Soft tissue
Fibrohistiocytic tumors
Juvenile xanthogranuloma variant of fibrous histiocytoma

Topic Completed: 1 August 2012

Revised: 22 March 2019

Copyright: 2002-2018,, Inc.

PubMed Search: Juvenile xanthogranuloma [title] soft tissue

Vijay Shankar, M.D.
Page views in 2018: 6,306
Page views in 2019 to date: 3,792
Cite this page: Shankar V. Juvenile xanthogranuloma variant of fibrous histiocytoma. website. Accessed August 21st, 2019.
Definition / general
  • Uncommon (< 0.5% of pediatric tumors in one study)
  • Usually infants (median age 5 months) with a congenital mark, although 10 - 30% occur in adults
  • Male / female = 1.4 : 1
Clinical features
  • Skin of face or trunk, less commonly in subcutis, skeletal muscle, eye, peripheral nerve or testis
  • Also liver, spleen, kidney; rarely intracranial (Am J Neuroradiol 2011;32:E132)
  • 20% of patients have multiple lesions, usually males
  • In neonates, rarely associated with giant cell hepatitis and tumor in liver and viscera, requiring chemotherapy
Case reports
  • Conservative excision (Am J Surg Pathol 2005;29:21)
  • May spontaneously regress leaving depressed area of skin with variable hyperpigmentation
  • Multisystemic disease requires Langerhans cell histiocytosis-type chemotherapy (Pediatr Blood Cancer 2008;51:130)
  • Can be fatal if massive hepatic or CNS involvement
Clinical images

Images hosted on PathOut servers:

Supraorbital, courtesy of Mark R. Wick, M.D.

Images hosted on other servers:

Smooth domed papule

Nodule over dorsum of tongue

Various images

Various images

Gross description
  • Up to 2 cm, yellow-red or brown, papulonodular
Gross images

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.

Microscopic (histologic) description
  • Dense dermal infiltrate of lymphocytes, histiocytes, Touton giant cells (usually), eosinophils and neutrophils, which may extend into subcutis
  • 0 - 2 mitotic figures per 10 HPF, rarely numerous
  • Late: epidermis thins out, rete ridges become elongated
  • Deep lesions: more cellular and monotonous with fewer Touton cells, can have more mitosis

  • Dermoscopy description: Orange-yellow setting sun appearance (Dermatology 2007;215:256)
Microscopic (histologic) images

Images hosted on PathOut servers:

20 year old woman (Case of the Week #5)

Cutaneous, courtesy of Mark R. Wick, M.D.

Images hosted on other servers:

Nasal papule

Touton type giant cells: ring of nuclei surround foamy cytoplasm, with cytoplasm visible around nuclei

Langhans type giant cells

Foreign body type giant cell (for comparison)

Foam cells, Touton giant cells and scattered lymphocytes

Cytology description
Negative stains
Electron microscopy description
  • No Birbeck granules; may have cytoplasmic lipid
Differential diagnosis
  • Langerhans cell histiocytosis: more common, tumor cells have coffee bean nuclei / nuclear grooves, no Touton giant cells, are S100+ and CD1a+ and negative for CD68, HAM56 and Factor XIIIa, have Birbeck granules by EM
  • Spindle cell xanthogranuloma: storiform array of cells and Touton type giant cells
  • Xanthoma: associated with hyperlipidemia, uniform collection of foam cells and variable Touton giant cells, but no other inflammatory cells
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