Case Presentation:
A 42-year-old female with no past medical history presented with increased dyspnea on exertion. She was initially diagnosed with pericarditis and pulmonary nodules at an outside hospital. After continued symptoms, studies revealed a heterogenous, echodense mass was visualized in the right atrium near the entrance of the superior vena cava and the interatrial septum, approximately 3.5cm x 4.5cm, small pericardial effusion, narrowing of the main and bilateral pulmonary vessels, with multiple, ill-defined, pulmonary nodules seen. The patient later presented to the ED with worsening chest pain, shortness of breath, right shoulder and head pain and worsening exercise tolerance. She endorsed a 20-lb weight loss since discharge, with nausea, vomiting, dysphagia, orthopnea, paroxysmal nocturnal dyspnea, and lower extremity edema. Her blood pressure was 94/62 and exam revealed a fixed split S2, a soft S3, fullness of the supraclavicular spaces, clear lungs, and a notably increased JVD even with the patient sitting at 90 degrees.
The patient was admitted and cardiology, cardiothoracic surgery, and oncology were consulted. Cardiac MRI revealed an infiltrative pericardial and posterior atrial myocardial mass, now encasing several major vascular structures and causing narrowing of the SVC. Due to the tumor location, obtaining tissue for diagnosis was challenging; it was not amenable to biopsy via transesophageal echocardiogram and there was concern that a surgical biopsy could pose potential risks of post-procedural ventilator-dependence because of the patient’s tenuous clinical state. The patient’s symptoms continued to deteriorate. She had evidence of demand ischemia and a large right sided pleural effusion. A pigtail catheter was placed to drain the effusion and the patient developed hypoxemia which required non-invasive ventilation. She was transferred to the ICU, where she developed hypothermia and hypotension requiring vasopressor therapy. Empiric chemotherapy was initiated to treat presumed lymphoma. The patient had a myocardial infarction, and her clinical condition continued to deteriorate, with multi-organ failure and DIC ensuing. She expired despite resuscitative measures. Autopsy and subsequent pathology revealed angiosarcoma with extensive infiltration throughout the heart and great vessels.
Discussion:
Primary cardiac tumors are rare, with a 0.002-0.3% incidence, based on autopsies, with angiosarcoma as the most common malignant tumor. Metastasis to the heart is a more common occurrence with an incidence ranging from 2.3% and 18.3%. When a patient presents with an aggressive cardiac mass, angiosarcoma should be considered. Patients will have a history of fever, weight loss, exhaustion, night sweats, and coughing. Our patient had these symptoms, along with signs consistent with angiosarcoma: pericarditis and pleural effusion. Definitive diagnosis is made primarily by histology, but clinical symptoms and a rapidly declining course, in conjunction with imaging.
Conclusions:
Confirming a diagnosis of angiosarcoma can be challenging in a patient with a rapidly declining course, as the risks of diagnostic procedures can outweigh the perceived benefits. However, a clinical diagnosis with symptomology, rapid course and imaging can lead to a more prompt diagnosis.