Journal of Thoracic Oncology:
*CancerCare Manitoba; and †Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada.
Disclosure: The authors declare no conflicts of interest.
Address for correspondence: Derek Suderman, MD, 675 McDermot Avenue, Winnipeg, MB, Canada R3E 0V9. E-mail: Derek.Suderman@cancercare.mb.ca
A 58-year-old male shop keeper presented with asymptomatic hypochromic microcytic anemia in August 2007. He admitted to heavy alcohol consumption and 40 pack years of smoking. A year later, he presented to an emergency room with shortness of breath and tachycardia and was admitted to hospital. Electrocardiography was noncontributing. Further cardiac evaluation showed a pericardial effusion and an 8.8 cm × 6.4 cm × 6.6-cm cardiac mass immediately adjacent to the lower esophagus involving the pericardium, left atrium, inferior left ventricle, interatrial septum, circumflex coronary artery, and inferior vena cava (Figure 1). Ejection fraction was 49%.
A jugular transvenous biopsy demonstrated central necrosis with hyperchromatic and pleomorphic polygonal cells disposed singly and in loose aggregates (Figure 2). Immunostaining was positive for CD31 and CD34 and negative for S-100, human melanoma black-45, CD45, CD20, thyroid transcription factor-1, and calretinin consistent with primary cardiac angiosarcoma. The tumor was determined to be inoperable.
Based on the report of Nakamura-Horigome et al.,1 the patient was treated with concomitant chemoradiotherapy consisting of 3D conformal radiation and docetaxel at a dose of 25 mg/m2 weekly during radiation and up to 7 months after. Magnetic resonance imaging in December 2008 showed a partial response, which was unchanged 4 months later (Figure 3). Chemotherapy continued until June 2009. In September 2009, he developed shortness of breath and presented with a left pleural effusion. Cytology was negative. Imaging suggested no progression of his tumor, but he required a continuous pleural drainage catheter to remain at home. In March 2010, he was readmitted for worsening shortness of breath. He died 2 weeks later, 16 months after completing radiotherapy. His tumor mass was radiologically stable.
In adults, angiosarcoma is the most common type of cardiac sarcoma but is still rare and metastatic tumors to the heart occur far more often.2 Presentation is usually late, and most are unresectable.3 Treatment has been pursued in an ad hoc manner with surgery, chemotherapy, and radiotherapy, all playing roles without the benefit of clinical trials.4
A recent case report from Japan noted a durable response of 12 months using radiation with concomitant and then maintenance docetaxel. By using the same, we observed a partial response followed by progression-free survival for 16 months. The literature suggests survival in the range of 4 to 5 months for patients with extensive unresectable disease.5
The patient was dependent on chest tubes to drain pleural effusions for his last several months. The pleural effusions were not clearly related to his tumor. We were not able to ascertain to what degree his final symptoms were related to radiation-related damage to the lungs, heart, or pericardium. It is possible that chronic pericardial changes from radiation may have contributed, but radiation-induced pericardial changes would be obscured on computed tomography by pericardial tumor involvement and pleural effusion. By using chemoradiotherapy, we were able to obtain a documented partial response and a prolonged course of stable disease for 16 months including a return to work for 11 months. This regimen deserves further investigation.
1. Nakamura-Horigome M, Koyama J, Eizawa T, et al. Successful treatment of primary cardiac angiosarcoma with docetaxel and radiotherapy. Angiology 2008;59:368–371.
2. Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001;85:218–222.
3. Bakaeen FG, Reardon MJ, Coselli JS, et al. Surgical outcome in 85 patients with primary cardiac tumors. Am J Surg 2003;186:641–647.
4. Truong PT, Jones SO, Martens B, et al. Treatment and outcomes in adult patients with primary cardiac sarcoma: the British Columbia Cancer Agency experience. Ann Surg Oncol 2009;16:3358–3365.
5. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report. Cancer 1986;57:852–859.