Article ID: CJ-21-0158
A 54-year-old woman visited a primary care clinic with palpitations and was referred to hospital for a detailed examination of coronary artery disease. ECG revealed premature ventricular contraction. Computed tomography revealed diffuse thickness of the left atrial wall (Figure A) without coronary artery stenosis. Transthoracic and transesophageal echocardiography revealed a diffuse left atrial mural pseudotumor (Figure B,C) and magnetic resonance imaging demonstrated that the mural involvement had a heterogeneous signal on T2-weighted imaging (Figure D). 18F-fluorodeoxyglucose positron emission tomography demonstrated a high metabolic response (maximum standardized uptake value was 21.99) limited to the left atrium (Figure E). We performed a biopsy of the left atrial pseudotumor with a percutaneous transvenous and transseptal approach (Figure F). Pathological findings were non-Langerhans cell histiocytosis characterized by widespread tissue infiltration by CD68-positive (Figure G) and CD1a-negative (Figure H) foamy histiocytes, and we diagnosed the patient as having Erdheim-Chester disease (ECD). The patient was treated with interferon α-2a and the tumor effectively shrank (Figure I).
Images of the diffuse left atrial mural pseudotumor (arrows) by computed tomography (A), transesophageal echocardiography (TEE) (B,C), magnetic resonance imaging (D), and 18F-fluorodeoxyglucose positron emission tomography (E). Percutaneous transvenous and transseptal biopsy of the left atrial pseudotumor (F, arrows). Pathology specimen showing non-Langerhans cell histiocytosis characterized by CD68-positive (G) and CD1a-negative (H) foamy histiocytes. TEE image of shrunk pseudotumor after interferon α-2a treatment (I, arrow). LA, left atrium; RA, right atrium.
Pseudotumor of the right atrium and auriculoventricular sulcus is the most common cardiac manifestation of ECD, so its location in the left atrium is very rare. The images of the pseudotumor were characteristic and early pathological diagnosis led to successful treatment.
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