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Cureus
Primary cardiac angiosarcoma is a rare malignant neoplasm and occurs most frequently in middle-aged males. It has an aggressive nature, with highly variable clinical features, which results in delayed diagnosis and high mortality. We report a 19-year-old man presented to the ED with a three-month history of hemoptysis and one-week history of anterior chest pain. Additionally, an aortic diastolic murmur grade II/VI was found on physical examination. Thoracic CT scan revealed bilateral dispersed hypodense pulmonary nodes with peripheral halo, alveolar densification, and pericardial effusion. The transthoracic echocardiogram confirmed sizeable pericardial effusion and bicuspid aortic valve, without other significant findings. A pericardiocentesis removed 1300 mL of hemorrhagic fluid, consistent with an exudate without malignant cells. Both cardiac magnetic resonance and transesophageal echocardiogram revealed a large mass on the right atrium's anterior wall. Mass biopsy was performed, revealing malignant cardiac angiosarcoma. The biopsy of the lung lesions was compatible with lung metastasis of primary cardiac angiosarcoma. The patient was submitted to palliative chemotherapy but died 12 months after the 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.
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.
European Heart Journal: Case Reports, 2021
Background Malignant primary cardiac neoplasms are rare and primary cardiac angiosarcoma is the most common and aggressive subtype. It most commonly presents in middle-aged males and due to its non-specific clinical presentation, the diagnosis is often delayed until advanced disease is already present. Clinical presentation is determined by manifestations of local infiltration or metastatic disease and making an early diagnosis is extremely challenging. Case summary A 15-year-old previously healthy boy was admitted to the emergency department with a history of pathological weight loss and functional decline. The patient was found to have a left-sided pneumothorax as well as bilateral diffusely spread pulmonary nodules on plain chest radiograph. Computed tomography chest confirmed widespread pulmonary metastases and a right atrial filling defect. Echocardiography revealed a right atrial tumour and transvenous endomyocardial biopsy of the tumour was done under fluoroscopic and echocar...
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.
International Journal of Cardiology, 2013
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 ...
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.
The Heart Surgery Forum, 2013
Cardiac sarcomas are rare malignant tumors. Angiosarcoma is the most common cardiac sarcoma and is present in up to 33% of cases. Angiosarcomas have a poor prognosis, with a short survival expectancy. We report a case of a right atrial angiosarcoma treated by partial tumor resection followed by chemotherapy.
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.
2000
A 63-year-old lady presented with syncope and atypical chest pain. Conventional two-dimensional (2D) contrast echocardiogram showed a highly vascular mass compressing the right atrium without direct communication. A real-time three-dimensional echocardiogram (RT3DE) revealed its outer spatial relationship with the surrounding structures and its inner heterogeneity as well. A chest computed tomography revealed a solid cardiac mass (6 x 7 cm(2)) arising from the right atrium and multiple pulmonary nodules. Histopathology of the pericardial fluid confirmed angiosarcoma. To our knowledge, this is the first report of histology-confirmed primary cardiac angiosarcoma, which was completely evaluated by conventional 2D, contrast, and RT3DE.
World Journal of Surgical Oncology, 2007
Background: Primary cardiac neoplasms occur rarely and most of them are benign. Malignant tumors including angiosarcoma are extremely rare and have a non specific clinical presentation and a poor prognosis. Case presentation: We present a case of a young male who was transferred to our hospital because of shock and multiple organ failure after a complicated pericardial biopsy. During the previous seven months he presented with recurrent episodes of pericardial effusions and tamponade. Chest computed tomography revealed a mass in the right atrium, infiltrating the myocardium and pericardium. During emergency surgery that followed, the patient died because of uncontrolled hemorrhage. Autopsy revealed the mass of the right atrium, which was identified on histological examination as primary cardiac angiosarcoma. Conclusion: This case highlights the difficulties both in early diagnosis and in the management of patients with cardiac angiosarcoma.
Case Reports in Cardiology, 2016
Primary cardiac neoplasms are particularly unusual. Angiosarcoma is the most frequently seen histological subtype and is described by its infiltrating and damaging nature. Inappropriately, primary cardiac angiosarcoma is often missed as a preliminary diagnosis because of its scarcity. We present a 29-year-old previously healthy man with complete heart block and pericardial effusion who was finally diagnosed with angiosarcoma of the right atrium with extension to SVC and IVC.
Heart, 1984
A 49 year old man presented with severe cyanosis and dyspnoea on exercise. Clinical examination together with echocardiography, cardiac catheterisation, and angiography showed a balloting tumour in the right atrium, intermittently occluding the tricuspid ostium, and an atrial right to left shunt. At operation a pedunculated vascular tumour was found with a broad base which was embedded in the atrial wall and continued into the interventricular septum. Histological examination showed angiosarcomatous features and signs of a less than radical excision. The patient, who made an uneventful recovery, was given postoperative radiotherapy. After 36 months there are no signs of recurrence or metastasis.
International Journal of Cardiology, 2003
Annals of Diagnostic Pathology, 2011
Primary cardiac angiosarcoma is a rare but the most common malignant neoplasm of the heart in adults. The objective of this study is to analyze the clinicopathologic characteristics of primary cardiac angiosarcoma. Ten cases of primary cardiac angiosarcoma treated in a single institution were analyzed for their clinical, pathologic, and immunohistochemical features. There were 6 men and 4 women, with a mean age of 40 years (range, 20-61 years). The patients commonly presented with dyspnea and distant metastasis. All tumors were located in the right atrium, with a mean tumor size of 6.8 cm. Tumors were hemorrhagic, with variegated tan-brown solid areas. Histologically, they exhibited high-grade morphology with mixed solid growth and anatomizing channels. Frequent mitoses and tumor necrosis were common. The tumors were strongly positive for CD31, CD34, FLI-1, and WT-1 but negative for AE1/3, D2-40, human herpesvirus 8, and epidermal growth factor receptor. The tumor cells were focally reactive to p53, with a high rate of Ki-67 expression. A complete tumor resection was not possible in any of the patients because of the size or extensive local invasion of the tumor. Overall survival ranged from 1 to 81 months (mean, 26.6 months) after initial histologic diagnosis. Primary cardiac angiosarcomas are rare tumors that commonly arise in the right atrium. The mean age is much younger than that of soft tissue angiosarcoma. Regional tumor extension and distant metastasis are extremely common at the time of diagnosis. Surgical resection with adjuvant chemotherapy is currently the preferred treatment, and survival time appears to be inversely correlated with the tumor size and degree of regional tumor extension at the time of surgery.
The Heart Surgery Forum, 2002
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...
The Journal of Thoracic and Cardiovascular Surgery, 2002
International Journal of Medicine and Medical Research, 2019
Background. Primary tumors of the heart are rarely detected at autopsy, especially angiosarcomas which are primary malignant one. Objective. We presented autopsy case of cardic angiosarcoma with morphologic findings. Methods. We described adult man died in emergency service of the hospital. Results. Reported case was 33 year-old-man who was died in emergency service of hospital where he was taken when he was ill after leaving home. According the prosecution documents, and the expressions of family, it was reported that he had a heart disease; his symptoms repeated 3 day ago before he died, he thought to attend the Cardiology Clinic due to his symptoms. At autopsy on macroscopic internal examination, mass with rough surface in the right atrium, hematoma at the posterior of the right atrium, blood in the pericardia, nodular lesions in hemorrhagic appearance in the sections of lung, liver and spleen were detected. In histopathologic examination; in the heart angiosarcioma as primary ma...
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