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Difference between revisions of "Juvenile xanthogranuloma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Juvenile_xanthogranuloma_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Juvenile xanthogranuloma. [[H&E stain]]. | |||
| Micro = dermal histiocytes with abundant cytoplasm, +/-Touton [[giant cell]]s (large multi-nucleated cells where nuclei are distributed at the cell periphery) | |||
| Subtypes = | |||
| LMDDx = [[Langerhans cell histiocytosis]], [[Spitz nevus]] (reported to have Touton cells), [[Dermatofibroma]] - aneurysmal type | |||
| Stains = | |||
| IHC = CD68 +ve, CD1a -ve, CD207 -ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[skin]] | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = usually in children and infants, sometimes in adults | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = benign | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Juvenile xanthogranuloma''', abbreviated '''JXG''', is a relatively common distinctive diagnosis in dermatopathology. It is also known as '''nevoxanthoendothelioma'''. In adults, it is called ''adult xanthogranuloma''.<ref name=Ref_Derm622>{{Ref Derm|622}}</ref> | '''Juvenile xanthogranuloma''', abbreviated '''JXG''', is a relatively common distinctive diagnosis in dermatopathology. It is also known as '''nevoxanthoendothelioma'''. In adults, it is called ''adult xanthogranuloma''.<ref name=Ref_Derm622>{{Ref Derm|622}}</ref> | ||
==General== | ==General== | ||
*Usually in children and infants, sometimes in adults.<ref name=Ref_Derm622>{{Ref Derm|622}}</ref> | *Usually in children and infants, sometimes in adults.<ref name=Ref_Derm622>{{Ref Derm|622}}</ref> | ||
**In adults, it may be referred to as ''cutaneous xanthogranuloma'',<ref name=pmid24801255>{{Cite journal | last1 = Chiang | first1 = E. | last2 = Lissner | first2 = G. | last3 = Bryar | first3 = PJ. | title = Unusual presentation of xanthogranuloma on the eyelid of an adult. | journal = Ophthal Plast Reconstr Surg | volume = 30 | issue = 6 | pages = e155-6 | month = | year = | doi = 10.1097/01.iop.0000440703.67932.37 | PMID = 24801255 }}</ref> ''adult onset xanthogranuloma'', and ''adult xanthogranuloma''.<ref name=pmid24154556>{{Cite journal | last1 = Tan | first1 = LC. | last2 = Tan | first2 = KB. | last3 = Aw | first3 = CW. | title = Unusual presentation of adult xanthogranuloma: a case report. | journal = Singapore Med J | volume = 55 | issue = 2 | pages = e25-7 | month = Feb | year = 2014 | doi = 10.11622/smedj.2013207 | PMID = 24154556 }}</ref> | |||
*Most common form of non–Langerhans cell histiocytosis.<ref name=emed_jxg>URL: [http://emedicine.medscape.com/article/1111629-diagnosis http://emedicine.medscape.com/article/1111629-diagnosis]. Accessed on: 3 February 2011.</ref> | *Most common form of non–Langerhans cell histiocytosis.<ref name=emed_jxg>URL: [http://emedicine.medscape.com/article/1111629-diagnosis http://emedicine.medscape.com/article/1111629-diagnosis]. Accessed on: 3 February 2011.</ref> | ||
*Can rarely be found in the brain.<ref>URL: [http://path.upmc.edu/cases/case245/dx.html http://path.upmc.edu/cases/case245/dx.html]. Accessed on: 13 January 2012.</ref> | *Can rarely be found in the brain.<ref>URL: [http://path.upmc.edu/cases/case245/dx.html http://path.upmc.edu/cases/case245/dx.html]. Accessed on: 13 January 2012.</ref> | ||
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*+/-Touton [[giant cell]] - '''key feature'''. | *+/-Touton [[giant cell]] - '''key feature'''. | ||
**Large multi-nucleated cells where nuclei are distributed at the cell periphery. | **Large multi-nucleated cells where nuclei are distributed at the cell periphery. | ||
Notes: | |||
*Must prove they are non-Langerhans cell histiocytes, esp. if no Touton giant cells. | |||
DDx: | DDx: | ||
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*[[Spitz nevus]] - uncommon; reported to have Touton cells.<ref name=pmid18312437>{{Cite journal | last1 = Guitart | first1 = J. | last2 = Gerami | first2 = P. | title = Touton-like giant cells in a Spitz's nevus. | journal = J Cutan Pathol | volume = 35 | issue = 7 | pages = 694-5 | month = Jul | year = 2008 | doi = 10.1111/j.1600-0560.2007.00877.x | PMID = 18312437 }}</ref> | *[[Spitz nevus]] - uncommon; reported to have Touton cells.<ref name=pmid18312437>{{Cite journal | last1 = Guitart | first1 = J. | last2 = Gerami | first2 = P. | title = Touton-like giant cells in a Spitz's nevus. | journal = J Cutan Pathol | volume = 35 | issue = 7 | pages = 694-5 | month = Jul | year = 2008 | doi = 10.1111/j.1600-0560.2007.00877.x | PMID = 18312437 }}</ref> | ||
*[[Dermatofibroma]], aneurysmal - has Touton [[giant cell]]s and hemosiderin deposition. | *[[Dermatofibroma]], aneurysmal - has Touton [[giant cell]]s and hemosiderin deposition. | ||
*[[Tuberculoma]]. | |||
*[[Erdheim-Chester disease]] - usu. [[BRAF V600E mutated]].<ref name=pmid22879539>{{Cite journal | last1 = Haroche | first1 = J. | last2 = Charlotte | first2 = F. | last3 = Arnaud | first3 = L. | last4 = von Deimling | first4 = A. | last5 = Hélias-Rodzewicz | first5 = Z. | last6 = Hervier | first6 = B. | last7 = Cohen-Aubart | first7 = F. | last8 = Launay | first8 = D. | last9 = Lesot | first9 = A. | title = High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. | journal = Blood | volume = 120 | issue = 13 | pages = 2700-3 | month = Sep | year = 2012 | doi = 10.1182/blood-2012-05-430140 | PMID = 22879539 }}</ref> | |||
* | *[[Rosai-Dorfman disease]] - Emperipolesis. | ||
*[[Necrobiotic xanthogranuloma]] - also may have Touton giant cells. | |||
===Images=== | ===Images=== | ||
<gallery> | <gallery> | ||
Image:Juvenile_xanthogranuloma_-_intermed_mag.jpg | Juvenile xanthogranuloma - intermed. mag. (WC) | Image:Juvenile_xanthogranuloma_-_intermed_mag.jpg | JXG - intermed. mag. (WC) | ||
Image:Juvenile_xanthogranuloma_-_very_high_mag.jpg | Touton giant cells in a | Image:Juvenile xanthogranuloma - high mag.jpg | | JXG - intermed. mag. (WC) | ||
Image:Juvenile_xanthogranuloma_-_very_high_mag.jpg | Touton giant cells in a JXG - very high mag. (WC) | |||
</gallery> | </gallery> | ||
===IHC=== | ===IHC=== | ||
Features:<ref name=emed_jxg/> | Features:<ref name=emed_jxg/> | ||