Primary pediatric cardiac tumors: a single-center experience from a high-volume pediatric cardiac center

Authors

Abstract

AimCardiac tumors in children are rare and often asymptomatic. Nevertheless, in a subset of pediatric patients, they may lead to serious clinical conditions such as arrhythmia, outflow tract obstruction, or even death. This study aimed to evaluate the clinical characteristics, surgical management, and outcomes of pediatric patients diagnosed with cardiac tumors in a single high-volume pediatric cardiac center.
MethodsThis retrospective study included all pediatric patients who underwent surgery for cardiac tumors between January 1, 2020, and January 1, 2025. Data regarding age at diagnosis, presenting symptoms, clinical status, imaging findings, and pathological diagnoses were collected and analyzed statistically.
ResultsDuring the study period, eight operated cases of primary cardiac tumors were identified. Fifty percent of the patients were male, and the median age at diagnosis was 2 years (IQR 1–4 years). Prenatal diagnosis was made in three cases. Thirty-eight percent of the patients were asymptomatic. Among symptomatic patients, the most common presenting complaint was arrhythmia in 37.5% of cases (n=3), followed by chest pain in 12.5% (n=1) and a heart murmur in 12.5% (n=1). The most frequently observed cardiac tumor was cardiac rhabdomyoma in three patients, followed by fibroma in two cases, myxoma in two cases, and teratoma in one case. One patient died after the surgical procedure.
ConclusionPediatric cardiac tumors are relatively rare, generally benign, and associated with favorable outcomes. However, tumors causing acute complications or significant clinical deterioration require timely and effective surgical intervention to prevent adverse outcomes.

Keywords

Introduction

Cardiac tumors are neoplasms originating from the pericardium, myocardium, or endocardium and can occur across all age groups. They are classified into two types: primary tumors, which arise within cardiac tissue, and secondary tumors, which metastasize to the heart from other sites. While primary cardiac tumors are rare in all age groups, secondary tumors are approximately 30 times more common. Most primary cardiac tumors are benign, and nearly half of pediatric cardiac tumors are rhabdomyomas. Other common pediatric cardiac tumors include fibromas, intrapericardial teratomas, myxomas, and hemangiomas.[1–2]
Cardiac tumors may present during fetal or postnatal life. Clinical manifestations depend on the size and location of the mass. In the fetus, a tumor may be detected as an intracardiac mass during routine antenatal anomaly screening or may cause arrhythmia, congestive heart failure, hydrops, and, rarely, stillbirth. In the postnatal period, cardiac tumors may severely impair blood flow due to inflow or outflow tract obstruction or compromise the integrity and function of adjacent cardiac structures, leading to cyanosis, murmur, respiratory distress, myocardial dysfunction, valvular insufficiency, arrhythmias, or sudden death.[1–3]
Diagnosis of cardiac tumors may be made in symptomatic patients, though in rare cases, sudden death may be the initial manifestation. Echocardiography or Magnetic Resonance Imaging (MRI) is usually sufficient to facilitate diagnosis, and cardiac catheterization is rarely required. Tumor biopsy with histological evaluation remains the gold standard for confirmation.^1,2
There is limited research on the diagnosis of primary cardiac tumors, with most reports consisting of single case presentations.³ Given the rarity of pediatric cardiac tumors and the limited number of multicenter surgical series, this study aimed to evaluate pediatric patients diagnosed with cardiac tumors who underwent surgical treatment in a single high-volume pediatric cardiac center.

Materials and Methods

Between January 1, 2020, and January 1, 2025, a total of 8 pediatric patients with cardiac tumors (4 males; median age 2 years (IQR 1–4)) who underwent surgical resection were included.
All patients were initially evaluated using transthoracic echocardiography (TTE) for tumor detection, localization, and hemodynamic assessment. Cardiac MRI was performed in selected patients to further characterize tumor size, tissue properties, and anatomical relationships when echocardiographic findings were inconclusive or additional detail was required for surgical planning.
The inpatient and outpatient medical records were retrospectively collected and classified for the analysis of various types of tumor growth locations, clinical manifestations, surgical indications, and early-term follow-up results.
Surgical indications included symptoms, valve impairment, cardiac dysfunction, outflow/inflow obstruction, arrhythmias, and high risk of systemic embolization.
The study was approved by the local ethics committee (approval number:2026.04.223).
Continuous variables were expressed as median (interquartile range), and categorical variables as number and percentage. Given the small sample size, only descriptive statistical analyses were performed, and no comparative statistical tests were applied.

