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

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

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Erdheim-Chester Disease (ECD) is a rare non Langerhans knowall.blog 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 echofix spring reverb 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.