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Published on: July 25, 2019
Right atrial myxoma with glandular differentiation: A rare entity in pediatric age group
Saumya R Mallick1, Prasenjit Das, Bhaskar Shukla
1Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Insights
This study reports a rare cardiac myxoma (CM) with glandular differentiation in a child. Recognizing this rare pediatric presentation is crucial to avoid misdiagnosis as adenocarcinoma.
Area of Science:
- Cardiovascular Pathology
- Pediatric Oncology
Background:
- Cardiac myxomas (CMs) are the most common primary cardiac tumors, typically affecting the elderly.
- These tumors originate from sub-endocardial cells with potential for divergent differentiation.
Observation:
- A 10-year-old child presented with exertional respiratory distress due to two large right atrial masses.
- Echocardiography revealed interconnected masses measuring up to 34x30 mm.
Findings:
- Microscopic examination confirmed a cardiac myxoma with prominent glandular differentiation.
- The tumor exhibited a characteristic immunophenotype, distinct from metastatic adenocarcinoma.
Implications:
- This case highlights the rare occurrence of cardiac myxoma with glandular differentiation in pediatric patients.
- Accurate histomorphologic recognition is essential to differentiate from malignant tumors like adenocarcinoma.
Abstract:
Cardiac myxomas (CMs) account for nearly half of the primary cardiac tumors in the elderly. They arise from sub-endocardial "reserve" or lepidic" cells, which may show divergent differentiation. We describe a CM with glandular differentiation in the right atrium of a 10-year-old child who presented with respiratory distress on exertion, of 2 months duration. On echocardiography, two large interconnected masses measuring 34×30 mm and 20×17 mm were seen to arise from the free wall of the right atrium. Cut surface of the excised mass was myxoid with areas of calcification. On microscopy, there were typical features of a myxoma with prominent glandular differentiation and characteristic immunophenotype. The case is being reported due to its rarity in pediatric age group as well as its glandular differentiation, which must be recognized as a spectrum of histomorphologic diversity and must not be mistaken for a metastatic adenocarcinoma.
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