Table of Contents
Definition / general | Clinical features | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosisCite this page: Hale C. Juvenile xanthogranuloma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticjuvenilexantho.html. 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)
Treatment
- 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
Positive 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