CTICasesErdheim-Chester disease
[...]. Erdheim-Chester disease. CTICases. Published August 31, 2018. https://str-case-explorer.pages.dev/c/exfkn
Imaging findings
Chest CT shows soft tissue infiltration surrounding the descending aorta and perinephric space. PET CT demonstrates intense FDG uptake in the posterior orbits bilaterally (retrobulbar infiltration), whereas the aortic and perinephric fibrotic lesions show minimal metabolic activity.
Key takeaways
Erdheim-Chester disease can involve multiple compartments, including the orbits where retrobulbar macrophage infiltration causes exophthalmos. PET CT is helpful to map disease activity, as chronic fibrotic lesions may be metabolically inactive.
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