We report a case of Erdheim-Chester disease (ECD) with isolated cardiac involvement
in a 74-year-old female patient. The patient initially presented with superior vena
cava syndrome and PET-CT imaging demonstrating an obstructing hypermetabolic lesion
in the right atrium, and a distinct nonobstructing hypermetabolic lesion in the left
atrium, expected to be malignant. There was no evidence of extracardiac disease. At
surgical exploration, consistent with malignancy, the right atrial tumor was found
to have grown into the pericardium and was resected to address symptoms and for histological
diagnosis which revealed ECD on immunohistochemistry. We conclude that isolated cardiac
ECD should be included in the surgical strategy for cardiac tumors showing infiltrative
growth.
Keywords
cardiac - inflammation - systemic (incl. cells, mediators) - tumor - Erdheim-Chester
disease - non-Langerhans cell histiocytosis - immunohistochemical staining - cardiac
tumor
