Erdheim-Chester Disease with Perirenal Masses Containing Macroscopic Fat Tissue

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.

Keywords

• erdheim-chester disease
• non-langerhans-cell histiocytosis
• kidney
• retroperitoneal fibrosis