Difference between revisions of "Juvenile xanthogranuloma"

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#redirect [[Pediatric pathology#Juvenile xanthogranuloma]]
{{ 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>


==General==
*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>
*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>
==Microscopic==
Features:<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>
*Dermal histiocytes:
**Abundant cytoplasm - may not be xanthomatous/foam cells.
*+/-Touton [[giant cell]] - '''key feature'''.
**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:
*[[Langerhans cell histiocytosis]].
*[[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.
*[[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===
<gallery>
Image:Juvenile_xanthogranuloma_-_intermed_mag.jpg | JXG - intermed. mag. (WC)
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>
===IHC===
Features:<ref name=emed_jxg/>
*Langerhans cell markers: CD1a, CD207 -- both should be negative.
**If Touton giant cells are absent -- this is essential.
*Histiocyte markers: CD68, CD163 -- both should be positive.
*Vimentin +ve.
Other markers:<ref name=pmid11285404>{{Cite journal  | last1 = Kraus | first1 = MD. | last2 = Haley | first2 = JC. | last3 = Ruiz | first3 = R. | last4 = Essary | first4 = L. | last5 = Moran | first5 = CA. | last6 = Fletcher | first6 = CD. | title = Juvenile xanthogranuloma: an immunophenotypic study with a reappraisal of histogenesis. | journal = Am J Dermatopathol | volume = 23 | issue = 2 | pages = 104-11 | month = Apr | year = 2001 | doi =  | PMID = 11285404 }}</ref>
*CD4 +ve (21 of 27 cases).
*CD45 +ve (25 of 27 cases).
*Factor XIIIa +ve (25 of 27 cases).
Negatives:<ref name=pmid9790615>{{cite journal |author=Thomas DB, Sidler AK, Huston BM |title=Radiological case of the month. Juvenile xanthogranuloma |journal=Arch Pediatr Adolesc Med |volume=152 |issue=10 |pages=1029–30 |year=1998 |month=October |pmid=9790615 |doi= |url=http://archpedi.ama-assn.org/cgi/content/full/152/10/1029}}</ref>
*Muscle markers: actin, desmin.
*Others: S100, factor VIII, cytokeratins.
==Sign out==
<pre>
SKIN LESION, CHIN, BIOPSY:
- JUVENILE XANTHOGRANULOMA.
</pre>
==See also==
*[[Dermatopathology]].
*[[Pediatric pathology]].
==References==
{{Reflist|2}}
[[Category:Dermatopathology]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Latest revision as of 20:37, 17 June 2016

Juvenile xanthogranuloma
Diagnosis in short

Juvenile xanthogranuloma. H&E stain.
LM dermal histiocytes with abundant cytoplasm, +/-Touton giant cells (large multi-nucleated cells where nuclei are distributed at the cell periphery)
LM DDx Langerhans cell histiocytosis, Spitz nevus (reported to have Touton cells), Dermatofibroma - aneurysmal type
IHC CD68 +ve, CD1a -ve, CD207 -ve
Site skin
Clinical history usually in children and infants, sometimes in adults
Prognosis benign

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.[1]

General

  • Usually in children and infants, sometimes in adults.[1]
    • In adults, it may be referred to as cutaneous xanthogranuloma,[2] adult onset xanthogranuloma, and adult xanthogranuloma.[3]
  • Most common form of non–Langerhans cell histiocytosis.[4]
  • Can rarely be found in the brain.[5]

Microscopic

Features:[4]

  • Dermal histiocytes:
    • Abundant cytoplasm - may not be xanthomatous/foam cells.
  • +/-Touton giant cell - key feature.
    • 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:

Images

  • JXG - intermed. mag. (WC) 

  • JXG - intermed. mag. (WC)

  • Touton giant cells in a JXG - very high mag. (WC)

IHC

Features:[4]

  • Langerhans cell markers: CD1a, CD207 -- both should be negative.
    • If Touton giant cells are absent -- this is essential.
  • Histiocyte markers: CD68, CD163 -- both should be positive.
  • Vimentin +ve.

Other markers:[8]

  • CD4 +ve (21 of 27 cases).
  • CD45 +ve (25 of 27 cases).
  • Factor XIIIa +ve (25 of 27 cases).

Negatives:[9]

  • Muscle markers: actin, desmin.
  • Others: S100, factor VIII, cytokeratins.

Sign out

SKIN LESION, CHIN, BIOPSY:
- JUVENILE XANTHOGRANULOMA.

See also

References

  1. ↑ 1.0 1.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 622. ISBN 978-0443066542.
  2. ↑ Chiang, E.; Lissner, G.; Bryar, PJ.. "Unusual presentation of xanthogranuloma on the eyelid of an adult.". Ophthal Plast Reconstr Surg 30 (6): e155-6. doi:10.1097/01.iop.0000440703.67932.37. PMID 24801255.
  3. ↑ Tan, LC.; Tan, KB.; Aw, CW. (Feb 2014). "Unusual presentation of adult xanthogranuloma: a case report.". Singapore Med J 55 (2): e25-7. doi:10.11622/smedj.2013207. PMID 24154556.
  4. ↑ 4.0 4.1 4.2 URL: http://emedicine.medscape.com/article/1111629-diagnosis. Accessed on: 3 February 2011.
  5. ↑ URL: http://path.upmc.edu/cases/case245/dx.html. Accessed on: 13 January 2012.
  6. ↑ Guitart, J.; Gerami, P. (Jul 2008). "Touton-like giant cells in a Spitz's nevus.". J Cutan Pathol 35 (7): 694-5. doi:10.1111/j.1600-0560.2007.00877.x. PMID 18312437.
  7. ↑ Haroche, J.; Charlotte, F.; Arnaud, L.; von Deimling, A.; Hélias-Rodzewicz, Z.; Hervier, B.; Cohen-Aubart, F.; Launay, D. et al. (Sep 2012). "High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses.". Blood 120 (13): 2700-3. doi:10.1182/blood-2012-05-430140. PMID 22879539.
  8. ↑ Kraus, MD.; Haley, JC.; Ruiz, R.; Essary, L.; Moran, CA.; Fletcher, CD. (Apr 2001). "Juvenile xanthogranuloma: an immunophenotypic study with a reappraisal of histogenesis.". Am J Dermatopathol 23 (2): 104-11. PMID 11285404.
  9. ↑ Thomas DB, Sidler AK, Huston BM (October 1998). "Radiological case of the month. Juvenile xanthogranuloma". Arch Pediatr Adolesc Med 152 (10): 1029–30. PMID 9790615. http://archpedi.ama-assn.org/cgi/content/full/152/10/1029.
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