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Among the sarcoma group&#44; the median survival in those with negative surgical margins was 42&#46;2 months&#44; whereas in patients with partial resection and&#47;or positive margins it was 14&#46;1 months&#46; At the end of follow-up 10 patients had died and two were alive&#58; one with lymphoma &#40;206 months&#41; and the one with epithelioid hemangioendothelioma &#40;92 months&#41;&#46; The results were very good and similar to other recent data&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">2</span></a> In agreement with the authors&#44; we conclude that even partial palliative debulking surgery improves the quality of life of these patients and that the establishment of a multicenter database could enhance knowledge and surgical management of these diseases&#46; Between January 1990 and December 2015&#44; we operated on four patients with a primary malignant cardiac tumor&#44; all of them sarcomas&#46; There were three male and one female patients with a mean age of 41&#46;75 years &#40;19-68&#41;&#46; The location of the tumor was in the left atrium &#40;LA&#41; for the first three cases and in the right atrium &#40;RA&#41; for the last&#46; The first three patients with an infiltrating LA tumor &#40;undifferentiated sarcoma&#44; leiomyosarcoma and lipomyxosarcoma&#41; were resected via sternotomy incision and under cardiopulmonary bypass&#46; Our youngest patient&#44; a 19-year-old man with an infiltrating RA intimal sarcoma&#44; was considered unresectable and underwent a biopsy through a right thoracotomy&#46; The four patients were operated by the same surgeon &#40;CA&#41;&#46; Postoperative chemotherapy was prescribed in all the patients and all four died as a consequence of local tumor recurrence and&#47;or metastasis&#46; The first three cases died at a mean of 19&#46;6 months postoperatively &#40;15&#44; 11 and 33 months&#44; respectively&#41; and the fourth seven months after the biopsy &#40;overall mean survival 16&#46;5 months&#41;&#46; On the basis of the small number of reported surgical series&#44; the limited experience of a single surgeon or institution and the complexity of the surgery&#44; we consider that these patients should be referred to a specialized center with an experienced multidisciplinary heart tumor team and no more than 1 or 2 operating surgeons&#46; Radical&#44; extensive and aggressive surgical resection in association with chemotherapy is the mainstay of treatment&#46; Complete excision of the tumor with negative margins leads to a better prognosis and survival&#46; The operation may include reconstruction of the atrial and ventricular walls and&#47;or septum with pericardial or synthetic grafts&#44; valve surgery&#44; coronary bypass&#44; a Fontan-type procedure&#44; partial replacement of the ascending aorta and&#47;or the pulmonary artery&#44; associated lung resection&#44; autotransplantation&#44; mechanical circulatory assistance or replacement&#44; delayed heart transplantation and other surgical strategies&#46; Cardiac transplantation as a first alternative is not contemplated by the majority of heart teams due to concerns about recurrence and the possibility that postoperative immunosuppressive therapy may stimulate further tumor growth or a new neoplasm&#46; There is no general consensus or guidelines on the treatment of patients with primary cardiac sarcoma and other type of primitive malignant heart tumors&#44; and the surgical management of these patients is not well defined&#46; An aggressive&#44; multidisciplinary and multimodality approach with complex&#44; highly specialized and demanding surgery markedly improves survival&#46; Taking into consideration the adverse outcome and dismal prognosis of these diseases&#44; in agreement with Ramlawi et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">2</span></a> we also consider that new approaches and innovative treatment strategies are required&#46;</p><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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Letter to the Editor
Surgery of primary malignant cardiac tumors
Cirurgia dos tumores cardíacos primários malignos
Cipriano Abad
Corresponding author
cprnabad2@gmail.com

Corresponding author.
