Teaching Case

A 33-year-old male without significant medical history. The patient presented to the emergency department with dyspnoea, night sweats and abdominal discomfort. Laboratory investigations revealed mildly elevated troponin levels, elevated inflammatory markers and discrete anaemia.

Transthoracic cardiac ultrasound showed a soft tissue mass of approximately 6.5 centimetres in the right atrium, with evidence of flow obstruction through the tricuspid valve (Figure 1). Further evaluation with a CT scan revealed a large necrotising mass centred on the right atrial wall with extension into the right atrium, the right atrial appendage, and the adjacent pericardium, with an associated malignant pericardial effusion (Figure 2). A cardiac MRI was performed for further characterisation of this tumour. This confirmed the diagnosis of a cardiac angiosarcoma based on localisation and heterogenous signal intensity due to intralesional necrosis and haemorrhage (Figures 3 and 4).

Background

Cardiac angiosarcoma, an exceedingly rare and highly aggressive malignancy arising from endothelial cells within the heart, represents the most prevalent subtype among primary cardiac sarcomas, constituting approximately 30% of cases. The tumour often arises around the free wall of the right atrium or the right atrioventricular groove and extends into the lumen. It typically affects young to middle-aged adults, with a slight male predilection [1]. Characteristic features of cardiac angiosarcoma are the rapid formation of abnormal tumoral blood and extensive intralesional necrosis and haemorrhage [2].

Clinical Perspective

Clinical presentation of cardiac angiosarcoma can be diverse and is often nonspecific, leading to delayed diagnosis. The most reported symptom by patients is dyspnoea, caused by the tumour’s obstructive effect on cardiac blood flow, resulting in right-sided heart failure. Patients may also experience chest pain and palpitations. Most cases have systemic metastasis at the time of diagnosis, leading to constitutional symptoms such as weight loss and fatigue [2].

Imaging Perspective

Cardiac angiosarcomas can be visualised on echocardiography, CT and MRI. MRI is the most useful modality for tissue and tumour characterisation, while CT plays a role in oncological staging. Radiologically, cardiac angiosarcomas manifest in two primary morphologic configurations. First, a well-defined mass protruding into a cardiac chamber, notably the right atrium, characterised by heterogeneous contrast enhancement attributed to intralesional necrosis and haemorrhage (as demonstrated above), this type presents as a low attenuation mass on CT. On MRI, the mass can present as a heterogeneous mass on T1WI and T2WI with focal haemorrhagic areas, known as the “cauliflower” appearance. The second type presents as a diffusely infiltrative mass extending along the pericardium, often resulting in obliteration of the pericardial space with haemorrhagic and necrotic tumour debris. On MRI, this can present as the “sunray” appearance with linear contrast enhancement. In general, cardiac angiosarcomas have a heterogeneous signal intensity on MRI due to haemorrhage and necrosis [3].

Outcome

The prognosis remains very poor, with a median survival typically ranging from a few months to a couple of years since most patients have systemic metastasis at the time of diagnosis. Surgical resection, when possible, remains the cornerstone of the treatment, often complemented by radio- and chemotherapy. However, complete resection is challenging due to the tumour's infiltrative nature. Thus, a prompt diagnosis is key to therapeutic management [2].

Take Home Message

Cardiac angiosarcomas are very rare but aggressive malignancies of endothelial origin, often presenting as a very heterogeneous mass arising from the right atrium and requiring early radiological recognition.

All patient data have been completely anonymised throughout the entire manuscript and related files.