Angiosarcoma of the heart. Unusual presentation and survival after treatment (original) (raw)
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Primary Cardiac Angiosarcoma with Rare Presenting Feature and Successful Surgical Treatment
Brazilian Journal of Cardiovascular Surgery
Primary angiosarcoma is a rare clinical entity, it's typically located within the right atrium and known to be rapidly fatal. A 37-year-old female was presented with a history of recurrent facial paralysis and left hemiparesis. A cranial mass was identified at cranial magnetic resonance imaging and she underwent neurosurgery operation. The immunohistochemical examination was determined as metastatic cardiac angiosarcoma. The tumor, as well as part of the right pericardium, were resected. A piece of bovine pericardium was used to reconstruct the right atrial wall. Tricuspid valve was reconstructed with ring annuloplasty. Due to resection of right coronary artery with the tumor, coronary bypass surgery was performed successfully. The patient is currently healthful without any recurrence and complaint 12 months after the diagnosis as followed up.
Cardiac angiosarcoma--a review
Revista portuguesa de cardiologia : orgão oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology, 2007
Based on a case of a patient with angiosarcoma (AS) of the right atrium with superior vena cava syndrome associated with urticaria and polyarthralgias, who died soon after surgery, the authors present a brief review of the subject of cardiac AS, an extremely rare pathology, usually diagnosed late due to its non-specific symptomatology. Several topics are discussed, including mechanisms of clinical manifestations caused by blood flow obstruction and valve dysfunction, local invasion with arrhythmias and pericardial effusion, embolic phenomena and constitutional symptoms. Imaging and histopathologic methods of diagnosis are considered, as well as references to cytogenetic analysis. Surgery is the first treatment choice, but heart AS are frequently not completely resectable and concomitant metastases at the time of surgery are common, both usually leading to a dismal prognosis. Chemotherapy, radiotherapy and even heart transplantation do not substantially improve the survival of these ...
Cardiac angiosarcoma: an unexpected diagnosis
Autopsy and Case Reports, 2014
Cardiac angiosarcoma is a rare entity. The incidence through autopsy findings ranges between 0.001% and 0.03%. The disease usually presents with non-specific symptoms, although asymptomatic cases are frequent; therefore, diagnosis is unexpected and consequently delayed. The authors report the case of a middle-aged man with a recent onset cough and dyspnea. He sought medical care several times without receiving a definite diagnosis until a plain chest radiography was taken showing a mediastinal enlargement, which was the reason why he was hospitalized for clinical investigation. During the diagnostic workup, an echodopplercardiogram and a thoracic computed tomography were performed, showing a heterogeneous soft-tissue mass infiltrating the pericardium and the anterior atrial wall. Multiple and scattered pulmonary nodules were also present. A pulmonary nodule was biopsied, which revealed an angiosarcoma. The clinical features added to the radiological and histological findings permitted the diagnosis of right atrial angiosarcoma. The authors highlight the unexpected pattern in the presentation of cardiac tumors.
Primary cardiac angiosarcoma: a fatal disease
Case reports in medicine, 2009
A 42-year-old man with a cardiac tamponade underwent an urgent pericardiotomy that showed tumoral tissue, covering the surface of the right atrium. The tumor was then partially excised, and the histological examination revealed the presence of a moderately-differentiated angiosarcoma. The patient was then referred to the oncology unit and scheduled for a chemotherapy schedule including Epirubicin (60 mg/m(2), on days 1 and 2) plus Ifosfamide (2000 mg/m(2), on days 1 to 3) and Uromitexan (2000 mg/m(2) at hours 0, 4, 8 after IFO). All drugs were administered every three weeks. After two cycles, a restaging work-up revealed a partial remission. The treatment was continued for another two cycles. A new evaluation by cardiac MRI evidenced a local and distant (lung) progression of disease. The patient died after three months. This paper confirms that cardiac angiosarcoma is a fatal disease, and the prognosis is usually 6-11 months from time of diagnosis.
Texas Heart Institute Journal, 2013
Primary cardiac tumors do not occur frequently, and only one quarter of them, chiefly sarcomas, are malignant. Patients with angiosarcoma typically have a shorter survival time than do patients with other sarcomas, and the prognosis for survival depends strictly on the stage of the disease at the time of diagnosis and the possibility of complete surgical excision. Chemotherapy and radiotherapy have well-established postoperative roles because of the high probability of metastasis. We report the case of a 25-year-old man who presented with pericardial effusion and echocardiographic evidence of an intracavitary right atrial mass but without the bulky, infiltrative growth typical of this location of the disease. Malignancy was suggested by the clinical presentation, the location of the mass in the right side of the heart, and the absence of conditions favoring thrombus formation. After complete surgical excision, the mass was confirmed to be an angiosarcoma. Conventional adjuvant chemo...
Cardiac Angiosarcoma-A Review [ 37 ]
Based on a case of a patient with angiosarcoma (AS) of the right atrium with superior vena cava syndrome associated with urticaria and polyarthralgias, who died soon after surgery, the authors present a brief review of the subject of cardiac AS, an extremely rare pathology, usually diagnosed late due to its non-specific symptomatology. Several topics are discussed, including mechanisms of clinical manifestations caused by blood flow obstruction and valve dysfunction, local invasion with arrhythmias and pericardial effusion, embolic phenomena and constitutional symptoms. Imaging and histopathologic methods of diagnosis are considered, as well as references to cytogenetic analysis. Surgery is the first treatment choice, but heart AS are frequently not completely resectable and concomitant metastases at the time of surgery are common, both usually leading to a dismal prognosis. Chemotherapy, radiotherapy and even heart transplantation do not substantially improve the survival of these patients. Urticaria is not generally assumed by most authors to be associated with malignancy, but there are rare reports of its association with some malignant tumors.
Primary cardiac angiosarcoma. A case report
Cardiology, 1995
A primary cardiac malignancy is a very rare diagnosis. The most prevalent malignant cardiac tumors are angiosarcomas. Autopsy specimens, microscopic slides, clinical and intraoperative data from a 72-year-old woman referred to our hospital with a suspected mediastinal tumor were studied. The patient was diagnosed with primary cardiac angiosarcoma by microscopic examination under surgery and underwent resection of the tumor, which compressed and obstructed the right atrium and ventricle. She was discharged from our hospital after 3 weeks and died of right heart failure 2 months after the operation.
A Rare Case Report of Left Atrial Angiosarcoma
The most primary cardiac tumors are benign, and malignant tumors comprise about 25%. Primary cardiac angiosarcoma is the most common malignant tumor and aggressive tumor with a high incidence of metastatic spread. The diagnosis is often delayed because of the nonspecific clinical presentation. Cardiac angiosarcoma usually arises from the right atrium. We report an extremely rare case of primary angiosarcoma originating from the left atrium in a 57-year-old woman that had been referred with a one month history of exertional dyspenea. Transesophageal echocardiography showed a large mass on the left atrial that protrude through mitral valve. The tumor was resected successfully with curative intent. The patient underwent adjuvant chemotherapy. We have no problem up to now in six months follow-up.