Neurofibroma overview: Difference between revisions
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==Overview== | ==Overview== | ||
A '''neurofibroma''' is a benign [[nerve sheath tumor]] in the [[peripheral nervous system]]. | A '''neurofibroma''' is a benign [[nerve sheath tumor]] in the [[peripheral nervous system]]. | ||
==Classification== | |||
Neurofibroma may be classified into 3 subtypes: localised neurofibroma, diffuse neurofibroma, and [[plexiform neurofibroma]].<ref name=radio> Neurofibroma. Dr Bruno Di Muzio and Dr Maxime St-Amant et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/neurofibroma </ref> | |||
==Pathophysiology== | |||
On gross pathology, a nonencapsulated superficial [[mass]] is the characteristic finding of localised or diffuse neurofibroma; whereas the "bag of worms" appearance is the characteristic finding of [[plexiform neurofibroma]].<ref name="pmid15486243">{{cite journal |vauthors=Wilkinson LM, Manson D, Smith CR |title=Best cases from the AFIP: plexiform neurofibroma of the bladder |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=24 Suppl 1 |issue= |pages=S237–42 |year=2004 |pmid=15486243 |doi=10.1148/rg.24si035170 |url=http://pubs.rsna.org/doi/10.1148/rg.24si035170?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed |issn= |accessdate=2015-11-13}}</ref> On microscopic histopathological analysis, [[spindle cell]]s with wavy [[nuclei]] without pleomorphism, wire-like [[collagen]], moderate increase of cellularity vis-a-vis normal [[dermis]], and [[mast cells]] are characteristic findings of neurofibroma.<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><ref name=pmid20233971>{{Cite journal | last1 = Staser | first1 = K. | last2 = Yang | first2 = FC. | last3 = Clapp | first3 = DW. | title = Mast cells and the neurofibroma microenvironment. | journal = Blood | volume = 116 | issue = 2 | pages = 157-64 | month = Jul | year = 2010 | doi = 10.1182/blood-2009-09-242875 | PMID = 20233971 }}</ref> | |||
==Causes== | |||
[[Plexiform neurofibroma]] may be caused by the bi-allelic inactivation of the [[neurofibromatosis type I]] tumor suppressor gene.<ref name=radio> Neurofibroma. Dr Bruno Di Muzio and Dr Maxime St-Amant et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/neurofibroma </ref><ref name="pmid7550323">{{cite journal |vauthors=Colman SD, Williams CA, Wallace MR |title=Benign neurofibromas in type 1 neurofibromatosis (NF1) show somatic deletions of the NF1 gene |journal=[[Nature Genetics]] |volume=11 |issue=1 |pages=90–2 |year=1995 |pmid=7550323 |doi=10.1038/ng0995-90 |url=http://dx.doi.org/10.1038/ng0995-90 |issn= |accessdate=2015-11-16}}</ref> | |||
==References== | ==References== |
Revision as of 20:40, 16 November 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
A neurofibroma is a benign nerve sheath tumor in the peripheral nervous system.
Classification
Neurofibroma may be classified into 3 subtypes: localised neurofibroma, diffuse neurofibroma, and plexiform neurofibroma.[1]
Pathophysiology
On gross pathology, a nonencapsulated superficial mass is the characteristic finding of localised or diffuse neurofibroma; whereas the "bag of worms" appearance is the characteristic finding of plexiform neurofibroma.[2] On microscopic histopathological analysis, spindle cells with wavy nuclei without pleomorphism, wire-like collagen, moderate increase of cellularity vis-a-vis normal dermis, and mast cells are characteristic findings of neurofibroma.[3][4]
Causes
Plexiform neurofibroma may be caused by the bi-allelic inactivation of the neurofibromatosis type I tumor suppressor gene.[1][5]
References
- ↑ 1.0 1.1 Neurofibroma. Dr Bruno Di Muzio and Dr Maxime St-Amant et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/neurofibroma
- ↑ Wilkinson LM, Manson D, Smith CR (2004). "Best cases from the AFIP: plexiform neurofibroma of the bladder". Radiographics : a Review Publication of the Radiological Society of North America, Inc. 24 Suppl 1: S237–42. doi:10.1148/rg.24si035170. PMID 15486243. Retrieved 2015-11-13.
- ↑ Bernthal, NM.; Jones, KB.; Monument, MJ.; Liu, T.; Viskochil, D.; Randall, RL. (2013). "Lost in translation: ambiguity in nerve sheath tumor nomenclature and its resultant treatment effect". Cancers (Basel). 5 (2): 519–28. doi:10.3390/cancers5020519. PMID 24216989.
- ↑ Staser, K.; Yang, FC.; Clapp, DW. (2010). "Mast cells and the neurofibroma microenvironment". Blood. 116 (2): 157–64. doi:10.1182/blood-2009-09-242875. PMID 20233971. Unknown parameter
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ignored (help) - ↑ Colman SD, Williams CA, Wallace MR (1995). "Benign neurofibromas in type 1 neurofibromatosis (NF1) show somatic deletions of the NF1 gene". Nature Genetics. 11 (1): 90–2. doi:10.1038/ng0995-90. PMID 7550323. Retrieved 2015-11-16.