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Skin - Nonmelanocytic tumors

Other tumors of skin

Xanthogranuloma

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 18 September 2012, last major update September 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

Juvenile xanthogranuloma

General
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● Also called nevoxanthoendothelioma
● Proliferative disorder of dendrocytes
● Uncommon (0.5% in one tumor registry), less common than Langerhans cell histiocytosis (3% incidence), the other principal histiocytic disorders of childhood (Am J Surg Pathol 2003;27:579, Am J Surg Pathol 2005;29:21)

Clinical features
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● Usually infants (median age 5 months) with a congenital mark, although 10-30% occur in adults
● Male/female = 1.4:1
● May spontaneously regress
● Skin, often face or trunk, but may affect any site
● Less commonly in subcutis, skeletal muscle, eye, peripheral nerve, testis
● 20% have multiple lesions (>90% are males, usually age 6 years or less)
● May be associated with glaucoma and amblyopia due to involvement of iris and ciliary body
● Also associated with neurofibromatosis type I, Niemann-Pick disease, urticaria pigmentosa, CMV infection
● Neonates may develop systemic disease and death due to hepatic failure (giant cell hepatitis and tumor in liver and viscera)

Treatment
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● Excision
● Some lesions may involute spontaneously
● Relapse rate of 7%
● Systemic cases need multiagent chemotherapy

Gross description
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● Yellow-red, papulonodular lesions
● Solitary or multicentric, 1 mm to 2 cm

Micro description
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● Initially dense lymphohistiocytic proliferation of dermis with no/rare giant cells
● Then foamy and Touton giant cells (giant cells are often lacking in extracutaneous lesions) or other types of giant cells
● Also short fascicles of spindle cells
● Late - short fascicles of fibrohistiocytic cells and fibrosis
● Usually poorly circumscribed, thin epidermis with elongated rete ridges, preservation of adnexae, prominent vasculature; may have mild nuclear atypia; variable storiform pattern, lymphocytes and eosinophils
● No/scattered mitotic figures

Positive stains
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● CD68, alpha-1-antichymotrypsin, lysozyme, vimentin, Factor XIIIa

Negative stains
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● S100, CD1a

Electron microscopy description
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● May have cytoplasmic lipid; no Birbeck granules

Differential diagnosis
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● Atheroma
● Dermatofibroma: dense collagenous stroma, storiform growth pattern, pseudoepitheliomatous hyperplasia
● Hyperlipidemia associated xanthomas: more uniform foamy histiocytes
● Langerhans cell histiocytosis: nuclear grooves, S100+, CD1a+, Birbeck granules by EM
● Lipoma
● Reticulohistiocytoma: random distribution of multinucleated histiocytes with eosinophilic or ground glass cytoplasm

Necrobiotic xanthogranulomas

General
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● Destructive lesions of dermis and subcutis, often involving face and trunk, and accompanied by monoclonal gammopathy or cryoglobulins

Case reports
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● 64 year old woman with multiple nodules (Dermatol Res Pract 2011;2011:927852)

Gross description
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● Yellowish papules/nodules

Micro description
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● Palisaded granulomas with necrobiosis

Micro images
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Various images

End of Skin - Nonmelanocytic tumors > Other tumors of skin > Xanthogranuloma

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