Juvenile Xanthogranuloma This is a typical appearance of a juvenile xanthogranuloma removed from the face of a small boy. The lesion is uniform and cellular and proliferates in the dermis with no grenz zone.
TERMINOLOGY
Abbreviations
• Juvenile xanthogranuloma (JXG)
Synonyms
• Nevoxanthoendothelioma
Definitions
• Stable or regressing histiocytic lesion that usually occurs in childhood
Form of non-Langerhans histiocytosis
CLINICAL ISSUES
Epidemiology
• Incidence
Rare
• Age
Majority in individuals under 3 years
– Visceral examples almost exclusively in infants and children
13-30% in older children and adults
• Sex
Slight male predominance
Presentation
• Solitary cutaneous lesion in majority of cases
Head and neck > trunk > extremities
• Up to 10% of patients with multiple cutaneous lesions
• Up to 5% of patients with visceral-systemic disease
Treatment
• Simple excision
• Chemotherapy administered to rare patients with systemic disease
Prognosis
• Usually excellent
Most lesions regress or stabilize (including large visceral ones)
Rare deaths associated with multiorgan disease
MICROSCOPIC
Histologic Features
• Mononuclear cells typically predominate
• Multinucleated cells ± Touton features
• Spindle cells (typically minor component)
Variable finely vacuolated cytoplasm
Often lightly eosinophilic
• Variable lipid and foamy histiocytes
Minimal lipid in early lesions
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