Cardiothoracic manifestations of Erdheim-Chester disease

The British Journal of Radiology
Jeeban Paul DasDarragh F Halpenny

Abstract

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis which can have a broad range of clinical and radiological presentations. Typically, ECD affects multiple organ systems, with skeletal involvement present in almost all ECD patients and cardiothoracic manifestations in more than half. Cardiac and thoracic involvement contributes significantly to morbidity and mortality in affected patients and may have prognostic implications. The diagnosis of ECD can be challenging due to its rarity and similarity to other systemic disease processes. Although the diagnosis can be suggested on imaging, histopathology and immunohistochemistry are required for confirmation. We describe the multimodal imaging features of mediastinal, cardiac, pleural and lung parenchymal ECD. This review identifies the most common radiological manifestations of cardiac and thoracic ECD on contrast-enhanced CT, fluorine18-fludeoxyglucose positron emission tomography/CT and cardiac MRI, and highlights the role of these cross-sectional techniques in disease diagnosis.

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Citations

Jul 2, 2020·Current Cardiology Reports·Johnny ChahineAllan L Klein
Sep 14, 2020·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Neeta Garg, Efrat Saraf Lavi
Jun 4, 2021·Survey of Ophthalmology·Menelaos KanakisIlias Georgalas

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