A 28-year-old man presented with sudden severe palpitations and moderate dyspnoea for 1 day.
Transthoracic echocardiography showed a mass in the right atrium and severe pericardial effusion (Panels A and B). The mass pulsated with the heartbeat and entered the right ventricle through the tricuspid valve during diastole, and the flow velocity of the tricuspid valve rapidly increased (Supplementary material online, Video S1). Anechoic areas were found within the mass (Panel B, Supplementary material online, Videos S2 and S3).
Computed tomography showed a mixed density mass of 70 mm × 60 mm × 85 mm in the right atrium (Panel C). Axial and sagittal cardiac cine magnetic resonance angiogram showed a marked round mass in the right atrium, and the signal was mixed (Panels D and E). Diffusion-weighted imaging (b = 600) showed a mass that appeared isointense to slightly hyperintense (Panel F), with mild heterogeneous enhancement after gadolinium injection (Panel G).
The patient then underwent surgery. Cauliflower-like masses were observed on the surface of the right atrium, left atrium, and aortic root, along with part of the right ventricle that could not be safely removed (Panel H, Supplementary material online, Video S4).
Histopathological examinations revealed that the specimens were positive for CD34, CD31, and FLI-1 and were negative for ckp and desmin (Panel I). The proliferation rate evaluated with the Ki67 antibody was 30%. The final diagnosis was primary cardiac epithelioid haemangioendothelioma (EHE).
Epithelioid haemangioendothelioma has a low incidence in the general population (<0.1 per 100,000,2). Primary cardiac EHEs are even rarer, with less than 20 reported cases; its aetiology and optimal treatment remain unknown.
Supplementary material is available at European Heart Journal online.
Conflict of interest: The authors have submitted their declaration which can be found in the article Supplementary Material online.
Author notes
Ruifeng Yan author is considered as co-first author.
