Keywords:
Nuclear medicine, Molecular imaging, Haematologic, PET-CT, Segmentation, Computer Applications-Detection, diagnosis, Cancer, Molecular, genomics and proteomics, Haematologic diseases
Authors:
G. Z. Papadakis1, G. MANIKIS1, F. Hannah-Shmouni2, A. H. Karantanas1, K. J. O’Brien2, W. A. Gahl2, J. I. Estrada-Veras2; 1Iraklion/GR, 2Bethesda, MD/US
DOI:
10.1594/ecr2018/C-3183
Aims and objectives
Erdheim-Chester disease (ECD) is a rare potentially fatal hematopoietic neoplasm of histiocytic origin occurring mainly in adults. ECD can affect several organs (Figure 1) including the adrenal glands [1].
ECD diagnosis relies upon clinical,
laboratory,
histologic,
and radiologic studies.
There is no established therapy for ECD with empiric treatments including antiinflammatory,
immunosuppressive,
and chemotherapeutic agents Recent data shows that the majority of ECD patients harbor BRAF V600E and MAPK pathway gene mutations,
initiating treatment strategies with BRAF and MEK inhibitors [2]. The estimated ECD mortality rate is 60% at 3 years from the time of diagnosis [3].
Since,
whole body 18F-FDG PET/CT imaging targets the abnormally increased metabolic rate of neoplastic cells,
it provides a highly efficient and sensitive method for the in-vivo monitoring of ECD actitivity given the numerous manifestations secondary to the disease.
Aim of the current study was to explore potential association between adrenal metabolic activity in ECD patients assessed by 18F-FDG PET/CT and the BRAF V600E mutation status.