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Intracardiac teratoma in a child simulating an atrioventricular nodal tumor
1Department of Laboratories, North Shore University Hospital-Cornell University Medical College, Manhasset, New York 11030.
Insights
Intracardiac teratoma, a rare pediatric tumor, can present initially as an atrioventricular nodal tumor. Recurrence revealed mature cystic teratoma with pancreatic islet-like structures.
Area of Science:
- Pediatric Oncology
- Cardiovascular Pathology
- Developmental Biology
Background:
- Intracardiac teratomas are exceedingly rare pediatric neoplasms.
- Diagnosis can be challenging due to initial presentation mimicking other cardiac tumors.
Observation:
- A 6-year-old girl presented with a right intraventricular cardiac mass.
- Initial biopsy suggested an atrioventricular nodal tumor.
- Recurrence three years later showed mature cystic teratoma with diverse germ layer elements.
Findings:
- Histological analysis of the recurrent tumor revealed mature elements from all three germ layers.
- Immunostaining demonstrated positivity for pancreatic islet hormones (insulin, glucagon, somatostatin) and chromogranin.
- These findings indicated overgrown pancreatic islets within the teratoma.
Implications:
- This case highlights the complex presentation and potential for diverse differentiation in pediatric cardiac teratomas.
- The presence of functional pancreatic islet-like tissue within a teratoma offers insights into germ cell tumor development.
- Understanding these rare entities is crucial for accurate diagnosis and management in pediatric cardiology and oncology.
Abstract:
Intracardiac teratoma is an extremely rare pediatric neoplasm. We studied the case of a 6-year-old girl with a right intraventricular cardiac mass. The tumor consisted of clusters of monotonous round epithelial cells scattered in a dense fibrotic stroma and was thought to represent an atrioventricular nodal tumor. Three years later the tumor recurred, with multiple mature elements derived from all three germ layers, and was diagnosed as mature cystic teratoma. Still present, however, were multiple areas that were histologically similar to the earlier lesion. Immunostaining revealed strong positivity for insulin, glucagon, somatostatin, and chromogranin consistent with overgrown pancreatic islets of Langerhans within a mature teratoma.