Multimodality imaging showing complete cardiovascular involvement by Erdheim-Chester disease

Area of Science:

• Cardiology
• Oncology
• Pathology

Background:

• Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytosis.
• Characterized by xanthogranulomatous infiltrates affecting multiple organs.
• Cardiac involvement significantly worsens prognosis, second only to central nervous system disease.

Observation:

• This report details the first documented case of Erdheim-Chester disease with pan-cardiac involvement.
• Diagnosis was achieved through comprehensive multimodality imaging techniques.

Findings:

• Multimodality imaging successfully identified extensive cardiac infiltration in Erdheim-Chester disease.
• This case highlights the potential for widespread cardiac manifestation in ECD.

Implications:

• Early and accurate diagnosis of cardiac ECD is crucial for patient management.
• Understanding ECD's cardiac impact can guide therapeutic strategies and improve outcomes.