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Published on: March 7, 2017
Primary cardiac tumours in a paediatric population
1Queensland Centre for Congenital Heart Disease, Prince Charles Hospital, Chermside, Queensland, Australia.
Insights
Primary cardiac tumors in children, though rarely malignant, can lead to significant mortality and morbidity. Early diagnosis and management are crucial for improving outcomes in pediatric patients with these rare heart conditions.
Area of Science:
- Pediatric Cardiology
- Cardiac Oncology
- Congenital Heart Disease
Background:
- Primary cardiac tumors are rare in children.
- These tumors can present with diverse clinical manifestations and histological types.
Purpose of the Study:
- To review the presentation, diagnosis, histology, and outcomes of primary cardiac tumors in a pediatric cardiac unit.
- To analyze the management challenges and mortality associated with these tumors.
Main Methods:
- Retrospective review of hospital records and databases.
- Data collected over a 20-year period (1980-2000).
Main Results:
- Twelve pediatric patients were identified with four histological types: rhabdomyoma, myxoma, fibroma, and myocardial hamartoma.
- Diagnosis occurred prenatally (ultrasound) in five cases and neonatally in three.
- Fifty percent of cases required surgical intervention, with three tumor-related deaths. Two infants developed univentricular physiology due to large left ventricular tumors.
Conclusions:
- Primary cardiac tumors in children, despite lacking malignant histology, are associated with significant morbidity and mortality.
- The development of univentricular physiology in infants with large left ventricular tumors presents a rare but challenging management problem.
Objective:
To review the presentation, diagnosis, histology and outcome of primary cardiac tumours presenting to a paediatric cardiac unit over a 20-year period.
Methods:
Hospital records and data bases were searched for the years 1980-2000.
Results:
There were 12 patients with four histological tumour types including a predominance of rhabdomyoma, as well as myxoma, fibroma and myocardial hamartoma. Diagnosis was made in utero, by ultrasound in five cases and in the neonatal period in a further three cases. Six cases (50%) required surgical intervention and there were three tumour-related deaths. Two infants with large left ventricular tumours diagnosed in utero developed univentricular physiology, acting like hypoplastic left heart syndrome at birth.
Conclusions:
Despite an absence of malignant histology there was significant mortality and morbidity among the patients reviewed. The development of univentricular physiology in infants with large left ventricular tumours is rare and is a difficult management problem.
