Erdheim-Chester Disease: Utility of 18F-FDG Positron Emission Tomography
Dr. Angelina Cistaro,
V. O. Vigliani 89/A, 10138 Turin, Italy.
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.