Difference between revisions of "Juvenile xanthogranuloma"

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#redirect [[Pediatric pathology#Juvenile xanthogranuloma]]
'''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, may be in adults.<ref name=Ref_Derm622>{{Ref Derm|622}}</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.
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.
Notes:
*Must prove they are non-Langerhans cell histiocytes, esp. if no Touton giant cells.
===Images===
<gallery>
Image:Juvenile_xanthogranuloma_-_intermed_mag.jpg | Juvenile xanthogranuloma - intermed. mag. (WC)
Image:Juvenile_xanthogranuloma_-_very_high_mag.jpg | Touton giant cells in a juvenile xanthogranuloma - 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]]

Revision as of 16:06, 6 June 2013

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, may be in adults.[1]
  • Most common form of non–Langerhans cell histiocytosis.[2]
  • Can rarely be found in the brain.[3]

Microscopic

Features:[2]

  • 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.

DDx:

Notes:

  • Must prove they are non-Langerhans cell histiocytes, esp. if no Touton giant cells.

Images

  • Juvenile xanthogranuloma - intermed. mag. (WC)

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

IHC

Features:[2]

  • 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:[5]

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

Negatives:[6]

  • 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. ↑ 2.0 2.1 2.2 URL: http://emedicine.medscape.com/article/1111629-diagnosis. Accessed on: 3 February 2011.
  3. ↑ URL: http://path.upmc.edu/cases/case245/dx.html. Accessed on: 13 January 2012.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ 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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