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Difference between revisions of "Neurofibroma"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = Neurofibroma_(3).jpg | | Image = Neurofibroma_(3).jpg | ||
| Width = | | Width = | ||
| Caption = Neurofibroma. [[H&E stain]]. | | Caption = Neurofibroma. [[H&E stain]]. | ||
| Micro = spindle cells with wavy nuclei without pleomorphism | | Micro = spindle cells with wavy nuclei without pleomorphism, +/-arranged in fascicles and intermixed with (wire-like) collagen ("shredded carrots"), moderately increased cellularity, poorly ''or'' well-circumscribed, +/-plexiform growth pattern ("bag of worms"), +/-mast cells (useful) | ||
| Subtypes = [[plexiform neurofibroma]] | | Subtypes = [[plexiform neurofibroma]] | ||
| LMDDx = [[schwannoma]], [[dermatofibrosarcoma protuberans]], [[ganglioneuroma]], neurotized [[melanocytic nevus]], [[MPNST]] | | LMDDx = [[schwannoma]], [[dermatofibrosarcoma protuberans]], [[ganglioneuroma]], neurotized [[melanocytic nevus]], [[MPNST]] | ||
| Line 24: | Line 24: | ||
| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = benign | | Prognosis = benign | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
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Classification:<ref name=pmid15486243>{{Cite journal | last1 = Wilkinson | first1 = LM. | last2 = Manson | first2 = D. | last3 = Smith | first3 = CR. | title = Best cases from the AFIP: plexiform neurofibroma of the bladder. | journal = Radiographics | volume = 24 Suppl 1 | issue = | pages = S237-42 | month = Oct | year = 2004 | doi = 10.1148/rg.24si035170 | PMID = 15486243 }}</ref> | Classification:<ref name=pmid15486243>{{Cite journal | last1 = Wilkinson | first1 = LM. | last2 = Manson | first2 = D. | last3 = Smith | first3 = CR. | title = Best cases from the AFIP: plexiform neurofibroma of the bladder. | journal = Radiographics | volume = 24 Suppl 1 | issue = | pages = S237-42 | month = Oct | year = 2004 | doi = 10.1148/rg.24si035170 | PMID = 15486243 }}</ref> | ||
#Localized - | #Localized - sporadic. | ||
#Diffuse - usually poorly defined, young adults and children; | #Diffuse - usually poorly defined, young adults and children; sporadic. | ||
#Plexiform - associated with NF1. | #Plexiform - associated with NF1. | ||
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Gross features (plexiform NF):<ref name=pmid15486243>{{Cite journal | last1 = Wilkinson | first1 = LM. | last2 = Manson | first2 = D. | last3 = Smith | first3 = CR. | title = Best cases from the AFIP: plexiform neurofibroma of the bladder. | journal = Radiographics | volume = 24 Suppl 1 | issue = | pages = S237-42 | month = Oct | year = 2004 | doi = 10.1148/rg.24si035170 | PMID = 15486243 }}</ref> | Gross features (plexiform NF):<ref name=pmid15486243>{{Cite journal | last1 = Wilkinson | first1 = LM. | last2 = Manson | first2 = D. | last3 = Smith | first3 = CR. | title = Best cases from the AFIP: plexiform neurofibroma of the bladder. | journal = Radiographics | volume = 24 Suppl 1 | issue = | pages = S237-42 | month = Oct | year = 2004 | doi = 10.1148/rg.24si035170 | PMID = 15486243 }}</ref> | ||
*"Bag of worms" appearance. | *"Bag of worms" appearance. | ||
<gallery> | |||
File:Neurofibroma_large_NF1.jpg |Large neurofibroma in a NF1 case (WC/jensflorian) | |||
File:Neurofibroma -1.jpg | Neurofibroma (Calicut College) | |||
File:Neurofibroma -2.jpg | Neurofibroma with typical whitish surface (Calicut College) | |||
</gallery> | |||
Radiologic:<ref name=pmid15486243/> | Radiologic:<ref name=pmid15486243/> | ||
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Features: | Features: | ||
*Spindle cells with wavy nuclei without pleomorphism - '''key feature'''. | *Spindle cells with wavy nuclei without pleomorphism - '''key feature'''. | ||
* | *Intermixed with wire-like collagen. | ||
**Often no pattern is apparent. | **Often no pattern is apparent. | ||
**Often described as "shredded carrots".<ref name=pmid24216989>{{Cite journal | last1 = Bernthal | first1 = NM. | last2 = Jones | first2 = KB. | last3 = Monument | first3 = MJ. | last4 = Liu | first4 = T. | last5 = Viskochil | first5 = D. | last6 = Randall | first6 = RL. | title = Lost in translation: ambiguity in nerve sheath tumor nomenclature and its resultant treatment effect. | journal = Cancers (Basel) | volume = 5 | issue = 2 | pages = 519-28 | month = | year = 2013 | doi = 10.3390/cancers5020519 | PMID = 24216989 }}</ref> | |||
*Moderate increase of cellularity vis-a-vis normal dermis. | *Moderate increase of cellularity vis-a-vis normal dermis. | ||
*May be poorly or well-circumscribed. | *May be poorly or well-circumscribed. | ||
| Line 62: | Line 68: | ||
DDx: | DDx: | ||
*[[Plexiform neurofibroma]]. | *[[Plexiform neurofibroma]]. | ||
*[[Schwannoma]]. | *[[Schwannoma]] - calretinin +ve, CD34 mostly -ve.<ref>URL: [http://www.ihcworld.com/_newsletter/2004/2004-10_NF_vs_schwannoma_v1.pdf http://www.ihcworld.com/_newsletter/2004/2004-10_NF_vs_schwannoma_v1.pdf]. Accessed on: 25 November 2013.</ref> | ||
*[[Dermatofibrosarcoma protuberans]] (DFSP) - S-100 -ve. | *[[Dermatofibrosarcoma protuberans]] (DFSP) - S-100 -ve. | ||
*[[Ganglioneuroma]]. | *[[Ganglioneuroma]]. | ||
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Image:Neurofibroma_(2).jpg | Neurofibroma - intermed. mag. (WC) | Image:Neurofibroma_(2).jpg | Neurofibroma - intermed. mag. (WC) | ||
Image:Neurofibroma_(3).jpg | Neurofibroma - high mag. (WC) | Image:Neurofibroma_(3).jpg | Neurofibroma - high mag. (WC) | ||
File:Neurofibroma_-3.jpg | Neurofibroma (WC/Netha Hussain) | |||
File:Plexiform_neurofibroma.jpg | Neurofibroma (WC/Netha Hussain) | |||
File:Plexiform_neurofibroma_-1.jpg | Plexiform Neurofibroma (WC/Netha Hussain) | |||
</gallery> | </gallery> | ||
www: | www: | ||
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====Alternate==== | ====Alternate==== | ||
The sections show skin with an unencapsulated dermal spindle cell lesion with | The sections show skin with an unencapsulated dermal spindle cell lesion with wavy nuclei that have a likeness to shredded carrots. Occasional mast cells are present within the lesion. There is no nuclear atypia. Mitotic activity is not appreciated. No melanocytic nests are identified. | ||
==See also== | ==See also== | ||
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[[Category:Peripheral nerve sheath tumours]] | [[Category:Peripheral nerve sheath tumours]] | ||
[[Category:Neuropathology tumours]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||