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

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

• Pancreatitis
• Erdheim-Chester disease
• PET scan
• Histiocytes
• Retroperitoneum
• BRAFV600E