Dev Period Med. 18(3):297-306, 2014
Ahlawat S et al: Magnetic resonance neurography of peripheral nerve tumors and tumorlike conditions. Neuroimaging Clin N Am. 24(1):171-92, 2014
Hirbe AC et al: Neurofibromatosis type 1: a multidisciplinary approach to care. Lancet Neurol. 13(8):834-43, 2014
Hirbe AC et al: BRAFV600E mutation in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors. Neuro Oncol. 16(3):466-7, 2014
Evans HL: Sporadic superficial diffuse neurofibromas with repeated local recurrence over many years and a tendency toward malignant change: a report of 3 cases. Am J Surg Pathol. 37(7):987-94, 2013
Canelas MM et al: Lipomatous neurofibroma associated with segmental neurofibromatosis. J Cutan Pathol. 37(6):705-6, 2010
Dimou J et al: Recurrent malignant peripheral nerve sheath tumour in a patient with neurofibromatosis Type 1: a case report. J Clin Neurosci. 16(9):1221-3, 2009
Geller M et al: Plexiform neurofibroma in the ear canal of a patient with type I neurofibromatosis. Braz J Otorhinolaryngol. 75(1):158, 2009
Gerber PA et al: Neurofibromatosis. Eur J Med Res. 14(3):102-5, 2009
Greig AV et al: Giant hemifacial plexiform neurofibroma arising from trigeminal ganglion. J Craniofac Surg. 20(3):833-6, 2009
Jhas S et al: An intracranial leiomyosarcoma in a child with neurofibromatosis type 1. Can J Neurol Sci. 36(4):491-5, 2009
Jokinen CH et al: Atypical neurofibroma of the skin and subcutaneous tissue: clinicopathologic analysis of 11 cases. J Cutan Pathol. 37(1):35-42, 2009
Le LQ et al: Cell of origin and microenvironment contribution for NF1-associated dermal neurofibromas. Cell Stem Cell. 4(5):453-63, 2009
Lu-Emerson C et al: The Neurofibromatoses. Part 1: NF1. Rev Neurol Dis. 6(2):E47-53, 2009
Mantripragada KK et al: Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array. Genes Chromosomes Cancer. 48(10):897-907, 2009
Morales J et al: Glaucoma and globe enlargement associated with neurofibromatosis type 1. Ophthalmology. 116(9):1725-30, 2009
Morris ZS et al: The neurofibroma cell of origin: SKPs expand the playing field. Cell Stem Cell. 4(5):371-2, 2009
Parrinello S et al: Neurofibroma development in NF1--insights into tumour initiation. Trends Cell Biol. 19(8):395-403, 2009
Parsons CM et al: Surgical management of neurofibromatosis. Surg Oncol Clin N Am. 18(1):175-96, x, 2009
Rallis E et al: Giant plexiform neurofibroma in a patient with neurofibromatosis type I. Dermatol Online J. 15(5):7, 2009
Riccardi VM: Diagnostic and management considerations posed by multiple café au lait spots. Arch Dermatol. 145(8):929-30, 2009
Santaolalla F et al: Severe exophthalmos in trigeminal plexiform neurofibroma involving the orbit and the infratemporal fossa. J Clin Neurosci. 16(7):970-2, 2009
Scheithauer BW et al: Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol. 33(3):325-38, 2009
Sehgal VN et al: Evaluation of plexiform neurofibroma in neurofibromatosis type 1 in 18 family members of 3 generations: ultrasonography and magnetic resonance imaging a diagnostic supplement. Int J Dermatol. 48(3):275-9, 2009
Sehgal VN et al: Plexiform neurofibromas in neurofibromatosis type 1. Int J Dermatol. 48(9):971-4, 2009
Thomas C et al: Cutaneous malignant peripheral nerve sheath tumors. J Cutan Pathol. 36(8):896-900, 2009
Yang L et al: An unusual presentation of a solitary benign giant neurofibroma. Case report. J Neurosurg Spine. 11(1):49-52, 2009
MacCollin M et al: Diagnostic criteria for schwannomatosis. Neurology. 64(11):1838-45, 2005
Tucker T et al: Association between benign and malignant peripheral nerve sheath tumors in NF1. Neurology. 65(2):205-11, 2005
Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
Friedrich RE et al: Growth type of plexiform neurofibromas in NF1 determined on magnetic resonance images. Anticancer Res. 23(2A):949-52, 2003
Poussaint TY et al: Interobserver reproducibility of volumetric MR imaging measurements of plexiform neurofibromas. AJR Am J Roentgenol. 180(2):419-23, 2003
Woodruff JM et al: Neurofibroma. In Kleihues P et al: Tumours of the Nervous System. Lyon: IARC Press. 167-8, 2000
Gutmann DH et al: The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA. 278(1):51-7, 1997
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