A mimicker of constriction: a case report of a rare case of cardiac angiosarcoma

Cardiac angiosarcoma is an exceptionally rare primary malignant tumour with an aggressive course and typically poor prognosis. Diagnosis is difficult, and patients often present with metastatic disease. We report the rare case of a patient with cardiac angiosarcoma who presents with constrictive physiology due to tumour encasement.

A 65-year-old female with a past medical history of Hodgkin's lymphoma and limited scleroderma presented with progressive dyspnoea on exertion. Multimodality imaging and haemodynamics with echocardiography, cardiac magnetic resonance imaging (MRI), and cardiac catheterization showed findings of constrictive physiology. Cardiac MRI showed areas of pericardial enhancement, so she was initially started on colchicine, prednisone, and mycophenolate mofetil to treat pericardial inflammation. However, her symptoms progressed, and she underwent pericardiectomy with cardiac surgery. Pericardium was noted to be thickened and a mass-like substance was densely adherent and potentially invading the heart itself and could not be dissected free. Surgical pathology showed features consistent with epithelioid angiosarcoma. Patient had rapid progression of her disease and was started on chemotherapy. Her course, however, was complicated by acute gastrointestinal bleeding, atrial fibrillation with rapid rates, and persistent volume overload. She elected for comfort measures and passed away shortly after her diagnosis.

Our case shows an extremely rare diagnosis, cardiac angiosarcoma, presenting with typical findings of constrictive physiology. The case shows the typical features of constrictive physiology using multimodality imaging and haemodynamics and emphasizes the need to always think broadly in creating a differential diagnosis for constriction to ensure that rare diseases are considered.