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CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2021; 31(03): 729-734
DOI: 10.1055/s-0041-1736164
Case Report

Role of 18F-FDG PET/CT in Erdheim–Chester Disease in the Era of Multimodality Imaging

Bhargavi Jois
1   Department of Nuclear Medicine, Manipal Hospitals, Bengaluru, Karnataka, India
,
Rupa Ananthasivan
2   Department of Radiology, Manipal Hospitals, Bengaluru, Karnataka, India
,
Parameswaran R. V. Sudarshan Rawat
2   Department of Radiology, Manipal Hospitals, Bengaluru, Karnataka, India
,
Susmita Rakshit
3   Department of Pathology, Manipal Hospitals, Bengaluru, Karnataka, India
› Author Affiliations Funding None.
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Abstract

Erdheim–Chester disease is a rare disease with systemic non-Langerhans cell histiocytosis, the diagnosis of which with conventional imaging modalities is challenging. We describe a case of a 73-year-old woman who was referred with a progressive history of bilateral proptosis. The magnetic resonance imaging (MRI) orbit demonstrated bilateral orbital masses with optic nerve encasement. A subsequent 18F-FDG PET/CT scan showed multi-organ disease with involvement of the orbits, pericardium, aorta, pararenal fascia, and appendicular bones. Metabolically active, easily accessible areas were selected for CT-guided biopsy. The biopsy showed sheets of foamy histiocytes with the expression of CD 68 and CD 163 consistent with a diagnosis of Erdheim–Chester disease. The FDG PET/CT played a pivotal role in establishing the diagnosis with the assessment of disease extent and further guided in the targeted biopsy.

Keywords

Erdheim–Chester disease - FDG PET/CT - multimodality imaging


Publication History

Article published online:
06 October 2021

© 2021. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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