CTICasesErdheim-Chester Disease
Imaging findings
CT abdomen and chest demonstrate a circumferential, symmetric rind of soft-tissue density infiltrating the retroperitoneum and perirenal space, encasing the kidneys (hairy kidney sign) and abdominal aorta. An infiltrate of soft tissue is also present in the chest wall. PET/CT shows mild FDG uptake in the periaortic tissue.
Key takeaways
Erdheim-Chester disease is a rare, non-Langerhans cell histiocytic neoplasm. It classically presents with symmetric perirenal and retroperitoneal infiltration (hairy kidney sign) and osteosclerosis of the long bones. Tissue biopsy showing foamy histiocytes and fibrosis is diagnostic, though often difficult to distinguish from idiopathic fibrosis.
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