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      Calcified amorphous tumor of the heart with mitral annular calcification: a case report

      case-report

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          Abstract

          Background

          Calcified amorphous tumor of the heart is a rare, non-neoplastic cardiac mass characterized by nodular calcium in the background of amorphous degenerating fibrinous material. Clinical diagnosis of calcified amorphous tumor can be difficult, and current single imaging techniques do not specifically differentiate calcified amorphous tumor from other cardiac tumors such as calcified atrial myxoma, calcified thrombi, or vegetation. Complete surgical resection is the treatment of choice for both symptom improvement and prevention of embolization, as well as for pathological diagnosis.

          Case presentation

          A 70-year-old Asian man with end-stage renal disease complained of chest discomfort during exercise. He had no history of thromboembolism or endocarditis. A transthoracic echocardiogram revealed mitral annular calcification as well as a highly mobile mass (8 × 6 mm) attached to the ventricular side of the posterior mitral valve leaflet. As the mass was highly mobile, suggesting a high risk of embolization, he underwent surgical resection. A histopathological examination revealed multiple nodular amorphous calcifications, along with fibrous connective tissue. There were no identifiable myxoma or malignancy cells. Consequently, the diagnosis of calcified amorphous tumor was confirmed.

          Conclusions

          In the present case, a calcified amorphous tumor arose from mitral annular calcification. A characteristic of mitral annular calcification-related calcified amorphous tumor is its highly mobile nature, with a high risk of stroke or other systemic embolism. Therefore, surgical therapy should be considered for treatment of calcified amorphous tumors.

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          Most cited references9

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          Mitral annular calcification and the risk of stroke in an elderly cohort.

          Previous clinical studies have suggested that there is an association between mitral annular calcification and the risk of stroke, but it is unclear whether this association is independent of the traditional risk factors for stroke. We examined the relation between mitral annular calcification and the incidence of stroke in a population-based study. Subjects in the Framingham Study receiving a routine examination underwent M-mode echocardiography to determine the presence and severity (thickness in millimeters) of mitral annular calcification. The incidence of stroke during eight years of follow-up was analyzed with a proportional-hazards model adjusting for the calcification, age, sex, systolic blood pressure, diabetes mellitus, cigarette smoking, atrial fibrillation, and coronary heart disease or congestive heart failure. Among 1159 subjects whose echocardiograms could be assessed for mitral annular calcification and who had no history or current evidence of stroke at the index examination (51 percent of all subjects), the prevalence of mitral annular calcification was 10.3 percent in the men and 15.8 percent in the women. Multivariate analysis demonstrated that the presence of mitral annular calcification was associated with a relative risk of stroke of 2.10 (95 percent confidence interval, 1.24 to 3.57; P = 0.006). There was a continuous relation between the incidence of stroke and the severity of mitral annular calcification; each millimeter of thickening as shown on the echocardiogram represented a relative risk of stroke of 1.24 (95 percent confidence interval, 1.12 to 1.37; P less than 0.001). Furthermore, even when subjects with coronary heart disease or congestive heart failure were excluded from the analysis, subjects with mitral annular calcification still had twice the risk of stroke. In an elderly, longitudinally followed population-based cohort, mitral annular calcification was associated with a doubled risk of stroke, independently of traditional risk factors for stroke. Whether such calcification contributes causally to the risk of stroke or is merely a marker of increased risk because of its association with other precursors of stroke remains unknown.
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            Calcified amorphous tumor of the heart (cardiac CAT).

            Eleven cases of poorly characterized nonneoplastic endocardially based intracavitary cardiac masses have been seen at the Mayo Clinic between 1965 and 1994. They occurred in 7 women and 4 men from age 16 to 75 years (mean, 52 years). The patients presented with a wide variety of symptoms and underlying diseases. The lesions were diagnosed as a primary cardiac neoplasm in 6 of 8 patients having echocardiography. Surgical excision was the treatment of choice in 10 patients; the 11th patient died of noncardiac causes 30 days after the mass was discovered. Grossly, the lesions were firm, yellow-white, and partially calcified, and arose in any of the four chambers. Microscopically, all lesions were characterized by nodular calcium in a background of degenerating blood elements and chronic inflammation. All patients had a benign course relative to their cardiac lesion. Repeat echocardiogram in two patients showed residual calcium in the region of the initial tumor. Although these lesions may represent calcified thrombi, the clinical presentation did not suggest thrombosis as the most likely explanation for their occurrence. Based on the combined clinical presentation and microscopic appearance, the authors propose the descriptive name calcified amorphous tumors (cardiac CAT) to describe this group to nonneoplastic cardiac masses.
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              Cardiac swinging calcified amorphous tumors in end-stage renal failure patients.

              We recently encountered 2 patients with mobile cardiac calcified amorphous tumors who were successfully treated by surgery. Both patients had mitral annular calcification and were on hemodialysis. These tumors showed swinging motion on echocardiography and they grew rapidly. Intraoperatively, the tumors were found to be fragile and they easily detached from their origin. The histologic findings were thrombus with angiogenesis, fibrin, and calcium deposition. This rapid-growing mobile tumor in end-stage renal failure patients is speculated to increase the risk of embolic events and should be included as a special entity of cardiac amorphous tumors. Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Contributors
                r_nakamaru@watanabe-hsp.or.jp
                81-6-6341-8651 , hirokioe@gmail.com
                iwakura@mac.com
                tmasai@watanabe-hsp.or.jp
                fujiken@watanabe-hsp.or.jp
                Journal
                J Med Case Rep
                J Med Case Rep
                Journal of Medical Case Reports
                BioMed Central (London )
                1752-1947
                18 July 2017
                18 July 2017
                2017
                : 11
                : 195
                Affiliations
                ISNI 0000 0004 0409 6927, GRID grid.416720.6, Department of Cardiology, , Sakurabashi Watanabe Hospital, ; 2-4-32, Umeda, Kita-ku, Osaka, 5300001 Japan
                Article
                1337
                10.1186/s13256-017-1337-9
                5516357
                b7ca1cd5-0439-4c0c-8705-3f9080dcb43c
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 3 March 2017
                : 30 May 2017
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2017

                Medicine
                calcified amorphous tumor,mitral annular calcification,end-stage renal disease
                Medicine
                calcified amorphous tumor, mitral annular calcification, end-stage renal disease

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