Juvenile and Adult-Type Xanthogranuloma


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.



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Juvenile Xanthogranuloma at High Magnification
A cellular example at high magnification shows numerous mononucleated histiocytes associated with an inflammatory background composed mostly of mononuclear cells.

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Juvenile Xanthogranuloma: Touton Cells
Many Touton giant cells image can be seen in this juvenile xanthogranuloma. The background cells are spindled to ovoid with eosinophilic cytoplasm, which shows only minimal lipid in this case.

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CD68 Staining in Juvenile Xanthogranuloma
Essentially all examples of juvenile xanthogranuloma are reactive with CD68 (shown) and factor XIIIa by immunohistochemistry, but are negative for S100 and CD1a.


TERMINOLOGY


Abbreviations




• Juvenile xanthogranuloma (JXG)


Synonyms




• Nevoxanthoendothelioma


Definitions




• Stable or regressing histiocytic lesion that usually occurs in childhood
image Form of non-Langerhans histiocytosis


CLINICAL ISSUES


Epidemiology




• Incidence
image Rare

• Age
image Majority in individuals under 3 years
– Visceral examples almost exclusively in infants and children

image 13-30% in older children and adults

• Sex
image Slight male predominance


Presentation




• Solitary cutaneous lesion in majority of cases
image 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
image Most lesions regress or stabilize (including large visceral ones)

image Rare deaths associated with multiorgan disease


MICROSCOPIC


Histologic Features




• Mononuclear cells typically predominate
• Multinucleated cells ± Touton features

• Spindle cells (typically minor component)

image Variable finely vacuolated cytoplasm

image Often lightly eosinophilic

• Variable lipid and foamy histiocytes
image Minimal lipid in early lesions

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Juvenile and Adult-Type Xanthogranuloma

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