Juvenile xanthogranuloma

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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

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.