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      Primary Cardiac Angiosarcoma in a Middle Aged Woman

      case-report

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          Abstract

          Introduction:

          Primary cardiac angiosarcoma is the most common primary sarcoma in adults between the 3rd and 4th decades of life. Nearly 90% of angiosarcomas occur in the right atrium, which is responsible for the late onset of symptoms.

          Case Presentation:

          We presented a 56-year-old woman admitted to our center with lung emboli symptoms. Transthoracic and transesophageal echocardiography (TTE and TEE) demonstrated very large size (more than 10 cm diameter) multilobulated mass with mobile particles extended from the right atrium to the right ventricle and the right ventricular outflow tract which destructed the right atrium (RA) wall and penetrated to the pericardial space.

          Conclusions:

          Unfortunately the tumor was unresectable and just an incisional biopsy was performed. She received chemotherapy as palliative care.

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          Cardiac tumours: diagnosis and management.

          Jagdish Butany, Vidhya Nair, Ather Naseemuddin … (2005)
          Primary cardiac tumours are rare, with an autopsy incidence ranging from 0.001% to 0.030%. Three-quarters of these tumours are benign and nearly half of the benign tumours are myxomas. Metastases to the heart are far more common than primary cardiac tumours. Primary cardiac tumours present with one or more of the symptoms of the classic triad of: cardiac symptoms and signs resulting from intracardiac obstruction; signs of systemic embolisation; and systemic or constitutional symptoms. They are diagnosed by use of transthoracic and transoesophageal echocardiograms, MRI, and CT scan. Whereas surgery is indicated in patients with benign tumours, systemic chemotherapy is indicated in those who have widespread or unresectable malignant disease, and chemotherapy and radiotherapy are usually combined in treatment of patients with primary cardiac lymphomas. The prognosis after surgery is usually excellent in the case of benign tumours but is unfortunately still limited in localised malignant diseases. Patients with sarcomas live for a mean of 3 months to 1 year, and those with lymphomas live up to 5 years if treated, but usually die within 1 month if untreated.
          Article 0 comments Cited 209 times – based on 0 reviews     Review now
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            Echocardiographic and pathologic characteristics of primary cardiac tumors: a study of 149 cases.

            Qingyi Meng, Hong Lai, João Lima … (2002)
            To investigate the characteristics and pathological features of primary cardiac tumors and to evaluate the diagnostic sensitivity of echocardiography in primary cardiac tumors, all pathologic and echocardiographic records at the Chinese PLA general hospital and its satellite hospitals between January 1st, 1990 and January 1st, 2000 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumors. A total of 149 patients who had complete echocardiographic records and who were diagnosed with primary cardiac tumors were included in the study. Pathologic and echocardiographic records were reviewed retrospectively to evaluate the presence, location and histologic type of the tumors. The majority (n=118, 79.2%) of cases had been diagnosed with benign tumors. Myxoma was the most common histologic type accounting for 50.0% of total cardiac tumors. Lipoma was the second most common type of benign tumor. Among cases with malignant tumors (n=31, 20.8%), unclassified sarcoma (n=7), angiosarcoma (n=6) and rhabdomyosarcoma (n=6) were the common histologic types of primary malignant tumor. Non-myxomatous benign tumors were more likely to have occurred in the ventricle than myxomas (17/43, 39.5% vs. 7/75, 9.3%; P=0.00). The proportion of pericardium involvement in the malignant tumors (8/31, 25.8%) was significantly higher than that in the myxomas (0/75, 0%; P=0.00) and non-myxomas (2/43, 4.7%; P=0.01). The diagnostic sensitivity of transthoracic and transesophageal echocardiography was 93.3% (139/149) and 96.8% (30/31), respectively. The study, using a relatively large sample, confirms that myxoma was the most common primary cardiac tumor. The locations of tumor involvement varied by types of tumor. Echocardiography may be a useful tool for early diagnosis of primary cardiac tumors.
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              Cardiac sarcomas: an update.

              L. Spagnoli, Mario Roselli, Amedeo Ferlosio … (2010)
              Primary cardiac sarcomas are rare and represent 20% of all primary cardiac tumors. Symptoms depend on the chambers and the cardiac structures involved. Transthoracic echocardiography is commonly used to identify a cardiac mass. The diagnosis of cardiac sarcoma requires adequate sampling and the careful use of ancillary diagnostic techniques. In the most recent histologic classification, angiosarcoma is the most common malignant tumor of the heart with recognizable differentiation. Undifferentiated sarcomas account for one-third of all cardiac sarcomas and have been incorporated in the malignant fibrous histiocytoma/pleomorphic sarcoma subgroup. Elective cardiac sarcoma therapy includes complete surgical excision when possible, followed by radio and chemotherapeutic regimen, the latter preferably containing anthracyclines, ifosfamide, or taxanes. Prognosis of cardiac sarcomas is very poor, with mean survival ranging from 9.6 to 16.5 months. A less-aggressive course seems related to the left atrium location, a low histologic grading with scarce cellular pleomorphism and low-mitotic activity, absence of necrosis, myxoid tumor appearance, and absence of metastasis at diagnosis.
              Article 0 comments Cited 37 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Iran Red Crescent Med J
                Journal ID (iso-abbrev): Iran Red Crescent Med J
                Journal ID (doi): 10.5812/ircmj
                Journal ID (publisher-id): Kowsar
                Title: Iranian Red Crescent Medical Journal
                Publisher: Kowsar
                ISSN (Print): 2074-1804
                ISSN (Electronic): 2074-1812
                Publication date (Electronic): 23 June 2015
                Publication date Collection: June 2015
                Volume: 17
                Issue: 6
                Electronic Location Identifier: e23067
                Affiliations
                [1 ]Department of Cardiology, Mazandaran University of Medical Sciences, Sari, IR Iran
                [2 ]Department of Pathology, Mazandaran University of Medical Sciences, Sari, IR Iran
                [3 ]Department of Cardiothoracic Surgery, Mazandaran University of Medical Sciences, Sari, IR Iran
                Author notes
                [* ]Corresponding Author: Rozita Jalalian, Department of Cardiology, Mazandaran University of Medical Sciences, Sari, IR Iran. Tel: +98-1512224002, Fax: +98-1512224002, E-mail: Rozita_jalalian@ 123456yahoo.com
                Article
                DOI: 10.5812/ircmj.23067v2
                PMC ID: 4553020
                SO-VID: 39f5b87c-2ee5-44d9-849e-2b94c99d58b9
                Copyright © Copyright © 2015, Iranian Red Crescent Medical Journal.
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

                History
                Date received : 26 August 2014
                Date revision received : 11 November 2014
                Date accepted : 15 March 2015
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Medicine
                Keywords: cardiac tumors sarcoma,angiosarcoma,echocardiography
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: cardiac tumors sarcoma, angiosarcoma, echocardiography

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