2017PubMedRequires access

Symptomatic Fetal-Type Cardiac Rhabdomyoma.

Şeyma Kayalı, Vehbi Doğan, Nilüfer Arda, Murat Koç, İlker Ertuğrul, Senem Özgür, Utku Arman Örün, Selmin Karademir

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Abstract

Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

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Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

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Available abstract

Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

Key concepts: Rhabdomyoma, Medicine, Autopsy, Fetus, Cardiology, Cardiac surgery, Internal medicine, Cardiac Tumors

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