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      Sarcomas of the heart as a difficult interdisciplinary problem

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          Abstract

          Cardiac tumors are assumed to be a rare entity. Metastases to the heart are more frequent than primary lesions. Sarcomas make up the majority of cardiac malignant neoplasms. Among them angiosarcoma is the most common and associated with the worst prognosis. Malignant fibrous histiocytoma comprises the minority of cardiac sarcomas and has uncertain etiology as well as pathogenesis. Transthoracic echocardiography remains the widely available screening examination for the initial diagnosis of a cardiac tumor. The clinical presentation is non-specific and the diagnosis is established usually at an advanced stage of the disease. Sarcomas spread preferentially through blood due to their immature vessels without endothelial lining. Surgery remains the method of choice for treatment. Radicalness of the excision is still the most valuable prognostic factor. Adjuvant therapy is unlikely to be effective. The management of cardiac sarcomas must be individualized due to their rarity and significant differences in the course of disease.

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          Most cited references123

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          Phase II study of sorafenib in patients with metastatic or recurrent sarcomas.

          PURPOSE Since activity of sorafenib was observed in sarcoma patients in a phase I study, we performed a multicenter phase II study of daily oral sorafenib in patients with recurrent or metastatic sarcoma. PATIENTS AND METHODS We employed a multiarm study design, each representing a sarcoma subtype with its own Simon optimal two-stage design. In each arm, 12 patients who received 0 to 1 prior lines of therapy were treated (0 to 3 for angiosarcoma and malignant peripheral-nerve sheath tumor). If at least one Response Evaluation Criteria in Solid Tumors (RECIST) was observed, 25 further patients with that sarcoma subtype were accrued. Results Between October 2005 and November 2007, 145 patients were treated; 144 were eligible for toxicity and 122 for response. Median age was 55 years; female-male ratio was 1.8:1. The median number of cycles was 3. Five of 37 patients with angiosarcoma had a partial response (response rate, 14%). This was the only arm to meet the RECIST response rate primary end point. Median progression-free survival was 3.2 months; median overall survival was 14.3 months. Adverse events (typically dermatological) necessitated dose reduction for 61% of patients. Statistical modeling in this limited patient cohort indicated sorafenib toxicity was correlated inversely to patient height. There was no correlation between phosphorylated extracellular signal regulated kinase expression and response in six patients with angiosarcoma with paired pre- and post-therapy biopsies. CONCLUSION As a single agent, sorafenib has activity against angiosarcoma and minimal activity against other sarcomas. Further evaluation of sorafenib in these and possibly other sarcoma subtypes appears warranted, presumably in combination with cytotoxic or kinase-specific agents.
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            Metastases to the heart.

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              Malignant primary cardiac tumors: review of a single institution experience.

              Primary cardiac sarcomas are uncommon. The authors undertook to review the Mayo Clinic's experience with primary cardiac sarcomas consisting of 34 patients seen over a 32-year period. The patient database at the Mayo Clinic was searched to identify patients with malignant tumors of the heart seen during the 32-year period between 1975 and 2007. Thirty-four patients with primary cardiac sarcomas were identified and their medical records reviewed for details pertaining to presenting symptoms, staging modalities, treatment approaches, and outcomes. Of the 34 patients, 17 were men and 17 were women. The median age was 44 years. The mean duration of symptoms at the time of diagnosis was 3.6 months. The most common histologic type was angiosarcoma (41%). The median follow-up for the entire group was 12 months (range, 0-61 months). The median survival for those who underwent a complete surgical excision was 17 months compared with 6 months for those in whom a surgical complete remission could not be achieved (P = .01). Patients with angiosarcoma had a lower survival compared with patients with other histologies (5 months vs 17 months; P = .01). The median survival of patients with metastatic disease was 5 months versus 15 months in patients without metastatic disease (P = .03 by the log-rank test). Cardiac sarcomas remain a rare but lethal disease. Compared with extracardiac sarcomas, the prognosis for patients with cardiac sarcomas remains very poor. A complete surgical excision should be performed if possible. Innovative treatment strategies are required. (c) 2008 American Cancer Society.
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                Author and article information

                Journal
                Arch Med Sci
                Arch Med Sci
                AMS
                Archives of Medical Science : AMS
                Termedia Publishing House
                1734-1922
                1896-9151
                23 February 2014
                24 February 2014
                : 10
                : 1
                : 135-148
                Affiliations
                [1 ]Department of Cardiac Surgery, Chair of Cardiology and Cardiac Surgery, Medical University of Lodz, Poland
                [2 ]Military Teaching Hospital – Veterans Central Hospital, Lodz, Poland
                Author notes
                Corresponding author: Assoc. Prof. Stanisław Ostrowski MD, PhD, Department of Cardiac Surgery, Chair of Cardiology and Cardiac Surgery, Medical University of Lodz, Lodz, Poland. Phone/fax: +48 42 633 15 58. E-mail: stanislaw.ostrowski@ 123456umed.lodz.pl
                Article
                22292
                10.5114/aoms.2014.40741
                3953983
                2abc54fb-1301-4ccc-b460-14bef3998d7e
                Copyright © 2014 Termedia & Banach

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial 3.0 Unported License, permitting all non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 28 October 2010
                : 10 January 2011
                : 20 March 2011
                Categories
                State of the Art Paper

                Medicine
                cardiac tumors,angiosarcoma,transthoracic echocardiography,surgery
                Medicine
                cardiac tumors, angiosarcoma, transthoracic echocardiography, surgery

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