Cardiothoracic manifestations of Erdheim-Chester disease Review
| Authors: | Das, J. P.; Xie, L.; Riedl, C. C.; Hayes, S. A.; Ginsberg, M. S.; Halpenny, D. F. |
| Review Title: | Cardiothoracic manifestations of Erdheim-Chester disease |
| 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. © 2019 The Authors. |
| Keywords: | immunohistochemistry; treatment response; clinical feature; histopathology; review; nuclear magnetic resonance imaging; magnetic resonance imaging; radiopharmaceuticals; heart disease; lung disease; tomography, x-ray computed; diagnostic imaging; radiologist; fluorodeoxyglucose f 18; fluorodeoxyglucose f18; radiopharmaceutical agent; contrast medium; contrast media; heart diseases; lung diseases; lung parenchyma; pleura disease; complication; multimodal imaging; procedures; pleural diseases; mediastinum disease; mediastinal diseases; cardiovascular magnetic resonance; humans; human; erdheim chester disease; erdheim-chester disease; x-ray computed tomography; positron emission tomography-computed tomography; positron emission tomography computed tomography |
| Journal Title: | British Journal of Radiology |
| Volume: | 92 |
| Issue: | 1104 |
| ISSN: | 0007-1285 |
| Publisher: | British Institute of Radiology |
| Date Published: | 2019-01-01 |
| Start Page: | 20190473 |
| Language: | English |
| DOI: | 10.1259/bjr.20190473 |
| PUBMED: | 31386554 |
| PROVIDER: | scopus |
| PMCID: | PMC6913371 |
| DOI/URL: | |
| Notes: | Source: Scopus |
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