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Primary Malignant Tumors of the Heart

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Cardiac Tumor Pathology

Part of the book series: Current Clinical Pathology ((CCPATH))

Abstract

The rate of malignant cardiac tumors is often wrongly reported as up to 30 %, when based on the data derived from pathology tertiary centers, where the most difficult cases are sent for expert opinion. Instead, primary malignant cardiac tumors are very rare representing nearly 10 % of all primary cardiac neoplasms. Sarcomas represent 95 % of these tumors, angiosarcoma, leiomyosarcoma, and unclassified sarcoma being the most common, followed by primary lymphoma and mesothelioma. All varieties of soft tissue sarcomas have been reported to arise from cardiac tissue.

There is no TNM classification for primary malignant cardiac tumors, and, due to their low frequency, there is no specific grading scheme for sarcomas. Thus, the general criteria proposed for grading malignant tumors of the soft tissues are utilized also for the cardiac one. According to the grading system for soft tissue sarcomas. a histological score from 1 to 3 is assigned to each of three parameters, i.e., tumor differentiation, mitotic rate, and amount of necrosis in untreated primary soft tissue sarcomas. The final grade is obtained by adding the three scores.

Primary cardiac sarcomas can occur at any age but are more frequently diagnosed between the third and fifth decades, and equally in men and women, whereas they are extremely rare in pediatric age. Surgical excision is the most effective treatment for primary cardiac malignancies. However, prognosis is very poor in spite of additional treatments, such as radiotherapy and chemotherapy, with a median survival less than 1 year and 80 % of patients already presenting diffuse infiltration of the heart and metastases at the time of diagnosis. 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, and absence of metastasis at diagnosis.

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Authors and Affiliations

  1. Pathological Anatomy, Department of Cardiac, Thoracic and Vascular Sciences, Azienda Ospedaliera-University of Padua Medical School, via A. Gabelli, 61, Padua, 35121, Italy

    Marialuisa Valente M.D., Stefania Rizzo M.D. & Cristina Basso M.D., Ph.D.

  2. Department of Pathology, S. Orsola-Malpighi Hospital, Via Massarenti, 9, Bologna, 40138, Italy

    Ornella Leone M.D.

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  1. Marialuisa Valente M.D.
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  2. Stefania Rizzo M.D.
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  3. Ornella Leone M.D.
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  4. Cristina Basso M.D., Ph.D.
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Correspondence to Marialuisa Valente M.D. .

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  1. Thoracic and Vascular Sciences, Department of Cardiac, University of Padua, Padova, 35121, Italy

    Cristina Basso

  2. Thoracic and Vascular Sciences, Department of Cardiac,, University of Padua, Padova, 35121, Italy

    Marialuisa Valente

  3. Thoracic and Vascular Sciences, Department of Cardiac,, University of Padua, Padova, 35121, Italy

    Gaetano Thiene

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Valente, M., Rizzo, S., Leone, O., Basso, C. (2013). Primary Malignant Tumors of the Heart. In: Basso, C., Valente, M., Thiene, G. (eds) Cardiac Tumor Pathology. Current Clinical Pathology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-143-1_6

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