Cardiothoracic manifestations of Erdheim-Chester disease

Area of Science:

• Radiology
• Histopathology
• Oncology

Background:

• Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with diverse clinical and radiological presentations.
• Skeletal and cardiothoracic involvement are common in ECD, significantly impacting patient morbidity and mortality.
• Diagnosing ECD is challenging due to its rarity and resemblance to other systemic diseases.

Purpose of the Study:

• To describe the multimodal imaging features of mediastinal, cardiac, pleural, and lung parenchymal ECD.
• To identify common radiological manifestations of cardiac and thoracic ECD.
• To highlight the role of cross-sectional imaging techniques in ECD diagnosis.

Main Methods:

• Review of multimodal imaging features.
• Analysis of contrast-enhanced CT, 18F-FDG PET/CT, and cardiac MRI findings.
• Correlation with histopathology and immunohistochemistry for diagnosis confirmation.

Main Results:

• Detailed description of imaging findings in various thoracic compartments affected by ECD.
• Identification of characteristic radiological patterns for cardiac and thoracic ECD.
• Demonstration of the utility of advanced imaging modalities in diagnosing ECD.

Conclusions:

• Multimodal imaging, including CT, PET/CT, and MRI, is crucial for diagnosing cardiac and thoracic Erdheim-Chester disease.
• Recognizing specific imaging features can aid in the early diagnosis of this rare condition.
• Histopathology and immunohistochemistry remain essential for definitive ECD confirmation.