Neurofibroma pathophysiology: Difference between revisions
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==Microscopic Pathology== | ==Microscopic Pathology== | ||
Features: | |||
*Spindle cells with wavy nuclei without pleomorphism - '''key feature'''. | |||
*Intermixed with wire-like collagen. | |||
**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. | |||
*May be poorly or well-circumscribed. | |||
*+/-Plexiform growth pattern - "bag of worms".<ref name=pmid17893219/> | |||
**Multiple well-circumscribed nests. | |||
*Mast cells<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> - one has to look for them at high power. | |||
**Very useful for confirming the low power suspicion. | |||
==Genetics== | ==Genetics== | ||
Revision as of 21:06, 13 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
Pathogenesis
- Neurofibromas arise from nonmyelinating-type Schwann cells.[1]
- Plexiform neurofibromas can grow from nerves in the skin or from more internal nerve bundles, and can be very large.[2]
- About 10% of plexiform neurofibromas undergo transformation into a malignant peripheral nerve sheath tumor (MPNST).[3]
Gross Pathology
Localised neurofibroma/Diffuse neurofibroma[4]
- Superficial mass
- Not encapsulated
Plexiform neurofibroma[5]
- "Bag of worms" appearance
![Neurofibroma[6]](/index.php/File:Neurofibroma1.jpg)
![Neurofibroma[7]](/index.php/File:Neurofibroma2.jpg)
Microscopic Pathology
Features:
- Spindle cells with wavy nuclei without pleomorphism - key feature.
- Intermixed with wire-like collagen.
- Often no pattern is apparent.
- Often described as "shredded carrots".[8]
- Moderate increase of cellularity vis-a-vis normal dermis.
- May be poorly or well-circumscribed.
- +/-Plexiform growth pattern - "bag of worms".[9]
- Multiple well-circumscribed nests.
- Mast cells[10] - one has to look for them at high power.
- Very useful for confirming the low power suspicion.
Genetics
- NF1 is involved in the pathogenesis of plexiform neurofibroma.[11]
References
- ↑ Muir D, Neubauer D, Lim IT, Yachnis AT, Wallace MR. (2003). "Tumorigenic properties of neurofibromin-deficient neurofibroma Schwann cells". American Journal of Pathology. 158 (2): 501–13. doi:10.1016/S0002-9440(10)63992-2. PMC 1850316. PMID 11159187.
- ↑ Neurofibroma. Wikipedia 2015. https://en.wikipedia.org/wiki/Neurofibroma#cite_note-Yamashiroya2002-3
- ↑ Mautner VF, Friedrich RE, von Deimling A, Hagel C, Korf B, Knöfel MT, Wenzel R, Fünsterer C. (2003). "Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma". American Journal of Pathology. 45 (9): 618–25. doi:10.1007/s00234-003-0964-6. PMID 12898075.
- ↑ 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.
- ↑ Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2
- ↑ Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2
- ↑ 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
|month=ignored (help) - ↑ Kluwe L, Friedrich R, Mautner VF (1999). <283::AID-GCC15>3.0.CO;2-K "Loss of NF1 allele in Schwann cells but not in fibroblasts derived from an NF1-associated neurofibroma". Genes, Chromosomes & Cancer. 24 (3): 283–5. PMID 10451710. Retrieved 2015-11-13.