Neurofibroma classification: Difference between revisions

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==Classification==
==Classification==
[[Neurofibroma]] may be [[Classification|classified]] into following subtypes:<ref name="libre">Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2 Accessed on November 17, 2015 </ref><ref>http://www.nfmidwest.org/wp-content/uploads/2016/03/Neurofibromas.pdf</ref><ref>https://pubs.rsna.org/doi/10.1148/rg.24si035170#REF8</ref><ref>http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/</ref>
[[Neurofibroma]] may be [[Classification|classified]] into following subtypes:<ref name="WilkinsonManson2004">{{cite journal|last1=Wilkinson|first1=Lana M.|last2=Manson|first2=David|last3=Smith|first3=Charles R.|title=Best Cases from the AFIP|journal=RadioGraphics|volume=24|issue=suppl_1|year=2004|pages=S237–S242|issn=0271-5333|doi=10.1148/rg.24si035170}}</ref><ref>https://pubs.rsna.org/doi/10.1148/rg.24si035170#REF8</ref>
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* [[Malignant]] [[transformation]] very rare
* [[Malignant]] [[transformation]] very rare
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| style="background:#DCDCDC;" align="center" + |'''Localized Intraneural neurofibroma'''
| style="background:#DCDCDC;" align="center" + |'''Localized Intraneural [[neurofibroma]]'''
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* Second-most common type
* Second-most common type
* Segmental, fusiform nerve enlargement
* [[Segmental analysis (biology)|Segmental]], [[fusiform]] [[nerve]] enlargement
* Residual [[axons]] traverse through lesion
* [[Residual]] [[axons]] traverse through [[lesion]]
** Neurofilament [[immunohistochemistry]] and Bielshowsky stain show axons within center of lesion
** [[Neurofilament]] [[immunohistochemistry]] and Bielshowsky [[stain]] show [[axons]] within center of lesion
* Often contains coarse, refractile [[collagen]]
* Often contains coarse, refractile [[collagen]]
* [[Malignant]] change infrequent
* [[Malignant]] [[Change detection|change]] infrequent
|-
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| style="background:#DCDCDC;" align="center" + |[[Subcutaneous|'''Subcutaneous''']] '''neurofibroma'''
| style="background:#DCDCDC;" align="center" + |[[Subcutaneous|'''Subcutaneous''']] '''[[neurofibroma]]'''
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* Common
* Common
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* They are often a form of [[Plexiform neurofibroma|plexiform neurofibromas]]
* They are often a form of [[Plexiform neurofibroma|plexiform neurofibromas]]
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| style="background:#DCDCDC;" align="center" + |[[Diffuse|'''Diffuse''']] '''neurofibroma'''
| style="background:#DCDCDC;" align="center" + |[[Diffuse|'''Diffuse''']] '''[[neurofibroma]]'''
'''(superficial)'''
'''([[superficial]])'''
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* [[Superficial]]
* [[Superficial]]
* Uncommon
* Uncommon
* Feels soft and squishy
* Feels [[Soft tissue|soft]] and squishy
* Most common in head (scalp) and neck region or trunk (often protruding out, like a “love handle”)
* Most common in [[head]] ([[scalp]]) and [[neck]] region or [[trunk]] (often protruding out, like a “[[Love handles|love handle]]”)
* Ill defined margins, can't tell where tumor stops
* Ill defined margins, can't tell where [[tumor]] stops
* Runs through the full [[skin]] thickness (from the surface all the way down to the base of the skin ([[subcutaneous]] fascia/fat)
* Runs through the full [[skin]] thickness (from the [[Surface anatomy|surface]] all the way down to the [[base]] of the [[skin]] ([[subcutaneous]] [[fascia]]/[[fat]])
* Doesn't go deeper than [[Tenon's capsule|fascia]]
* Doesn't go deeper than [[Tenon's capsule|fascia]]
* Nondestructive, envelops normal structures (e.g., fat cells and adenexal structures)
* [[Nondestructive testing|Nondestructive]], [[Envelope (biology)|envelops]] normal [[Structure factor|structures]] (e.g., [[fat cells]] and adenexal [[Structure factor|structures]])
* Uniform matrix of fine, [[Collagen|fibrillary collagen]]
