CTICasesErdheim-Chester Disease
Imaging findings
Chest CT demonstrates an irregular, soft-tissue rind of infiltration within the anterior mediastinum, accompanied by a moderate pericardial effusion and pleural effusion. Abdominal CT demonstrates symmetric, soft-tissue halos of perinephric infiltration surrounding both kidneys, representing the hairy kidney sign, as well as retrocrural soft-tissue infiltration. A PET/CT scan shows unexpectedly low metabolic activity within these soft-tissue lesions.
Key takeaways
Erdheim-Chester disease is a rare, non-Langerhans cell histiocytosis characterized by systemic lipid-laden histiocytic infiltration and fibrosis. Classic imaging features include perinephric soft-tissue infiltration and mediastinal or pericardial encasement. Although often highly FDG-avid at baseline, treated lesions, such as those following interferon therapy, may show minimal metabolic uptake due to the resolution of active inflammation and persistence of dense fibrosis.
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