JXG
12/30/2009
Systemic Juvenile Xanthogranuloma; 1 yo F; hematuria & bladder mass on presentation; subsequent skin/soft tissue & bony lesions
Cedars Sinai Medical Center
3.875

GROSS
Well-circumscribed, yellow, semi-firm 1 cm mass on thumb

MICROSCOPIC
Mixed mononuclear cells within dermis, which consist primarily of histiocyte-type cells with abundant foamy cytoplasm, round to oval nuclei with irregular nuclear contours, fewer lymphocytes and neutrophils. These cells are interposed with numerous Touton-type multinucleated giant cells.

IMMUNOHISTOCHEMISTRY
Positive: CD68, FXIIIa
Negative: S-100, CD1a, myoD1, desmin, smooth muscle actin

DESCRIPTION
- Juvenile xanthogranuloma (JXG) is an uncommon non-Langerhans cell histiocytosis (negative for CD1a and S100).
- The incidence among pediatric tumors is 0.5% (Kiel Pediatric Tumor Registry). The male/female ration is 1.4:1. The mean age is 22.4 months; median age is 5 months; age range 0-244 months.
- Sites of involvement: mostly cutaneous (face or trunk), soft tissue, CNS, eye, visceral organs
- Focality: 81% of cutaneous lesions are solitary lesions; less than 4% of cases with systemic (visceral) involvement
- Morphologic subtypes: early (dense monomorphic histiocytic infiltration without lipidization, generally lacking Touton-type gian cells), classic (foamy histiocytes with variations of fully developed Touton giant cells), transitional (predominance of spindle-shaped cells, with foci of foamy histiocytes and giant cells, seen more in visceral organs) or combined
- Prognosis/Treatment: generally favorable; low relapse rate (7%); conservative excision; involution even after incomplete resection; multimodal chemotherapy (LCH-type chemotherapy) for the very rare systemic JXG

DIFFERENTIAL DIAGNOSIS
- Langerhans cell histiocytosis – positive for S100 and CD1a; negative for FXIIIa; only weak positive for monocyte/macrophage antibodies; lack or small number of Touton giant cells; EM shows Birbeck granules in histiocytes (JXG cells do not)
- Xanthoma – history (associated with hyperlipidemia); no other inflammatory cells

History:
Biopsy is of thumb mass. Prior bladder mass, chest wall mass, scalp mass and skull masses (all same diagnosis)
H & E

Comments

04/08/2011
Emory University Hospital
4

very nice representative slide.

05/28/2010
Loyola University Medical Center
5

Excellent case and description.

02/08/2010
Ranbax Lab
4

Very good case. Thanx.

Similar Cases

Image4-82
12/28/2011
California Hospital Medical Center
4

An elderly male in his mid 60s presented bilateral orbital tumors.
Procedure: resection of orbital tumor.
Gross: pink-tan, yellow fatty tissue fragments.
Micro: see photos (10x to 100x oil).

Image1
11/02/2011
Cure Inernational hospital
2.714285

41 years old lady with left breast mass

Groups: