Juvenile Xanthogranuloma (aka Nevoxanthoendothelioma)

General: An uncommon (~0.5%, which is less than Langerhans cell histiocytosis) tumor of dendrocytes and is classified as a histiocytic disorder of childhood. Usually congenital, but ~20% occur in adults, found most commonly as a skin lesion of the face or trunk — but may be found anywhere, including testis or peripheral nerve, and ~20% will have multiple lesions. Associated with neurofibromatosis type I, Neimann Pick disease, urticaria pigmentosa, and cytomegalovirus infection. Death may occur in neonates as a result of giant cell hepatitis and widespread tumorous lesions. Lesions may, however, resolve spontaneously; treatment is typically by simple excision, though systemic disease may be treated with chemotherapy.

Gross: Yellow-red papulonodular lesions, 0.1-2.0cm.

Microscopic: Progressive changes: initially dense dermal lymphohistiocytic proliferation with perhaps rare giant cells; then foamy and Touton or other types of giant cells (often absent in extracutaneous lesions), and short fascicles of spindle cells; late, short fascicles of fibrohistiocytic cells and fibrosis. There may be a variable storiform pattern. Often poorly circumscribed, with a thin epidermis and elongated rete ridges. Adnexae are preserved. Lymphocytes, eosinophils, and prominent vasculature may be evident. Scattered mitotic figures are allowed, and may show mild nuclear atypia.

Differential Diagnosis: Atheroma, dermatofibroma, hyperlipidemia associated xanthomas, Langerhans cell histiocytosis, reticulohistiocytoma.

Stains:

• Positive: CD68+, alpha-1-antichymotrypsin+, lysozyme+, vimentin+, Factor XIIIa+
• Negative: S100-, CD1a-
• Suggested, focused panel:

(PathologyOutlines.com*)

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