Radiological findings in Erdheim Chester disease: A very rare multisistemic disease

Marcello Chiocchi, MD, PhD; Alessandra Luciano, MD; Vincenzo De Stasio, MD; Luca Pugliese, MD; Carlo Di Donna, MD; Martina Cerocchi, MD; Paola Gigliotti, MD; Alessandro Carini, MD; Flavia Chirico, MD; Riccardo Camedda, MD; Daniele Di Biagio, PhD; Paolo Francesco Sbordone, MD; Francesco Garaci, PhD; Roberto Floris, MD, PhD

Radiology Case Reports (May 2023)

Radiological findings in Erdheim Chester disease: A very rare multisistemic disease

Marcello Chiocchi, MD, PhD,
Alessandra Luciano, MD,
Vincenzo De Stasio, MD,
Luca Pugliese, MD,
Carlo Di Donna, MD,
Martina Cerocchi, MD,
Paola Gigliotti, MD,
Alessandro Carini, MD,
Flavia Chirico, MD,
Riccardo Camedda, MD,
Daniele Di Biagio, PhD,
Paolo Francesco Sbordone, MD,
Francesco Garaci, PhD,
Roberto Floris, MD, PhD

Affiliations

Marcello Chiocchi, MD, PhD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Alessandra Luciano, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy; Corresponding author.
Vincenzo De Stasio, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Luca Pugliese, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Carlo Di Donna, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Martina Cerocchi, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Paola Gigliotti, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Alessandro Carini, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Flavia Chirico, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Riccardo Camedda, MD: Department of Diagnostic Oncoematology and Nuclear Medicine, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Daniele Di Biagio, PhD: Department of Diagnostic Oncoematology and Nuclear Medicine, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Paolo Francesco Sbordone, MD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Francesco Garaci, PhD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy
Roberto Floris, MD, PhD: Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy

Journal volume & issue: Vol. 18, no. 5
pp. 2047 – 2054

Abstract

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Erdheim-Chester disease is an uncommon non-Langerhans cell histiocytosis affecting multiple systems. There is limited knowledge on the imaging capabilities of this disease. We present an extremely rare case of Erdheim-Chester illness in a 67-year-old man with multisystem involvement, including the cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration) and the neurologic system. The involvement of the various organs was thoroughly assessed using multimodal imaging modalities such as computed tomography, magnetic resonance imaging, positron emission tomography and bone scintigraphy. Erdheim-Chester illness was revealed by a bone biopsy. Especially when there is cardiac and cerebral involvement, Erdheim-Chester illness is a rare condition with a poor prognosis. Knowing the imaging characteristics of Erdheim-Chester disease may be helpful in understanding the radiological results of many organs affected by the disease as described and discussed in the current case report.

Published in Radiology Case Reports

ISSN: 1930-0433 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Medicine (General): Medical physics. Medical radiology. Nuclear medicine
Website: https://www.sciencedirect.com/journal/radiology-case-reports

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