CTICasesErdheim-Chester disease
[...]. Erdheim-Chester disease. CTICases. Published August 23, 2018. https://str-case-explorer.pages.dev/c/ma7gs
Imaging findings
Chest and abdominal CT show a symmetric soft tissue infiltrate encasing the pericardium, right atrium, interatrial septum, and coronary arteries, as well as a symmetric perinephric soft tissue rind. Brain and orbital MRI show pons/cerebellar enhancement and retro-orbital soft tissue infiltration. Tibia MRI confirms marrow infiltration.
Key takeaways
Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis characterized by infiltration of lipid-laden histiocytes. Classic imaging findings include symmetric perinephric infiltration (hairy kidney), cardiovascular infiltration (coated aorta/cardiac pseudotumor), retro-orbital/pons infiltration, and symmetric osteosclerosis of the long bones.
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