Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography

Abstract

Erdheim-Chester Disease (ECD) is a rare non Langerhans cell histiocytosis of unknown origin with multiorgan involvement. We report a case of a man who presented to us with haematuria, asthenia, fever, nausea and malleolar oedema. After computed tomography and magnetic resonance imaging, the patient underwent a Positron Emission Tomography/Computed Tomography (PET/CT) with 18F-fluorodeoxyglucose (18F-FDG) that revealed multiple focal uptake in the skeleton and also visceral and vascular involvement. After a retroperitoneal biopsy, diagnosis of ECD was made. The treatment begun with steroids and after one month, a second 18F-FDG PET/CT was performed, highlighting a partial response. After five months of specific treatment with inhibitors of tyrosine kinases, a third 18F-FDG PET/CT was done showing a complete response. The 18F-FDG PET/CT allows assessing the extent of involvement in ECD, to detect the most easily accessible sites for diagnostic biopsy and to monitor disease activity and response to consolidated and new therapies.

Keywords

• fluorodeoxyglucose
• non langerhans cell histiocytosis
• vemurafenib