Juvenile Xanthogranuloma
Christine J. Ko, MD
Key Facts
Microscopic Pathology
Dermal nodule of mixed inflammation
Predominantly histiocytes
Varying morphologies: Vacuolated, xanthomatized, scalloped, oncocytic, spindle-shaped
Admixed lymphocytes, eosinophils, occasional neutrophils and plasma cells, and giant cells
Touton giant cells are characteristic
 This is a juvenile xanthogranuloma on the forehead of a child. The lesion has a slightly pink-yellow tinge. (Courtesy R. Antaya, MD.) |
 In this juvenile xanthogranuloma, there are numerous foamy (xanthomatized) histiocytes as well as prominent Touton giant cells  . There is admixed lymphocytic inflammation. |
TERMINOLOGY
Synonyms
Juvenile xanthogranuloma (JXG)
Adult xanthogranuloma
Spindle cell xanthogranuloma
Definitions
Generally solitary tumor composed of histiocytes
Most common non-Langerhans cell histiocytosis
ETIOLOGY/PATHOGENESIS
Unknown
Controversy as to cell of origin
Macrophage
Dermal dendritic cell
Plasmacytoid dendritic cell/monocyte
CLINICAL ISSUES
Epidemiology
Age
More common in infants and children
May present in adulthood
Gender
Male predominance, especially if multiple lesions
Site
Most commonly head/neck, may also involve trunk or other unusual sites
Can affect orbit or internal organs
Presentation
Solitary or multiple papules or nodules
Early lesions more pink, later lesions more yellow
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