CTICasesErdheim-Chester Disease
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Imaging findings
Chest CT demonstrates severe pulmonary emphysema alongside synchronous, bilateral, spiculated and lobulated nodules in the right upper and right lower lobes, which were biopsy-proven adenocarcinomas. Strikingly, soft tissue windows reveal extensive, abnormal homogeneous soft tissue enveloping the thoracic aorta, producing a classic "coated aorta" appearance, along with thickening and soft tissue encasement of the right atrium. Abdominal imaging shows symmetric retroperitoneal soft tissue infiltration wrapping around the kidneys in a characteristic "hairy kidney" pattern. FDG-PET imaging demonstrates intense, hypermetabolic uptake in all these areas, particularly the right atrial wall, the retroperitoneal renal masses, and the primary lung tumors.
Key takeaways
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis characterized by multiorgan infiltration by foamy histiocytes. Diagnostic radiological hallmarks include a "coated aorta," retroperitoneal soft tissue causing a "hairy kidney" appearance, and right atrial infiltration. This case highlights a patient diagnosed with ECD who also presented with synchronous, bilateral primary lung adenocarcinomas.
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