Skin nonmelanocytic tumor
Other tumors of skin
Juvenile xanthogranuloma

Topic Completed: 1 September 2012

Revised: 5 April 2019

Copyright: 2001-2016,, Inc.

PubMed search: juvenile xanthogranuloma [title] skin

Christopher S. Hale, M.D.
Page views in 2019: 7,708
Page views in 2020 to date: 1,524
Cite this page: Hale C. Juvenile xanthogranuloma. website. Accessed February 27th, 2020.
Definition / general
  • 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
  • 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)
  • Excision
  • Some lesions may involute spontaneously
  • Relapse rate of 7%
  • Systemic cases need multiagent chemotherapy
Gross description
  • Yellowish red, papulonodular lesions
  • Solitary or multicentric, 1 mm to 2 cm
Microscopic (histologic) description
  • 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
Negative stains
Electron microscopy description
  • May have cytoplasmic lipid; no Birbeck granules
Differential diagnosis
  • 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
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