Pancreatic involvement in Erdheim-Chester disease: Rare presentation of a rare disease

oleh: Moezedin Javad Rafiee, MD, Jana Taylor, MD, FRCP, Marc Hickeson, MD, Matthias G. Friedrich, MD, FESC, FACC, Michael Chetrit, MD, FACC

Format: Article
Diterbitkan: Elsevier 2023-05-01

Deskripsi

Erdheim-Chester disease (ECD) as a rare non-Langerhans histiocytosis has various clinical manifestations. It is characterized histologically by infiltration of every organ, more commonly bone, retroperitoneum, cardiovascular and CNS systems with foamy, lipid -laden macrophage. Pancreatic involvement as a manifestation of this uncommon disease has very rarely been reported. Here we report a 73-year-old woman with ECD and pancreas involvement in CT, MRI and PET scans. We also aim to increase radiologist knowledge about considering ECD as a differential diagnosis for pancreas mass in the appropriate clinical situation.