Key result
Resection plus gemcitabine and docetaxel achieves zero recurrence at 6 months for right atrial composite hemangioendothelioma.
Why the study?
Primary cardiac vascular tumors are rare in children, and clinical presentation and management details are limited.
Case Report (n=1)
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“We utilized a multimodality imaging approach. This included echocardiography, contrast-enhanced computed tomography, and cardiac MRI to diagnose this rare tumor. The findings were confirmed via histopathology and immunohistochemistry of the surgically resected tumor. Additionally, eight weeks post-surgery, the patient underwent an 18F-FDG PET-CT, which is a molecular and functional imaging modality to check for residual tumor and for staging purposes”
Surgical excision and adjuvant chemotherapy successfully managed a rare composite hemangioendothelioma of the right atrium presenting with cardiac tamponade in an adolescent.
Primary cardiac vascular tumors are rare in children. We report a 14-year-old girl with hemorrhagic pericardial effusion, cardiac tamponade, and a large right atrial mass. Echocardiogram identified the lesion and tamponade physiology. Pericardial drainage and stabilization preceded computed tomography (CT) and cardiac magnetic resonance imaging. The mass was excised under cardiopulmonary bypass, and the atrial defect was reconstructed with an autologous pericardial patch. Histopathology and immunohistochemistry favored composite hemangioendothelioma of low malignant potential. Postoperative FDG PET-CT showed no residual or metastatic disease. Adjuvant gemcitabine/docetaxel-based chemotherapy was given after multidisciplinary review. Six-month echocardiogram showed no recurrence.
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Chandran et al. (2026) conducted a case report in Composite hemangioendothelioma of the right atrium (n=1). Surgical excision and adjuvant gemcitabine/docetaxel-based chemotherapy was evaluated on Tumor recurrence or metastasis. Surgical excision followed by adjuvant gemcitabine/docetaxel-based chemotherapy for a right atrial composite hemangioendothelioma resulted in no recurrence or metastasis at 6 months.
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