Difference between revisions of "Juvenile xanthogranuloma"

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| Image      = Juvenile_xanthogranuloma_-_very_high_mag.jpg
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| Caption    = Juvenile xanthogranuloma. [[H&E stain]].
| Caption    = Juvenile xanthogranuloma. [[H&E stain]].

Revision as of 00:07, 4 November 2014

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]
  • 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

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.