Neurofibroma pathophysiology
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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
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.[1] 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.[2][3]
Pathogenesis
- Neurofibromas arise from nonmyelinating-type Schwann cells.[4]
- Plexiform neurofibromas can grow from nerves in the skin or from more internal nerve bundles, and can be very large.[5]
- About 10% of plexiform neurofibromas undergo transformation into a malignant peripheral nerve sheath tumor (MPNST).[6]
Gross Pathology
Localised neurofibroma/Diffuse neurofibroma[7]
- Superficial mass
- Not encapsulated
Plexiform neurofibroma[1]
- "Bag of worms" appearance
![Neurofibroma[8]](/index.php/File:Neurofibroma1.jpg)
![Neurofibroma[9]](/index.php/File:Neurofibroma2.jpg)
Microscopic Pathology
- Spindle cells with wavy nuclei without pleomorphism
- Intermixed with wire-like collagen[2]
- Moderate increase of cellularity vis-a-vis normal dermis
- Mast cells[3]
-
![Neurofibroma[10]](/images/7/7b/Neurofibroma3.jpg)
Neurofibroma[10]
![Neurofibroma[10]](/index.php/File:Neurofibroma3.jpg)
Genetics
- NF1 is involved in the pathogenesis of plexiform neurofibroma.[7]
References
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 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) - ↑ 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.
- ↑ 7.0 7.1 Neurofibroma. Dr Bruno Di Muzio and Dr Maxime St-Amant et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/neurofibroma
- ↑ 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
- ↑ Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2