<abstract xmlns="http://www.w3.org/1999/xhtml">

<p>The most common primary cardiac tumors are myxomas, while cardiac hamartomas are rare. Cardiac hamartomas may constitute manifestation of a genetic disorder such as tuberous sclerosis, or could be diagnosed independently as a solitary lesion. We present two rare cases of primary cardiac tumors detected via diagnostic imaging and subsequent histopathology examination. The first described case is that of a male preterm foetus with multiple rhabdomyomas in the heart, discovered during foetal echocardiography. The finding was later confirmed by autopsy, which also revealed presence of cortical tubers with typical balloon cells constituting well known epileptogenic lesions in the foetal brain. The second case describes an even rarer benign cardiac lesion – a hamartoma of mature cardiomyocytes – which was discovered by chance in a 49-year old female patient during echocardiography. The performed CT scan revealed a 37 x 16 x 12 mm tumor in the right atrium obstructing the superior vena cava inlet. The malformation was surgically removed and examined histologically revealing disorganized hypertrophic mature cardiomyocytes, partly separated by fibrous strands and admixed with mature adipocytes. Differential diagnosis was performed to rule out other benign tumors with myocyte differentiation – cardiac rhabdomyoma, histiocytoid cardiomyopathy and adult cellular rhabdomyoma.</p>
<sec><h3>Conclusion</h3>
<p>The diagnosis of cardiac hamartomas was possible only after pathomorphological examination of material from the tumors. Reporting of rare neoplasms is crucial in order to determine their prevalence and to inform clinical practice.</p>
</sec>
</abstract>

The most common primary cardiac tumors are myxomas, while cardiac hamartomas are rare. Cardiac hamartomas may constitute manifestation of a genetic disorder such as tuberous sclerosis, or could be diagnosed independently as a solitary lesion. We present two rare cases of primary cardiac tumors detected via diagnostic imaging and subsequent histopathology examination. The first described case is that of a male preterm foetus with multiple rhabdomyomas in the heart, discovered during foetal echocardiography. The finding was later confirmed by autopsy, which also revealed presence of cortical tubers with typical balloon cells constituting well known epileptogenic lesions in the foetal brain. The second case describes an even rarer benign cardiac lesion – a hamartoma of mature cardiomyocytes – which was discovered by chance in a 49-year old female patient during echocardiography. The performed CT scan revealed a 37 x 16 x 12 mm tumor in the right atrium obstructing the superior vena cava inlet. The malformation was surgically removed and examined histologically revealing disorganized hypertrophic mature cardiomyocytes, partly separated by fibrous strands and admixed with mature adipocytes. Differential diagnosis was performed to rule out other benign tumors with myocyte differentiation – cardiac rhabdomyoma, histiocytoid cardiomyopathy and adult cellular rhabdomyoma.
Conclusion
The diagnosis of cardiac hamartomas was possible only after pathomorphological examination of material from the tumors. Reporting of rare neoplasms is crucial in order to determine their prevalence and to inform clinical practice.

Cardiac Hamartomas: Report of Two Rare Primary Cardiac Tumors (Rhabdomyoma and Hamartoma of Mature Cardiomyocytes) with Review of the Literature

University Hospital “Sv. Ivan Rilski” – Sofia

Department of Anatomy and Histology, Pathology and Forensic Medicine

University “Sv. Kliment Ohridski” – Sofia

Acta Medica Bulgarica

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

The most common primary cardiac tumors are myxomas, while cardiac hamartomas are rare. Cardiac hamartomas may constitute manifestation of a genetic disorder...

Cardiac Hamartomas: Report of Two Rare Primary Cardiac Tumors (Rhabdomyoma and Hamartoma of Mature Cardiomyocytes) with Review of the Literature

Pubblicato online: 14 mag 2023

Pagine: 66 - 72

Ricevuto: 24 giu 2022

Accettato: 15 set 2022

DOI: https://doi.org/10.2478/amb-2023-0021

Parole chiave
tuberous sclerosis, rhabdomyoma, hamartoma of mature cardiomyocytes, histiocytoid cardiomyopathy

© 2023 D. Metodiev et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Cardiac Hamartomas: Report of Two Rare Primary Cardiac Tumors (Rhabdomyoma and Hamartoma of Mature Cardiomyocytes) with Review of the Literature

Pubblicato online: 14 mag 2023

Pagine: 66 - 72

Ricevuto: 24 giu 2022

Accettato: 15 set 2022

DOI: https://doi.org/10.2478/amb-2023-0021

Parole chiavetuberous sclerosis, rhabdomyoma, hamartoma of mature cardiomyocytes, histiocytoid cardiomyopathy

© 2023 D. Metodiev et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Parole chiave
tuberous sclerosis, rhabdomyoma, hamartoma of mature cardiomyocytes, histiocytoid cardiomyopathy