A 44-year-old woman presented to the emergency room at our institution with chest pain and shortness of breath. She had no significant past medical history or family history. She denied any history of smoking, alcohol consumption or illicit drug use. A year prior to this visit, she had presented to another hospital with similar complaints. The workup at that time demonstrated pericardial effusion, and the cytology of the pericardial fluid was suggestive of exudative effusion. At that visit, she was diagnosed with latent tuberculosis after a positive tuberculin skin test and received treatment with isoniazid for 6 months. However, her symptoms kept worsening over 6 months, and she presented to our hospital with severe shortness of breath. She gave a history of dyspnea on exertion, occasional squeezing non-radiating chest pain and palpitations. The review of systems was notable for anorexia and significant weight loss over the last six months. At the time of the presentation, she was in significant respiratory distress. The systemic exam was significant for jugular venous distention, bilateral pitting edema of lower extremities, pallor, and use of accessory muscles of respiration. The cardiovascular exam was significant for soft first heart sound and non-palpable apex. The rest of the systemic examination was unremarkable.
Labs at presentation were remarkable for normochromic normocytic anaemia (Hb-11.4 gm/dL) only. The comprehensive metabolic panel, troponin-I and B-natriuretic peptide were normal at presentation. The bedside echocardiogram revealed an ejection fraction of 55%-60%, normal valvular structures, and a moderate, free-flowing, concentric pericardial effusion without tamponade physiology. CT scan of the chest revealed pericardial effusion with a possible pericardial sac mass and multiple bilateral hemorrhagic pulmonary nodules. Cardiac CT showed a highly vascular, right atrial wall soft tissue mass involving the adjacent pericardium associated with large pericardial effusion (Figure 1). CT guided biopsy of the mass showed a tumour with spindle and epithelioid cells, prominent nucleoli and irregular vascular channels (Figure 2). CD31 and CD34 stains were positive in the tumour cells and highlighted the vasculature. The tumour cells were negative for keratin AE1/AE3, EMA, BerEP4, CK7, TTF-1, calretinin, CK5/6, MART-1, HMB45 and S-100.
The patient had metastatic disease at the time of diagnosis. She was treated with AIM 75/10,000 regimen (doxorubicin 25 mg/m2 on day 1-3, ifosfamide 2,000 mg/m2 on day 1-5 with mesna and granulocyte colony-stimulating factor support). She completed six cycles of treatment with 25% dose reduction and minor treatment delays due to two episodes neutropenic fever. A partial response was noted after six cycles with at least 50% reduction in the tumour burden. However, within 6 weeks of completing treatment, she was diagnosed with progressive disease and opted for hospice.