CTICasesErdheim-Chester Disease with Peri-Renal and Mediastinal Infiltration
Imaging findings
CT in a patient with biopsy-confirmed Erdheim-Chester disease demonstrates a large infiltrating soft tissue mass in the paraspinal fat, abnormal soft tissue encasing the right atrium and encroaching on the right atrial lumen within the sub-epicardial fat, and bilaterally enlarged heterogeneous kidneys with peri-renal soft tissue infiltration. An incidental large hiatal hernia is also present.
Key takeaways
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with characteristic peri-renal fat infiltration (hairy kidney appearance), retroperitoneal soft tissue deposition, and cardiovascular involvement including pericardial and sub-epicardial infiltration. Cardiac ECD most commonly involves the right atrium and may compromise chamber dimensions. The distribution in fat-containing spaces (retroperitoneum, paraspinal fat, sub-epicardial fat) is characteristic and differs from lymphoma.
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