* Uniform [[matrix]] of fine, [[Collagen|fibrillary collagen]]
* Shorter, rounder [[Schwann cells]]
* Shorter, rounder [[Schwann cells]]
* Clusters of pseudo-meissnerian body-like structures may be seen
* [[Cluster (epidemiology)|Clusters]] of pseudo-meissnerian [[body]]-like structures may be seen
* Usually seen in early childhood and young adults
* Usually seen in early [[childhood]] and [[young adults]]
* Usually associated with diffuse [[hyperpigmentation]], so looks like a very large squishy [[café au lait spot]]
* Usually associated with [[diffuse]] [[hyperpigmentation]], so looks like a very large squishy [[café au lait spot]]
* Solitary lesions and not related to any inherited condition (rarely associated with [[NF1]])
* [[Solitary]] [[lesions]] and not related to any [[inherited]] [[condition]] (rarely associated with [[NF1]])
* Rare malignant change
* Rare [[malignant]] [[Change detection|change]]
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| style="background:#DCDCDC;" align="center" + |[[Intramuscular|'''Intramuscular''']] '''neurofibroma'''
| style="background:#DCDCDC;" align="center" + |[[Intramuscular|'''Intramuscular''']] '''[[neurofibroma]]'''
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* Common
* Common
* Usually isolated [[tumors]] in the [[muscle]]
* Usually isolated [[tumors]] in the [[muscle]]
* Growths along very small nerves
* [[Growth|Growths]] along very small [[nerves]]
* Causes pain sometimes
* Causes [[pain]] sometimes
* Can be removed  (leaves a scar behind)
* Can be removed  (leaves a [[scar]] behind)
* Sometimes can occur as [[Plexiform neurofibroma|plexiform neurofibromas]] in form of chains or networks
* Sometimes can occur as [[Plexiform neurofibroma|plexiform neurofibromas]] in form of [[Chain (sequence)|chains]] or [[Network effect|networks]]
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| style="background:#DCDCDC;" align="center" + |'''[[Plexiform neurofibroma|Plexiform]] neurofibroma'''
| style="background:#DCDCDC;" align="center" + |'''[[Plexiform neurofibroma|Plexiform neurofibroma]]'''
'''(deep)'''
'''(deep)'''
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* Deep
* Deep
* Associated with [[NF1]]
* Associated with [[NF1]]
* Diffuse involvement along a large nerve and its branches
* [[Diffuse]] involvement along a large [[nerve]] and its [[Branches of medicine|branches]]
* Mostly internal/ intraneural
* Mostly [[internal]]/ intraneural
* Can also involve small nerves and superficial skin
* Can also involve small [[nerves]] and [[superficial]] [[skin]]
* Have more [[connective tissue]]/extracellular matrix than cutaneous [[Neurofibroma|neurofibromas]], that separates the [[nerve fibers]]
* Have more [[connective tissue]]/[[extracellular matrix]] than [[cutaneous]] [[Neurofibroma|neurofibromas]], that separates the [[nerve fibers]]
* Gross enlargement of the nerve with nodular tumor development results in the gross pathologic appearance referred to as “bag of worms”
* [[Gross]] enlargement of the [[nerve]] with [[nodular]] [[tumor]] [[development]] results in the [[gross]] [[Pathological|pathologic]] [[appearance]] referred to as “bag of worms”
* Generally believed to be present at [[birth]] (congenital)
* Generally believed to be present at [[birth]] ([[congenital]])
* Disfiguring
* Disfiguring
* Affects function due to sheer size as well as neurovascular compromise
* Affects [[Function (biology)|function]] due to sheer size as well as [[Neurovascular bundle|neurovascular]] compromise
* Upto 5% risk of malignant transformation
* Upto 5% risk of [[malignant]] [[transformation]]
*Plexiform neurofibroma exhibits following features on T2-weighted MRI:
*[[Plexiform neurofibroma]] exhibits following [[Features (pattern recognition)|features]] on T2-weighted [[Magnetic resonance imaging|MRI]]:
**Target sign (low signal intensity centrally with a ring of high signal intensity peripherally)
**Target [[Sign (medicine)|sign]] (low [[Signal (biology)|signal]] [[intensity]] centrally with a ring of high [[Signal (biology)|signal]] [[intensity]] peripherally)
**Fascicular sign
**Fascicular [[Sign (medical)|sign]]
**May appear as a larger and more infiltrating mass with lobulated borders with inhomogeneous enhancement
**May [[Appearance|appear]] as a larger and more [[Infiltration (medical)|infiltrating]] [[mass]] with lobulated borders with inhomogeneous enhancement
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| style="background:#DCDCDC;" align="center" + |'''Pigmented neurofibroma'''  
