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CC BY 4.0 · Aorta (Stamford) 2013; 01(02): 146-148
DOI: 10.12945/j.aorta.2013.13-023
Images in Aortic Disease
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Imaging Assessment of Periaortic Inflammation in Erdheim-Chester Disease

Authors

  • Thierry Couvreur

    1   Department of Medical Imaging, University Hospital–Sart Tilman, Liège, Belgium

  • Györgyi Lipcsei

    2   Department of Pathology, CHC–St Joseph, Liège, Belgium

  • Alain Nchimi

    1   Department of Medical Imaging, University Hospital–Sart Tilman, Liège, Belgium
Further Information

Publication History

09 April 2013

25 May 2013

Publication Date:
28 September 2018 (online)

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Abstract

Reaching etiologic diagnoses for retroperitoneal fibrosis may be challenging. We report the case of a 75-year old male with history of ruptured abdominal aortic aneurysm and subsequent retroperitoneal fibrosis who developed four years later a soft tissue infiltration surrounding the ascending thoracic aorta. Thanks to his medical records and multimodality imaging assessment, the patient escaped an open-chest biopsy through histolgical reassessment of the abdominal periaortic samples that allowed the definitive diagnosis of Erdheim-Chester disease, a rare non-Langerhans histiocytosis.

Key Words

Aortitis - Histiocytosis - Erdheim-Chester - Magnetic Resonance Imaging - Positron Emission Tomography