CTICasesErdheim-Chester Disease
Imaging findings
Cardiac MRI and PET/CT demonstrate an avidly perfusing, hypermetabolic, non-invasive soft-tissue mass wrapping around the interventricular septum, posterior right atrial wall, ascending aorta, and superior vena cava. Concurrently, there is diffuse bilateral pleural thickening, hypermetabolic pericardial thickening, and symmetric perinephric soft-tissue encasement in the abdomen.
Key takeaways
Erdheim-Chester disease is a rare systemic non-Langerhans cell histiocytosis that classically presents with infiltrative, fibro-inflammatory histiocytic soft-tissue encasement. Multiorgan involvement is common, typically manifesting as a "hairy kidney" appearance in the retroperitoneum alongside symmetric pleural, pericardial, and cardiovascular wrapping.
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