CTICasesErdheim-Chester Disease with Cardiac and Retroperitoneal Involvement
[...]. Erdheim-Chester Disease with Cardiac and Retroperitoneal Involvement. CTICases. Published May 17, 2018. https://str-case-explorer.pages.dev/c/rft6y
Imaging findings
A large pericardial effusion is present along with abnormal soft tissue in the right atrioventricular groove partially encasing the right coronary artery, a pleural effusion, and circumferential soft tissue surrounding the descending aorta that extends down to involve the perirenal regions bilaterally. Limited cardiac MRI suggested additional soft tissue involving the interatrial septum.
Key takeaways
The circumferential periaortic and perirenal soft tissue is a classic pattern of Erdheim-Chester disease, a non-Langerhans cell histiocytosis. This case was notable for cardiac involvement with soft tissue encasing the right coronary artery and possibly the interatrial septum, a less commonly recognized manifestation than the typical retroperitoneal and skeletal findings.
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