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

Moezedin Javad Rafiee; Jana Taylor; Marc Hickeson; Matthias G Friedrich; Michael Chetrit

doi:10.1016/j.radcr.2023.02.012

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

Radiol Case Rep. 2023 Mar 4;18(5):1809-1820. doi: 10.1016/j.radcr.2023.02.012. eCollection 2023 May.

Authors

Moezedin Javad Rafiee¹, Jana Taylor², Marc Hickeson³, Matthias G Friedrich¹, Michael Chetrit⁴

Affiliations

¹ Department of Medicine and Diagnostic Radiology, McGill University Health Centre, 1001 Blvd, Decarie, Montreal, Québec, H4A3J1 Canada.
² Department of Diagnostic Radiology, McGill University Health Centre, 1001 Blvd, Decarie, Montreal, Québec, H4A3J1, Canada.
³ Department of Nuclear Medicine, McGill University Health Centre, 1001 Blvd, Decarie, Montreal, Québec, H4A3J1, Canada.
⁴ Department of Medicine, Division of Cardiology, McGill University Health Centre, 1001 Blvd, Decarie, Montreal, Québec, H4A3J1, Canada.

Abstract

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.

Keywords: BRAFV600E; CMR, Cardiac Magnetic Resonance Imaging; ECD, Erdheim-Chester disease; Erdheim-Chester disease; Histiocytes; PET scan; Pancreatitis; Retroperitoneum.

Publication types

Case Reports