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juvenile xanthogranuloma

Friday 24 October 2003

Digital cases

- JRC:11021 : Juvenile xanthogranuloma.

Definition: Juvenile xanthogranuloma (JXG) is a rare disorder of the non-Langerhans cell histiocytosis (LCH) type with unknown etiology and pathogenesis. It is still unclear whether JXG is a reactive or a neoplastic process. Clonality has been assessed (17460468).

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.

Juvenile xanthogranuloma (JXG) and LCH have been grouped in the category of dendritic cell-related disorders and separated from macrophage-related proliferations like the Rosai-Dorfman disease.

Despite the supposed similarities between JXG and LCH in terms of their histogenesis, they differ in their morphology and immunophenotype, as well as in their clinical presentation. These differences might explain the poorer outcome of LCH compared with the excellent prognosis of JXG.

JXG is a clonal proliferation of histiocytic/dendritic cells and does not differ in this respect from LCH.

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%)
- systemic juvenile xanthogranuloma (17230073)
- macrophagic activation syndrome (MAS)

Localization

- cutis and subcutis (cutaneous juvenile xanthogranuloma)
- mouth, oral cavity (12324792)
- peripheral nerve (11257628)
- heart (11178674)
- eyes (6819503): choroid (12107521), iris (9514494, 17317408), orbit (2123087)
- central nervous system (3082946), brain (9485160), intracranial (17332947)
- liver (12717244)
- spleen
- lung
- oropharynx
- muscle
- bone marrow JXG (17378689)

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

Associations

- neurilemmomatosis (multiple neurilemmomas) (9810909)
- cafe-au-lait spots (CALS) (11454088, 15078345)
- prolonged severe pancytopenia (16047346)
- juvenile myelomonocytic leukemia (juvenile myeloid leukemia, juvenile chronic myelogenous leukaemia) (6422862, 14550978) or acute myelomonocytic leukemia (6430255) -* with neurofibromatosis type 1 (NF1) (15342987, 14550978)

Clonality

- Monoclonality has been assessed in one case (17460468).

Cytogenetics: no data

Molecular biology: no data

Differential diagnosis

- histiocytic proliferations (histiocytoses)

  • dendritic cell-related proliferations
    • Langerhans cell histiocytosis
  • polyclonal proliferations of macrophages
    • Rosai-Dorfmann disease

See also

- xanthogranulomas

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

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