Lupus-like Syndrome and Cardiac Tamponade as Initial Presentation of a Primary Cardiac Malignant Tumor: Report of a Case

This is the case of a 38-year-old man with a history of two female relatives with Systemic Lupus Erythematosus and personal factors of high cardiovascular risk (gender, stress, smoking, chronic consumption of electronic cigarette). The patient presented tamponade due to pericardial effusion secondary to Lupus-like syndrome that preceded and accompanied at all times an angiosarcoma of the roof and anterior wall of the right atrium. His clinical evolution is described, as well as the surgical and pharmacological treatment in the Cardiovascular Care Unit of a third-level hospital and the possible causes of the poor response to management that led to his death.

A Case of Cardiac Angiosarcoma Successfully Treated with Postoperative Conformal Dynamic Arc Radiotherapy / 日本心臓血管外科学会雑誌

Cardiac angiosarcoma is a rare heart malignancy. The prognosis is reported to be very poor. Here, we report a case of cardiac angiosarcoma which was treated by postoperative conformal dynamic arc radiotherapy. The patient has been in good health with no recurrence for 18 months after surgery. The patient was a 71-year-old woman, who presented edema and general malaise. Echocardiography and computed tomography revealed a right atrial mass and massive pericardial effusion, which was thought to be the cause of tamponade. Intraoperatively, we found a large tumor arising from right atrial wall spreading and invading to the inferior vena cava, diaphragm, and right pericardium. We abandoned complete resection of the tumor. We only resected the part of the tumor under cardiopulmonary bypass. The subsequent defect of the right atrial wall was reconstructed with bovine pericardial patch. The pathological diagnosis was consistent with angiosarcoma, and the margin was positive for the tumor. In addition to reduction surgery, we adopted radiotherapy (conformal dynamic arc radiotherapy, 10MV-X-ray, 54 Gy/18 Fr/4.5 week, fractionated radiotherapy). The CT, one month after the radiation, showed a significant reduction in tumor size. Moreover, no tumor could be pointed out by echocardiography 18 months after surgery. Advances in technology and methodology has made tumor control possible without significant side effects. We conclude that reduction surgery accompanied with postoperative radiotherapy is promising in maintaining quality of life and in improving life expectancy.

A Case of Cardiac Angiosarcoma with Superior Vena Cava Syndrome / 日本心臓血管外科学会雑誌

A 31-year-old woman was admitted to our hospital with a sudden onset of chest pain and dyspnea. Echocardiography, chest CT, and chest MRI revealed a huge mass in the right atrium. She underwent pericardial drainage to alleviate cardiac tamponade. Emergency surgery was performed because of superior vena cava syndrome and the risk of tricuspid valve obstruction by the mass. The tumor was resected en bloc, including the right atrial wall and a large segment of the proximal superior vena cava. The right atrium was then reconstructed with a Xenomedica patch and the superior vena cava was reconstructed using an expanded polytetrafluoroethylene (ePTFE) vascular graft. The pathological diagnosis was haemangiosarcoma. Cardiac angiosarcoma is a rare tumor, and its prognosis is very poor. The patient could survive for about 5 months after surgical resection.

Echocardiographic Diagnosis of Right Atrial Perforation due to Primary Cardiac Angiosarcoma Using Hand-Agitated Saline Contrast

Primary cardiac angiosarcoma is a rare and rapidly progressing tumor characterized by high predilection for right-sided heart involvement and a very poor prognosis. In this report, we present a case of angiosarcoma involving right atrium and ventricle in 61-year old woman with clinical presentation of repeated bloody pericardial effusion. Right atrial perforation was confirmed by contrast echocardiography using hand-agitated saline contrast. Primary cardiac angiosarcoma was confirmed by histological and immunohistochemical studies.

Abnormal Blood Flow Between a Pericardial Mass and Right Atrium Detected by Echocardiography in a Case of a Ruptured Angiosarcoma of the Right Atrium

Spontaneous rupture of a primary cardiac angiosarcoma is a rare condition with a poor prognosis. The authors describe the case of a 48-year-old man with abnormal blood flow from a pericardial mass to the right atrium 3 months after pericardiocentesis. The flow was presumed to have occurred due to rupture of this cardiac angiosarcoma of the right atrial wall and pericardium into the right atrial cavity.

Abnormal Blood Flow Between a Pericardial Mass and Right Atrium Detected by Echocardiography in a Case of a Ruptured Angiosarcoma of the Right Atrium

Spontaneous rupture of a primary cardiac angiosarcoma is a rare condition with a poor prognosis. The authors describe the case of a 48-year-old man with abnormal blood flow from a pericardial mass to the right atrium 3 months after pericardiocentesis. The flow was presumed to have occurred due to rupture of this cardiac angiosarcoma of the right atrial wall and pericardium into the right atrial cavity.

A Case of Primary Right Atrial Angiosarcoma Manifested with Cardiac Tamponade

Primary cardiac malignancy is very rare. Angiosarcoma is the most frequent malignant cardiac tumor and associated with a very unfavourable outcome. We report the case of an cardiac angiosarcoma complicated with cardiac tamponade revealed by echocardiography with pericardiocentesis and confirmed histopathologically in a 25 years old man.

A Primary Cardiac Angiosarcoma

Primary tumors of the heart are rare and the most are benign. Malignant tumors constitute less than 25% of primary cardiac tumors and angiosarcomas are the most commonly reported histologic type. At least 160 cases have been reported in the world, but no previous report in Korea. We reported a case of primary cardiac angiosarcoma located in right atrium.