JXG is a proliferative disorder of dendrocytes, possibly dermal dendrocytes. Juvenile xanthogranulomas is a histiocytic disorder, primarily but not exclusively seen throughout the first two decades of life and principally as a solitary cutaneous lesion.

Presentation (#12717244#)

 solitary cutaneous lesion (67%)
 solitary subcutaneous or deep soft tissue mass (16%)
 multiple cutaneous lesions (7%)
 solitary extracutaneous, nonsoft tissue lesion (5%)
 multiple cutaneous and visceral-systemic lesions (5%)

Localization

 cutis and subcutis
 mouth, oral cavity (#12324792#)
 peripheral nerve (#11257628#)
 heart (#11178674#)
 eyes (#6819503#): choroid (#12107521#), iris (#9514494#), orbit (#2123087#)
 central nervous system (#3082946#), brain (#9485160#)
 liver (#12717244#)
 spleen
 lung
 oropharynx
 muscle

Variants

 solitary juvenile xanthogranuloma
 multiple juvenile xanthogranuloma
 systemic juvenile xanthogranuloma (#8765620#)
 nonlipidized juvenile xanthogranuloma (#9144693#)
 mitotically active juvenile xanthogranuloma (#12296763#)

Juvenile xanthogranuloma group histiocytic disorders (dermal dendrocyte)

 progressive nodular histiocytosis
 xanthoma disseminatum
 benign cephalic histiocytosis
 spindle cell xanthogranuloma
 generalized eruptive histiocytosis

Microscopical synopsis

 Touton giant cell (consistent in cutaneous lesions; absent or present in reduced numbers in extracutaneous lesions)
 spindle cells intermingled among the mononuclear cells or forming short fascicles

Immunohistochemistry

 vimentin+
 CD68+
 factor XIIIa+
 fascin+
 alpha-1-antitrypsine
 S-100-
 CD1a-

Predisposition

 neurofibromatoses

• neurofibromatosis type 1 (NF1)
• neurofibromatosis type 2 (NF2) (#9810909#)

Associations

 neurilemmomatosis (multiple neurilemmomas) (#9810909#)

Clonality: no data Cytogentics: no data Molecular biology: no data

References

 Janssen D, Harms D. Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. Am J Surg Pathol. 2005 Jan;29(1):21-8. PMID: #15613853#

 Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol. 2003 May;27(5):579-93. PMID: #12717244#

 Hu WK, Gilliam AC, Wiersma SR, Dahms BB. Fatal congenital systemic juvenile xanthogranuloma with liver failure. Pediatr Dev Pathol. 2004 Jan-Feb;7(1):71-6. PMID: #15255037#