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      Primary intimal sarcoma of the left atrium presenting with constitutional symptoms

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          Abstract

          Intimal (spindle-cell) sarcomas are exceptionally rare and are highly aggressive cardiac tumors. The authors describe a case of a 43-year-old female, presenting with a 3-month history of constitutional symptoms with fever, night sweats, anorexia and weight loss, associated with productive cough and pleural effusion that was admitted with clinical suspicion of pulmonary tuberculosis. The patient developed sudden acute heart failure symptoms during hospitalization, leading to mechanical ventilation. Computed tomography scan with contrast showed a cardiac tumor filling the left atrium causing compression of pulmonary veins. Surgical resection was performed and histologic examination revealed an intimal sarcoma. Although commenced on adjuvant chemotherapy, local tumor recurrence occurred with pericardium invasion. The patient died within 4 months of initial diagnosis. This report aims to describe an unusual presentation of this rare disease entity, and to discuss its highly aggressive clinical course.

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          Most cited references10

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          Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies.

          Cardiac involvement by primary and secondary tumors is one of the least investigated subjects in oncology. Seven cases of primary and 154 cases of secondary cardiac tumors from autopsies performed over a 20-year period (1972 through 1991) at Queen Mary Hospital, Hong Kong, were reviewed. During this period, 12,485 autopsies were performed, and the autopsy incidence for primary and secondary heart tumors is thus 0.056% and 1.23%, respectively. Only seven primary cardiac tumors were found, including two myxomas, two rhabdomyomas, two hemangiomas, and one lipoma. For secondary tumors involving the heart (including both metastasis and local extension), important primary tumors in male subjects were carcinoma of the lung (31.7%), esophageal carcinoma (28.7%), lymphoma (11.9%), carcinoma of the liver (6.9%), leukemia (4.0%), and gastric carcinoma (4.0%), while in female subjects, carcinoma of the lung (35.9%), lymphoma (17.0%), carcinoma of the breast (7.5%), and pancreatic carcinoma (7.5%) predominated. Overall, the three most common malignant neoplasms encountered were carcinoma of the lung, esophageal carcinoma, and lymphoma. Pericardium, including epicardium, was the most common location of cardiac involvement by secondary tumors, followed by myocardium and endocardium. The present study showed a higher percentage of esophageal carcinoma and carcinoma of the liver (reflecting the higher incidence of these tumors in Hong Kong Chinese), but a lower incidence of carcinoma of the breast when compared with other series. The metastatic lung tumors showed an unusual predominance of adenocarcinoma.
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            Clinical classification of cardiovascular tumors and tumor-like lesions, and its incidences

            Tumors of the heart and the great vessels are very rare disease, and there are many disorders such as tumors originated from the heart and great vessels, metastatic tumors, and tumor-like lesions which do not fit into the usual concept of tumor or neoplasm; thus, it is very difficult to classify these tumors. We proposed a new classification of cardiovascular tumors for clinical use based on the accumulated biological analyses and clinical data of the reported literatures and our own study as benign tumors, malignant tumors, ectopic hyperplasia/ectopic tumors/others, and tumors of great vessels, with reference to the series of Atlas of tumor pathology of the Armed Forces Institute of Pathology and the recent World Health Organization classification of cardiac tumors issued in 2004. More than 50 disorders have been reported as tumors originated from the cardiovascular system, and various metastatic tumors from nearby organs, distant lesions, and intravascular extension tumors to the heart were reported. Based on the new classification, we reviewed epidemiology and incidence of cardiovascular tumors. Metastatic tumors are more frequent than tumors originated from the heart and great vessels, and cardiac myxoma is the most frequent tumors in all cardiac tumors.
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              Malignant tumors of the heart.

              Primary malignant cardiac tumors are rare, and mostly manifest as sarcomas in various types. As non-invasive diagnostic modalities, e.g. echocardiography and magnetic resonance imaging, have become more sensitive, there is a marked increase in the number of patients diagnosed. Nevertheless, most patients die within one year of initial diagnosis, either because of the often asymptomatic presentation of cardiac tumors until advanced disease, or a low index of suspicion on the part of the physician. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumor's anatomic location. Cardiac sarcomas may present with a variety of symptoms and are known to be great mimickers. A quick diagnosis facilitates the initiation of a proper treatment (surgical resection, adjuvant chemotherapy), which may in turn improve the prognosis. Metastases to the heart are far more common, unfortunately, clinical manifestations are mainly dominated by generalized tumor spread. The article summarizes epidemiology, symptoms, diagnostic modalities, and possible treatment options.
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                Author and article information

                Journal
                Oxf Med Case Reports
                Oxf Med Case Reports
                omcr
                Oxford Medical Case Reports
                Oxford University Press
                2053-8855
                July 2017
                05 July 2017
                05 July 2017
                : 2017
                : 7
                : omx031
                Affiliations
                [1 ]Departamento de Medicina, Medicina 1, Unidade Local de Saúde do Alto Minho, Hospital SantaLuzia, Viana do Castelo,Portugal
                [2 ]Departamento de Anatomia Patológica, Centro Hospitalar de S.João, Porto, Portugal
                Author notes
                [* ]Correspondence address. Travessa Dr. Barros, nr 183; 1° andar, 4465-038 Matosinhos, Porto, Portugal. Tel: +351 258 802 100. E-mail: editemarquesmendes@ 123456gmail.com
                Article
                omx031
                10.1093/omcr/omx031
                5497514
                6fe4faa7-a14f-4e1a-a820-c8b87b2c78d9
                © The Author 2017. Published by Oxford University Press.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 07 March 2017
                : 06 April 2017
                : 25 April 2017
                Page count
                Pages: 4
                Categories
                Case Report

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