J Korean Soc Radiol.  2015 Feb;72(2):143-146. 10.3348/jksr.2015.72.2.143.

Erdheim-Chester Disease with Perirenal Masses Containing Macroscopic Fat Tissue

• Byeon J
• Kim KA
• Hwang SS
• Park SY
• Kim HA

• KMID: 2002813
• DOI: http://doi.org/10.3348/jksr.2015.72.2.143

Abstract

Erdheim-Chester disease is a rare non-Langerhans-cell histiocytosis involving multiple organs. On histological evaluation, lipid-laden histiocyte aggregates in Erdheim-Chester disease is detected, but fat tissue in affected organs is not noted grossly on computed tomography. A 40-year-old man presented with bilateral perirenal masses containing fat tissue. He was diagnosed as perirenal involvement of Erdheim-Chester disease. This report describes a case of Erdheim-Chester disease with perirenal involvement that demonstrates unusual features.

MeSH Terms

Adult
Erdheim-Chester Disease*
Histiocytes
Histiocytosis, Non-Langerhans-Cell
Humans
Kidney
Retroperitoneal Fibrosis

Figure

• 

  Fig. 1 A 40-year-old man with Erdheim-Chester disease. Bilateral perirenal masses with faint fat tissue (white arrows) are revealed on axial images (A) and coronal image of abdominal CT (B). Fused positron emission tomography (PET)/CT shows diffuse and moderate fluorodeoxyglucose (FDG) activity (maximum standardized uptake value range, 2.2-3.8) in bilateral perirenal masses (white arrows) (C). Some parts of lung and pleura (white arrow) also show mild patchy FDG activity on fused PET/CT (D). Multifocal ground glass opacities with interlobular and intralobular septal thickening and thickening of the right pleura (black arrow) are noted on chest CT (E). H&E stain (× 400) indicates lipid-laden histiocyte aggregation (black arrow for one among many lipid-laden histiocyte) (F). Immunohistochemical stains show that CD68 (× 200) (G) is positive and CD1a (× 200) (H) and S-100 (× 200) (I) are negative.

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