Skin nonmelanocytic tumor
Other tumors of skin
Juvenile xanthogranuloma

Topic Completed: 1 September 2012

Minor changes: 18 January 2021

Copyright: 2001-2021,, Inc.

PubMed search: juvenile xanthogranuloma [title] skin

Christopher S. Hale, M.D.
Page views in 2020: 11,320
Page views in 2021 to date: 843
Cite this page: Hale C. Juvenile xanthogranuloma. website. Accessed January 22nd, 2021.
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
  • M:F = 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
Microscopic (histologic) images

Contributed by Jijgee Munkhdelger, M.D., Ph.D. and Andrey Bychkov, M.D., Ph.D.

Low power

Inflammatory cells

Fibrohistiocytic proliferation with giants

Touton giant cell

Juvenile xanthogranuloma immunoprofile

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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