CTICasesEpicardial and perinephric infiltration in Erdheim-Chester disease
Imaging findings
Cardiac MRI shows an avidly enhancing, infiltrative soft tissue mass centered in the epicardial fat wrapping around the atria and encasing the coronary arteries without significant stenosis. Abdominal CT demonstrates symmetric perinephric soft tissue infiltration ('hairy kidney').
Key takeaways
Erdheim-Chester disease is a rare histiocytic neoplasm that characteristically infiltrates the epicardial fat (often encircling the atria) and the perinephric space. Despite vascular encasement, significant stenosis of the coronary arteries is uncommon.
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