Synonyms
Definition
Rhabdomyoma is a primary benign cardiac tumor of the striated cardiac myocytes. These characteristic together with the absence of proliferative activity support the concept of a myocardial hamartoma rather than a true neoplasm.
Clinical Features
Incidence and Clinical Presentation
Rhabdomyoma is the most common primary cardiac tumor in the pediatric population and is increasingly diagnosed prenatally in utero. There is a strong association with tuberous sclerosis since up to 86% of cardiac rhabdomyomas are reported in association with the disease and up to 60% of pediatric patients with tuberous sclerosis undergoing an echocardiographic examination present a cardiac rhabdomyoma. However, the true incidence of isolated rhabdomyomas remains unknown since rhabdomyomas are often asymptomatic and incidentally discovered during echocardiography performed for other reasons. Data coming from surgical series are also an underestimation since cardiac...
References and Further Reading
Basso, C., Valente, M., & Thiene, G. (2013). Cardiac tumor pathology. New York: Springer Humana Press.
Basso, C., Rizzo, S., Valente, M., & Thiene, G. (2016). Cardiac masses and tumours. Heart, 102, 1230–1245.
Demir, H. A., Ekici, F., Yazal Erdem, A., Emir, S., & Tunç, B. (2012). Everolimus: A challenging drug in the treatment of multifocal inoperable cardiac rhabdomyoma. Pediatrics, 130, e243–e247.
Padalino, M. A., Vida, V. L., Boccuzzo, G., Tonello, M., Sarris, G. E., Berggren, H., Comas, J. V., Di Carlo, D., Di Donato, R. M., Ebels, T., Hraska, V., Jacobs, J. P., Gaynor, J. W., Metras, D., Pretre, R., Pozzi, M., Rubay, J., Sairanen, H., Schreiber, C., Maruszewski, B., Basso, C., & Stellin, G. (2012). Surgery for primary cardiac tumors in children: Early and late results in a multicenter European Congenital Heart Surgeons Association study. Circulation, 126, 22–30.
Travis, W. D., Brambilla, E., Burke, A. P., et al. (2015). WHO classification of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press.
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Basso, C., Rizzo, S. (2020). Cardiac Rhabdomyoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5361-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5361-1
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