A 62-year-old African-American male smoker initially presented to the emergency department with acute sharp retrosternal pain that worsened with deep inspiration. He denied shortness of breath, fever, chills, chest trauma, sick contacts or recent travel. He worked as a custodian at a dairy factory and had no pets. His past medical history was notable for latent tuberculosis treated ten years prior. His physical examination revealed ectopic beats, but was otherwise normal. Laboratory studies demonstrated a normocytic anemia and elevated CRP (142 mg/L), normal troponin, complement levels, and negative ANA and ANCA. ECG showed diffuse elevation of ST segments in the precordial leads. Chest radiography was unremarkable. Computed tomography angiogram of the chest showed ground glass pulmonary nodules as well as a small pericardial effusion and pericardial enhancement. The initial concern was for pericarditis. Echocardiogram disclosed a reduced left ventricular ejection fraction of 38%, and a small circumferential pericardial effusion, without evidence of cardiac tamponade or constriction. Cardiac magnetic resonance imaging disclosed a thickened pericardium which enhanced on post contrast imaging suggesting active inflammation. He was presumptively diagnosed with viral myopericarditis and treated with colchicine.
Two weeks later, the patient again presented to the emergency department, this time with dyspnea. He was found to be hypoxemic, and chest radiography revealed a new large right-sided pleural effusion (Fig. 1).
A thoracentesis was performed with 2700 mL of dark amber fluid removed; evaluation of the pleural fluid revealed an exudative effusion. Gram stain and bacterial cultures were negative. Cytology did not reveal malignant cells. Cell differential demonstrated 2119 cells: 49% neutrophils, 18% lymphocytes, 24% monocytes/macrophages, and 7% mesothelial cells. Glucose was 105 mg/dL, and pH was 7.39. Echocardiogram was notable for newly found constrictive physiology. Left ventricular ejection fraction was 43%. After discharge, his pleural effusion re-accumulated rapidly, requiring multiple large volume thoracenteses over the next few weeks. Repeat pleural fluid cytology remained negative.
Given the recurrent unilateral pleural effusion and unrevealing fluid analysis, he underwent medical thorascopic pleural biopsy. Operative findings revealed scattered areas of fleshy nodularity involving the diaphragm and posterior sulcus, most consistent with fat (Fig. 2). Unexpectedly, pleural biopsies were consistent with high-grade pleural epithelioid angiosarcoma. Immunoperoxidase studies showed reactivity to CD31, FLI-1, AE1/3, OSCAR, WT-1, and D2-40 (Fig. 3).
Staging PET scan (Fig. 4) revealed malignant FDG-PET avidity of the pericardium, with a loculated pericardial effusion (SUV max 16.1). It also showed FDG activity in the right pleural effusion, right iliac bone, left shoulder soft tissue, and bilateral pulmonary nodules.
A few hours after the PET scan, he presented to the Emergency Department with a near-syncopal episode, and was found to be in shock. Electrocardiogram revealed ST-segment abnormalities concerning for acute myocardial infarction. The patient went into pulseless electrical activity arrest. Despite chest compressions, intravenous epinephrine, and emergent pericardiocentesis, the patient did not regain a pulse. Family requested cessation of resuscitation efforts, and the patient passed. Autopsy was declined.