| style="background:#DCDCDC;" align="center" + |'''[[Pigmented lesions|Pigmented]] [[neurofibroma]]'''  
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* Neurofibroma with melanin-bearing pigmented cells, usually only appreciated microscopically
* [[Neurofibroma]] with [[melanin]]-bearing [[Pigmented Lesions|pigmented]] [[Cells (biology)|cells]], usually only appreciated [[Microscopic|microscopically]]
* Not considered a true subtype
* Not considered a true subtype
* No increased risk of malignant transformation
* No increased risk of [[Malignancy|malignan]]<nowiki/>t [[transformation]]
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[[Plexiform neurofibroma|Plexiform neurofibromas]] can be further [[Subclass (biology)|subclassified]] into following:<ref>http://www.nfmidwest.org/wp-content/uploads/2016/03/Neurofibromas.pdf</ref>
[[Plexiform neurofibroma|Plexiform neurofibromas]] can be further [[Subclass (biology)|subclassified]] into following:
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! style="background:#4479BA; color: #FFFFFF;" align="center" + |Characteristics/Description
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Characteristics/Description
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| style="background:#DCDCDC;" align="center" + |'''[[Diffuse]] [[Plexiform neurofibroma|Plexiform]] neurofibroma'''
| style="background:#DCDCDC;" align="center" + |'''[[Diffuse]] [[Plexiform neurofibroma|Plexiform neurofibroma]]'''
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* It extends through the [[skin]] into [[fascia]] and [[muscle]]
* It [[extends]] through the [[skin]] into [[fascia]] and [[muscle]]
* Lacks clear margins
* [[Lack (manque)|Lacks]] clear margins
* Has “little [[fingers]]” that invade [[muscle]] or other [[Tissue (biology)|tissue]]
* Has “little [[fingers]]” that invade [[muscle]] or other [[Tissue (biology)|tissue]]
* May not be easy to see in infants
* May not be easy to see in [[infants]]
* May have a large [[café au lait spot]] “above” it
* May have a large [[café au lait spot]] “above” it
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| style="background:#DCDCDC;" align="center" + |'''[[Nodular]] [[Plexiform neurofibroma|Plexiform]] neurofibroma'''
| style="background:#DCDCDC;" align="center" + |'''[[Nodular]] [[Plexiform neurofibroma|Plexiform neurofibroma]]'''
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* Usually involves [[nerves]] coming off the [[spinal cord]] or off of larger [[nerves]] (such as [[sciatic nerve]])
* Usually involves [[nerves]] coming off the [[spinal cord]] or off of larger [[nerves]] (such as [[sciatic nerve]])

Latest revision as of 15:45, 1 November 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

Neurofibroma may be classified into 5 subtypes: cutaneous/dermal/localized, localized intraneural, subcutaneous, diffuse, intramuscular, plexiform and pigmented neurofibroma. Plexiform neurofibromas may be further sub-classified into diffuse and nodular plexiform.

Classification

Neurofibroma may be classified into following subtypes:[1][2]

Types of neurofibromas Characteristics/Description
Cutaneous/Dermal/Localized/Sporadic neurofibroma (90%)
Localized Intraneural neurofibroma
Subcutaneous neurofibroma
Diffuse neurofibroma

(superficial)

Intramuscular neurofibroma
Plexiform neurofibroma

(deep)

Pigmented neurofibroma

Plexiform neurofibromas can be further subclassified into following:

Types of neurofibromas Characteristics/Description
Diffuse Plexiform neurofibroma
Nodular Plexiform neurofibroma

References

  1. Wilkinson, Lana M.; Manson, David; Smith, Charles R. (2004). "Best Cases from the AFIP". RadioGraphics. 24 (suppl_1): S237–S242. doi:10.1148/rg.24si035170. ISSN 0271-5333.
  2. https://pubs.rsna.org/doi/10.1148/rg.24si035170#REF8


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