BEATING THE ODDS 4-YEAR SURVIVAL WITH PRIMARY CARDIAC ANGIOSARCOMA

A 49-year-old man presented with a 6-week history of exertional dyspnoea. Echocardiogram demonstrated a pericardial effusion. Pericardiocentesis was performed, but fluid cytology was negative for malignancy. Nine months later, repeat echocardiogram showed a right atrial mass and cardiac MRI showed a heterogeneous mass suspicious for cardiac sarcoma. Histology confirmed a cardiac angiosarcoma, deemed inoperable due to cardiac wall infiltration. He commenced weekly paclitaxel treatment and demonstrated a 70% reduction in cardiac mass on CT after two cycles of therapy. An application for cardiac transplant was refused. His tumour showed no response to two further cycles of paclitaxel and so he was switched to liposomal doxorubicin. This was stopped prematurely due to grade 3 hand-foot skin toxicity. CT scans also demonstrated further disease progression. He was transferred to the Royal Marsden for resection of his primary tumour, with clear margins on resection specimen. He remained disease-free for ten months when liver lesions were identified on staging CT. Biopsy confirmed metastatic disease. He was treated with a tyrosine kinase inhibitor and an mTOR inhibitor but ultimately progressed and therapy was discontinued at patient request. Four months later he represented with symptomatic anaemia. CT showed disease progression with a large hepatic metastasis eroding into omentum. He was treated with further chemotherapy but passed away 51 months after diagnosis. We discuss the treatment options available for this rare condition. Primary malignant cardiac tumours are rare. Clinical diagnosis is often delayed so patients present with advanced stage disease, usually involving the right atrium, often inoperable at presentation. Cardiac angiosarcoma is particularly aggressive, with median survival of 6-12 months. The benefit of chemotherapy in this setting is usually short-lived; with the regimens most commonly used involving doxorubicin or paclitaxel, although more recent studies have suggested a role for targeted agents such as tyrosine kinase inhibitors or mTOR inhibitors. To our knowledge, this case represents the longest documented survival in adult cardiac angiosarcoma and highlights the need for multidisciplinary care in this rare disease.