, José Angel López-Ruiz, Stefano Urso, Juan José Feijoo-Osorio
Hospital Universitario de Gran Canaria Dr Negrin, Service of Cardiac Surgery, Las Palmas de Gran Canaria, Spain
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            "entidad" => "Hospital Universitario de Gran Canaria Dr Negrin&#44; Service of Cardiac Surgery&#44; Las Palmas de Gran Canaria&#44; Spain"
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        "titulo" => "Cirurgia dos tumores card&#237;acos prim&#225;rios malignos"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We read with interest the recent article by Saraiva et al&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">1</span></a> of the Cardiothoracic Surgery Center&#44; University Medical Center&#44; Coimbra&#44; Portugal&#44; under the direction of Dr MJ Antunes&#46; It is an excellent study on 12 patients treated by surgical excision of a primary malignant cardiac tumor &#40;eight sarcomas&#44; three lymphomas and one epithelioid hemangioendothelioma&#41; in a 20-year period&#46; Complete resection &#40;negative margins&#41; was obtained in five cases and partial resection in seven &#40;five sarcomas and two lymphomas&#41;&#46; The operative mortality was zero&#46; Adjuvant therapy was administered in 11 patients&#46; Mean follow-up was 41&#46;7 months&#58; 24&#46;8 months for sarcomas&#44; 70&#46;1 months for lymphomas and 91&#46;9 months for the epithelioid hemangioendothelioma&#46; Among the sarcoma group&#44; the median survival in those with negative surgical margins was 42&#46;2 months&#44; whereas in patients with partial resection and&#47;or positive margins it was 14&#46;1 months&#46; At the end of follow-up 10 patients had died and two were alive&#58; one with lymphoma &#40;206 months&#41; and the one with epithelioid hemangioendothelioma &#40;92 months&#41;&#46; The results were very good and similar to other recent data&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">2</span></a> In agreement with the authors&#44; we conclude that even partial palliative debulking surgery improves the quality of life of these patients and that the establishment of a multicenter database could enhance knowledge and surgical management of these diseases&#46; Between January 1990 and December 2015&#44; we operated on four patients with a primary malignant cardiac tumor&#44; all of them sarcomas&#46; There were three male and one female patients with a mean age of 41&#46;75 years &#40;19-68&#41;&#46; The location of the tumor was in the left atrium &#40;LA&#41; for the first three cases and in the right atrium &#40;RA&#41; for the last&#46; The first three patients with an infiltrating LA tumor &#40;undifferentiated sarcoma&#44; leiomyosarcoma and lipomyxosarcoma&#41; were resected via sternotomy incision and under cardiopulmonary bypass&#46; Our youngest patient&#44; a 19-year-old man with an infiltrating RA intimal sarcoma&#44; was considered unresectable and underwent a biopsy through a right thoracotomy&#46; The four patients were operated by the same surgeon &#40;CA&#41;&#46; Postoperative chemotherapy was prescribed in all the patients and all four died as a consequence of local tumor recurrence and&#47;or metastasis&#46; The first three cases died at a mean of 19&#46;6 months postoperatively &#40;15&#44; 11 and 33 months&#44; respectively&#41; and the fourth seven months after the biopsy &#40;overall mean survival 16&#46;5 months&#41;&#46; On the basis of the small number of reported surgical series&#44; the limited experience of a single surgeon or institution and the complexity of the surgery&#44; we consider that these patients should be referred to a specialized center with an experienced multidisciplinary heart tumor team and no more than 1 or 2 operating surgeons&#46; Radical&#44; extensive and aggressive surgical resection in association with chemotherapy is the mainstay of treatment&#46; Complete excision of the tumor with negative margins leads to a better prognosis and survival&#46; The operation may include reconstruction of the atrial and ventricular walls and&#47;or septum with pericardial or synthetic grafts&#44; valve surgery&#44; coronary bypass&#44; a Fontan-type procedure&#44; partial replacement of the ascending aorta and&#47;or the pulmonary artery&#44; associated lung resection&#44; autotransplantation&#44; mechanical circulatory assistance or replacement&#44; delayed heart transplantation and other surgical strategies&#46; Cardiac transplantation as a first alternative is not contemplated by the majority of heart teams due to concerns about recurrence and the possibility that postoperative immunosuppressive therapy may stimulate further tumor growth or a new neoplasm&#46; There is no general consensus or guidelines on the treatment of patients with primary cardiac sarcoma and other type of primitive malignant heart tumors&#44; and the surgical management of these patients is not well defined&#46; An aggressive&#44; multidisciplinary and multimodality approach with complex&#44; highly specialized and demanding surgery markedly improves survival&#46; Taking into consideration the adverse outcome and dismal prognosis of these diseases&#44; in agreement with Ramlawi et al&#46;&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">2</span></a> we also consider that new approaches and innovative treatment strategies are required&#46;</p><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0025" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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ISSN: 21742049
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Revista Portuguesa de Cardiologia (English edition)
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