Composite hemangioendothelioma (CHE) is a rare vascular tumor which shows varying combination of benign, low‐grade, and malignant vascular components on pathology. CHE is usually located on the surface of the dermis and subcutaneous tissue of the extremities. We report an unusual case of CHE in the heart.

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Background: Primary pericardial synovial sarcoma is an extremely rare malignant invasive tumor with dismal prognosis. Methods: We herein report a case of a 29-year-old woman of Chinese descent, who was admitted to our hospital with a fever and subsequently diagnosed to have primary pericardial synovial sarcoma. Results: A giant mass was found on transthoracic echocardiography. Computed tomography and magnetic resonance imaging identified a giant pericardial mass compressing the left atrium. Ultrasound-guided biopsy confirmed a monophasic synovial sarcoma. The patient refused further treatment and died 22 months later. Conclusion: We report a rare case of primary pericardial synovial sarcoma, which generally has a poor prognosis. Molecular genetic analysis may greatly contribute to the definite diagnosis.

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