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Pediatric cardiac tumors: a 45-year, single-institution review
Laura Linnemeier1, Brian D Benneyworth2, Mark Turrentine1
1Department of Cardiovascular Surgery, Indiana University School of Medicine, Indianapolis, IN, USA.
Insights
Pediatric cardiac tumors are rare. Aggressive surgical and chemotherapy management offers good outcomes for benign and malignant cases, respectively, improving survival in children.
Area of Science:
- Pediatric Cardiology
- Pediatric Oncology
- Cardiovascular Surgery
Background:
- Cardiac tumors in children are exceptionally rare.
- Most reported pediatric cardiac tumors are benign and treated conservatively.
Purpose of the Study:
- To evaluate the presentation, pathology, management, and outcomes of pediatric cardiac tumors.
- To assess the efficacy of aggressive surgical and adjuvant treatment strategies.
Main Methods:
- Retrospective, observational study of pediatric patients (<18 years) undergoing surgical evaluation for cardiac tumors.
- Data collected from 1969 to 2014 at a tertiary care children's hospital.
- Analysis of tumor pathology, surgical intervention, chemotherapy, and patient outcomes.
Main Results:
- 64 pediatric patients evaluated for cardiac tumors over 45 years.
- Rhabdomyoma was the most frequent benign tumor (58%); 17% of tumors were malignant.
- Benign tumors often required surgery for hemodynamic compromise (42%); malignant tumors (73%) underwent radical excision and chemotherapy.
- Malignant tumors had a 37% mortality rate, but aggressive treatment offered long-term survival in some. Benign tumors had no mortality, with complications mainly related to mitral regurgitation.
Conclusions:
- Pediatric cardiac tumors, though rare, can be managed effectively with aggressive approaches.
- Benign tumors generally have excellent survival, with complications tied to location.
- Malignant tumors carry high mortality, yet selected patients benefit from surgery and chemotherapy for prolonged survival.
Background:
Cardiac tumors in children are rare. Of the cases reported in the literature, nearly all are benign and managed conservatively.
Methods:
This is a retrospective, observational study of pediatric patients <18 years who presented for surgical evaluation of a cardiac tumor, between 1969 and 2014 at a tertiary care children's hospital. Presentation, pathology, management, and outcomes were evaluated.
Results:
Over the last 45 years, 64 patients were evaluated for surgical resection of a cardiac tumor. Rhabdomyoma was the most common neoplasm (58%), and 17% of the tumors had malignant pathologies. While 42% of benign cardiac neoplasms required surgical intervention for significant hemodynamic concerns, 73% of malignant neoplasms underwent radical excision, if possible, followed by adjuvant chemotherapy. Despite a 37% mortality in patients with malignant pathology, an aggressive surgical approach can yield long-term survival in some patients. There were no deaths among patients with benign tumors and 17% had postoperative complications mostly related to mitral regurgitation.
Conclusion:
Cardiac tumors in children are rare but can be managed aggressively with good outcomes. Benign tumors have an excellent survival with most complications related to tumor location. Malignant tumors have a high mortality rate, but surgery and adjuvant chemotherapy allow for prolonged survival in selected patients.

