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Updated: Feb 10, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Surgically treated primary cardiac tumors in early infancy and childhood
Massimo A Padalino1, Cristina Basso, Ornella Milanesi
1Division of Pediatric Cardiovascular Surgery, University of Padova Medical School, Padova, Italy.
Insights
Surgical removal of primary heart tumors in children is safe and effective, with most patients remaining symptom-free post-operation. Heart transplantation is an option for extensive tumors.
Area of Science:
- Pediatric Cardiology
- Cardiac Surgery
- Pediatric Oncology
Background:
- Primary heart tumors in children are rare, with most being benign.
- Surgical intervention is recommended for symptomatic or hemodynamically impaired patients.
Purpose of the Study:
- To evaluate the feasibility and outcomes of surgical treatment for primary heart tumors in pediatric patients.
- To assess the role of echocardiography and histopathology in diagnosis and management.
Main Methods:
- Retrospective review of 8 pediatric patients (age 5 days to 6.7 years) who underwent surgery for cardiac masses between 1986 and 2003.
- Diagnosis was established using prenatal and 2-dimensional Doppler echocardiography.
- Surgical pathology identified myxoma, fibroma, rhabdomyoma, hamartoma, and teratoma.
Main Results:
- Complete surgical excision was achieved in 7 out of 8 patients; one patient required heart transplantation.
- The patient who underwent transplantation died of cerebral malignancy 38 months later.
- At a mean follow-up of 69.2 months, remaining patients were asymptomatic with good ventricular function and no tumor recurrence.
Conclusions:
- Surgical excision of obstructive cardiac tumors in childhood is a safe and feasible treatment.
- Heart transplantation may be the only option for tumors extensively invading ventricular walls.
- While 2-dimensional echocardiography is reliable for diagnosis, histopathology is crucial for definitive tumor characterization.
Objective:
Primary heart tumors in childhood are rare and mostly benign. Surgical treatment is advocated when symptoms or hemodynamic impairment is present.
Materials And Methods:
Between 1986 and 2003, 8 children (3 males and 5 females, age ranging 5 days to 6.7 years, median 78 days) with a clinical diagnosis of cardiac mass were treated with surgery. Diagnosis was made by prenatal echocardiography in 3 patients and by 2-dimensional Doppler echocardiography in 5 patients.
Results:
Complete surgical excision of the cardiac mass was feasible in all but 1 patient who underwent orthotopic heart transplantation. Surgical pathology examination revealed myxoma in 2 patients, fibroma in 2 patients, rhabdomyoma in 2 patients (multiple in 1), hamartoma in 1 patient, and teratoma in 1 patient. One patient died of cerebral malignancy 38 months after cardiac transplantation. At a mean follow-up of 69.2 months (range 3-190 months), all the remaining patients are asymptomatic, with good ventricular function on 2-dimensional echocardiography and no signs of residual or recurrent tumor.
Conclusion:
Surgical excision of obstructive cardiac tumors in childhood is safely feasible. Heart transplantation may represent the only therapeutic option when the tumor extensively invades the ventricular walls. Although 2-dimensional echocardiography remains a reliable diagnostic tool, a definite diagnosis of tumor histotype requires a thorough histopathologic characterization.
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