Results

A total of 28 patients were diagnosed with cardiac tumors during the study period, of whom 8 underwent surgical intervention. The most frequently observed cardiac tumor was cardiac rhabdomyoma in 3 patients, followed by fibroma in 2 cases, myxoma in 2 cases, and teratoma in 1 case. The overall characteristics of the patients are summarized in Table 1.
Among the 8 patients included in the study, tumor involvement was most commonly localized to the ventricles (n=3, 37.5%), followed by the atria (n=2, 25%). Involvement of both atrial and ventricular structures was observed in one case (12.5%), the ventricular septum in one case (12.5%), and the pericardium in one case (12.5%). No cases with valvular involvement were identified.
Prenatal diagnosis was established in three patients (37.5%). Thirty-eight percent of the cases were asymptomatic. Among symptomatic patients, the most frequent presenting complaint was arrhythmia in 38% (n=3), followed by chest pain in 12.5% (n=1) and murmur in 12.5% (n=1). The main clinical features of patients diagnosed with cardiac tumors are shown in Table 2.
Early postoperative mortality occurred in one patient (12.5%), a 3-month-old infant operated on for left ventricular fibroma, due to low cardiac output syndrome in the postoperative period.

Discussion

In this study, we evaluated pediatric patients who underwent surgery for cardiac tumors in a single high-volume cardiac center. The most common tumor observed was rhabdomyoma, which presented with a variety of clinical manifestations. Our study represents a small case series of eight patients.
Cardiac rhabdomyoma is a hamartoma that develops during cardiomyocyte maturation. It is often asymptomatic and tends to regress spontaneously. Approximately half of patients with rhabdomyoma experience partial tumor regression, and 18% achieve complete remission. Only a minority of patients present with symptoms such as heart failure, arrhythmia, or outflow tract obstruction.⁴ In a study by Shi et al.,⁵ which included 166 cases of cardiac tumors, the frequency of rhabdomyomas was reported as 63.4%, with 14% requiring surgery. Similarly, Craig et al. ⁴ reported a frequency of 77% among benign cardiac tumors. In our series, rhabdomyomas accounted for 45% of cases. The lower rate in our study may be explained by the tendency of rhabdomyomas to regress spontaneously or to be managed medically, thereby reducing the need for surgical intervention.
Cardiac fibroma is the second most common cardiac tumor after rhabdomyoma.⁶ Fibromas usually present as a single, non-convex growth projecting into the cardiac cavity, most commonly located in the anterior wall and interventricular septum. Because fibromas do not regress spontaneously, timely surgical resection is recommended for symptomatic patients and for asymptomatic young patients with large tumor size.⁷ Fibromas are the cardiac tumor type most strongly associated with arrhythmias. Miyake et al. ⁸ reported that fibromas may infiltrate the ventricular conduction system and lead to ventricular arrhythmias with an incidence as high as 64%. In the study by Shi et al.,⁵ surgical intervention was required in 11 out of 21 cases of fibroma, with 50% of the indications being ventricular arrhythmias. In our study, two patients underwent surgery for fibroma, one of whom had refractory ventricular arrhythmias.
Cardiac myxoma is the most common primary cardiac tumor in adults but is rare in children, typically occurring in the left atrium. Surgical treatment is required; however, recurrence may occur due to incomplete resection or the presence of multiple tumors.⁹ In our series, both cases of myxoma were located in the left atrium, and no recurrence was observed.
Teratomas are neoplasms derived from embryonic tissues and are characterized by a variable composition of cells from the three germ layers. They account for a small proportion of primary cardiac tumors, representing only about 7% of all cardiac neoplasms.³ Cardiac teratomas predominantly involve the pericardium, with fewer cases arising in the myocardium. Neonates or fetuses with teratomas may present with symptoms due to mass effect and pericardial effusion. Common clinical manifestations include cyanosis, congestive heart failure, and respiratory distress.¹ In our study, one case of cardiac teratoma was identified.
Pediatric cardiac hemangioma is rare and presents with nonspecific clinical features. Depending on its location and size, it may lead to coronary ischemia, dyspnea, arrhythmia, pericardial effusion, heart failure, or outflow tract obstruction, while sudden death is very uncommon.^5,10 Surgical resection is the treatment of choice for symptomatic patients. In our series, no hemangioma cases were present.
Despite their rarity, pediatric cardiac tumors may present with diverse and potentially life-threatening clinical manifestations. Our findings highlight the importance of individualized management and careful selection of patients for surgical intervention.

Limitations

This study has several limitations, including its retrospective design, single-center nature, and the small sample size. Additionally, only surgically treated patients were included, which may have introduced selection bias and limited the generalizability of the findings.

Conclusion

Cardiac tumors in children are relatively rare, mostly benign, and generally associated with favorable prognosis. However, in cases of tumors causing acute complications or adverse effects, timely and effective surgical intervention is required to avoid unfavorable outcomes.

Declarations

Abbreviations

MRI: Magnetic resonance imaging
TTE: Transthoracic echocardiography

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About This Article

Received:

May 8, 2026

Accepted:

June 19, 2026

Published Online:

June 22, 2026