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      Primary Intimal Sarcoma of the Left Atrium: An Incidental Finding on Routine Echocardiography

      case-report

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          Abstract

          Cardiac sarcomas are extremely rare primary malignant tumors of the heart. In this article, we present the case of a 70-year-old female, who was found to have a left atrial mass during a routine outpatient transthoracic echocardiography. Further investigation with cardiac magnetic resonance imaging confirmed the presence of a bilobulated mass with heterogeneous enhancement. Left atrial myxoma was the first diagnostic consideration, followed by other primary cardiac tumors, and thrombus. The patient subsequently underwent resection of the mass, utilizing cardiopulmonary bypass. Upon pathological examination, the mass was found to be an intimal sarcoma. The objective of this report is to describe a case of this rare disease entity, and to discuss its presentation, pathological findings and management.

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

            K Y Lam, P Dickens, A Chan (1993)
            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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              Primary sarcomas of the heart.

              Tricia A. Burke, Jack Cowan, R Virmani (1992)
              Seventy-five primary sarcomas of the heart were classified by histologic appearance as angiosarcoma (26 cases), undifferentiated sarcoma (18 cases), osteosarcoma (9 cases), fibrosarcoma (6 cases), malignant fibrous histiocytoma (6 cases), leiomyosarcoma (4 cases), myxosarcoma (3 cases), synovial sarcoma (2 cases), and neurofibrosarcoma (1 case). The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy. Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate. Immunostaining with commercially available antisera was useful in the diagnosis of sarcoma but not in subclassification of 19 tumors so tested. Although the prognosis for patients with cardiac sarcomas is dismal, histologic grading is useful in predicting outcome, as has been shown for soft tissue sarcomas of other sites.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Rare Tumors
                Journal ID (iso-abbrev): Rare Tumors
                Journal ID (publisher-id): RT
                Title: Rare Tumors
                Publisher: PAGEPress Publications, Pavia, Italy
                ISSN (Print): 2036-3605
                ISSN (Electronic): 2036-3613
                Publication date (Electronic): 17 November 2016
                Publication date Collection: 17 November 2016
                Volume: 8
                Issue: 4
                Electronic Location Identifier: 6389
                Affiliations
                [1 ]Department of Medicine, Staten Island University Hospital , Staten Island, NY, USA
                [2 ]Department of Cardiology, Staten Island University Hospital , Staten Island, NY, USA
                [3 ]Department of Pathology, Staten Island University Hospital , Staten Island, NY, USA
                [4 ]Department of Hematology-Oncology, Staten Island University Hospital , Staten Island, NY, USA
                Author notes
                Department of Medicine, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA. +1.718.226.9506 - +1.718.226.8695. gvalecha@ 123456northwell.edu

                Contributions: GV, wrote abstract, discussion and conclusion, coordinated the writing, corresponding author; DP, wrote case; NN, contributed by writing case with Dr. Pau; YL, provided with figures and wrote the histopathology part of discussion; FM, literature review and contributed to discussion; JPA, principal investigator, contributed to the writing, supervised the writing of entire manuscript.

                Conflict of interest: the authors declare no potential conflict of interest.

                Article
                DOI: 10.4081/rt.2016.6389
                PMC ID: 5136772
                PubMed ID: 27994830
                SO-VID: 75be58c5-a111-4a2f-8329-46202c483bbb
                Copyright © ©Copyright G. Valecha et al.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 02 January 2016
                Date revision received : 31 March 2016
                Date accepted : 12 April 2016
                Page count
                Figures: 5, Tables: 0, Equations: 0, References: 19, Pages: 4
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: intimal sarcoma,mesenchymal tumor,rare,incidental,aggressive
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: intimal sarcoma, mesenchymal tumor, rare, incidental, aggressive

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