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Large cardiac fibroma and teratoma in children- case reports.

Jha NK, Kiraly L, Tamas C, Talo H, Khan MD, El Badaoui H, Jain A, Hammad A - J Cardiothorac Surg (2015)

Bottom Line: There are several consecutive series published in the last decade on cardiac neoplasms.Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms.We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision.

View Article: PubMed Central - PubMed

Affiliation: Division of Paediatric Cardiac Surgery, Sheikh Khalifa Medical City (managed by Cleveland Clinic), PO Box-51900, Karamah Street, Abu Dhabi, United Arab Emirates. njha@skmc.ae.

ABSTRACT
Primary cardiac tumours in paediatric population are an unusual occurrence. Although, majority of such tumours are benign (90%), the frequency and type of cardiac tumours in this age group is different from the adult population. There are several consecutive series published in the last decade on cardiac neoplasms. Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms. We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision. A possible role of cardiac remodeling in myocardial tissue healing after extensive tissue resection in such patients is hypothesised through available experimental or limited clinical information.

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Related in: MedlinePlus

A well-demarcated spindle cell lesion with fibromyxoid and vascular stroma. Rare micro-calcifications are also noted (arrow) (hematoxylin and eosin stained, 10x).
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Fig3: A well-demarcated spindle cell lesion with fibromyxoid and vascular stroma. Rare micro-calcifications are also noted (arrow) (hematoxylin and eosin stained, 10x).

Mentions: A 12-month-old, Arabic, patient was admitted on emergent basis with multifocal ventricular ectopic beats associated with low cardiac output. He was intubated and resuscitated. Echocardiography demonstrated a large tumour mass filling the apical parts of both the ventricular cavities with severe restriction to the diastolic filling. Other cardiac structures were normal. The CT angiography of the chest revealed a well-defined, near-fluid density, large, space-occupying lesion within the heart involving the anterior-inferior part of the interventricular septum bulging into the ventricular cavity and compromising it (Figure 1). There were some cystic changes and patchy calcifications in the tumour. Emergent cardiac surgery was performed through the median sternotomy under standard cardiopulmonary bypass (CPB) using aortic and bicaval cannulation at moderate systemic hypothermia. Under cardioplegic arrest and aortic cross clamping, the right ventricle was opened parallel to the left anterior descending artery. Resection of the mass (4×4 cm) was done “in-toto” including the part of ventricular septum (Figure 2). The resultant thinning of the septum was reinforced with a 0.4mm polytetrafluoroethylene patch (PTFE). The PTFE was preferred due to low thrombogenicity, less porosity and biostability. The patient was weaned from the CPB and a postoperative transoesophageal echocardiography confirmed the removal of tumour mass and absence of residual shunts within the cardiac chambers. The histopathological examination of the excised tumour confirmed the diagnosis of a well encapsulated matured fibroma (Figure 3). The patient was discharged home and at 1 year follow up he was asymptomatic with no evidence of recurrence on echocardiography.Figure 1


Large cardiac fibroma and teratoma in children- case reports.

Jha NK, Kiraly L, Tamas C, Talo H, Khan MD, El Badaoui H, Jain A, Hammad A - J Cardiothorac Surg (2015)

A well-demarcated spindle cell lesion with fibromyxoid and vascular stroma. Rare micro-calcifications are also noted (arrow) (hematoxylin and eosin stained, 10x).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4373309&req=5

Fig3: A well-demarcated spindle cell lesion with fibromyxoid and vascular stroma. Rare micro-calcifications are also noted (arrow) (hematoxylin and eosin stained, 10x).
Mentions: A 12-month-old, Arabic, patient was admitted on emergent basis with multifocal ventricular ectopic beats associated with low cardiac output. He was intubated and resuscitated. Echocardiography demonstrated a large tumour mass filling the apical parts of both the ventricular cavities with severe restriction to the diastolic filling. Other cardiac structures were normal. The CT angiography of the chest revealed a well-defined, near-fluid density, large, space-occupying lesion within the heart involving the anterior-inferior part of the interventricular septum bulging into the ventricular cavity and compromising it (Figure 1). There were some cystic changes and patchy calcifications in the tumour. Emergent cardiac surgery was performed through the median sternotomy under standard cardiopulmonary bypass (CPB) using aortic and bicaval cannulation at moderate systemic hypothermia. Under cardioplegic arrest and aortic cross clamping, the right ventricle was opened parallel to the left anterior descending artery. Resection of the mass (4×4 cm) was done “in-toto” including the part of ventricular septum (Figure 2). The resultant thinning of the septum was reinforced with a 0.4mm polytetrafluoroethylene patch (PTFE). The PTFE was preferred due to low thrombogenicity, less porosity and biostability. The patient was weaned from the CPB and a postoperative transoesophageal echocardiography confirmed the removal of tumour mass and absence of residual shunts within the cardiac chambers. The histopathological examination of the excised tumour confirmed the diagnosis of a well encapsulated matured fibroma (Figure 3). The patient was discharged home and at 1 year follow up he was asymptomatic with no evidence of recurrence on echocardiography.Figure 1

Bottom Line: There are several consecutive series published in the last decade on cardiac neoplasms.Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms.We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision.

View Article: PubMed Central - PubMed

Affiliation: Division of Paediatric Cardiac Surgery, Sheikh Khalifa Medical City (managed by Cleveland Clinic), PO Box-51900, Karamah Street, Abu Dhabi, United Arab Emirates. njha@skmc.ae.

ABSTRACT
Primary cardiac tumours in paediatric population are an unusual occurrence. Although, majority of such tumours are benign (90%), the frequency and type of cardiac tumours in this age group is different from the adult population. There are several consecutive series published in the last decade on cardiac neoplasms. Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms. We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision. A possible role of cardiac remodeling in myocardial tissue healing after extensive tissue resection in such patients is hypothesised through available experimental or limited clinical information.

Show MeSH
Related in: MedlinePlus