Juvenile Xanthogranuloma
Christine J. Ko, MD
Key Facts
Microscopic Pathology
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Dermal nodule of mixed inflammation
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Predominantly histiocytes
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Varying morphologies: Vacuolated, xanthomatized, scalloped, oncocytic, spindle-shaped
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Admixed lymphocytes, eosinophils, occasional neutrophils and plasma cells, and giant cells
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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
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Juvenile xanthogranuloma (JXG)
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Adult xanthogranuloma
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Spindle cell xanthogranuloma
Definitions
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Generally solitary tumor composed of histiocytes
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Most common non-Langerhans cell histiocytosis
ETIOLOGY/PATHOGENESIS
Unknown
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Controversy as to cell of origin
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Macrophage
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Dermal dendritic cell
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Plasmacytoid dendritic cell/monocyte
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CLINICAL ISSUES
Epidemiology
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Age
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More common in infants and children
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May present in adulthood
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Gender
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Male predominance, especially if multiple lesions
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Site
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Most commonly head/neck, may also involve trunk or other unusual sites
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Can affect orbit or internal organs
Presentation
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Solitary or multiple papules or nodules
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Early lesions more pink, later lesions more yellow
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