Composite hemangioendothelioma: the first case of a right atrioventricular pericardial tumour

Comprehensive cardiac examination of a 50-year-old female patient with first time intermittent atrial fibrillation and suspected coronary artery disease (CAD) was performed on a 1.5 Tesla magnetic resonance imaging (MRI) system. Cardiac four-chamber (Figure 1A) and short-axis view cinematic MRI delineated an isolated inhomogeneous, but well-structured cardiac mass (9.2 × 5.9 × 5.8 cm³, Supplementary material online, Images and Movies ‘4CH and SA’). The mass was located at the pericardium of the right atrioventricular junction and clearly compressed the right atrium. Contrast-enhanced T₁-weighted perfusion MRI revealed that the mass had an independent blood supply. Interestingly, the mass showed marginal perfusion and missing signal intensity in the central region during the first pass of the gadolinium-based contrast agent (Supplementary material online, Images and Movie ‘Perfusion’). The mass was sharply demarcated and there was no evidence of an infiltration based on the perfusion-weighted MRI. A preoperative invasive coronary angiography confirmed the MRI findings and ruled out CAD. In angiography, the mass was directly supplied by branches from the right coronary artery (Supplementary material online, Movie ‘HearthCath’). According to imaging characteristics as well as patient characteristics, the mass was initially suspected as cardiac sarcoma. After surgical resection (Figure 1B), histology revealed that the tumour shared features suggestive of an epithelioid hemangioendothelioma. The tumour also had a separate blood supply with a cavernous blood vessel configuration. This aberrant perfusion together with the histology supported a diagnosis of ‘composite hemangioendothelioma’.

Figure 1.

Four-chamber view cine magnetic resonance image shows an inhomogeneous pericardial mass at the right atrioventricular junction (A, white arrow). Intraoperative picture of the completely resected tumour with a total volume of 9.2 × 5.9 × 5.8 cm³ (B).

Supplementary material

Supplementary material is available at European Heart Journal - Case Reports online.

Consent: The author/s confirm that written consent for submission and publication of this case report including image(s) and associated text has been obtained from the patient in line with COPE guidance.

Conflict of interest: none declared.