Surgical treatment of primary cardiac angiosarcoma: a case report

Primary cardiac angiosarcoma is a rare and highly aggressive vascular malignant neoplasm, with an autopsy detection rate of approximately 0.0017%–0.28% and accounting for 30%–40% of primary cardiac malignant tumors. The disease predominantly affects males aged 40–60 years, with a male-to-female ratio of about 2:1. Approximately 80% of lesions are located in the right atrium, and cases occurring in young patients are exceedingly rare. Clinical manifestations are non-specific, and most patients present with local tissue invasion or distant metastasis at the time of diagnosis, with a median natural survival of only 3–6 months. We report a 22-year-old male patient with primary right atrial angiosarcoma who presented with left eyelid edema as the initial symptom.Pericardial effusion analysis was unremarkable, and transvenous endomyocardial biopsy conducted at an external hospital yielded negative pathological results. The false-negative biopsy result is further discussed in the Discussion section.Subsequent examinations suggested a malignant tumor with possible mediastinal lymph node metastasis, accompanied by intermittent fever.The patient underwent surgical resection of the cardiac mass, coronary artery bypass grafting (CABG), tricuspid valvuloplasty, and right atrial reconstruction under cardiopulmonary bypass in our hospital. Postoperative pathological and immunohistochemical examinations (positive CD31, positive CD34, positive TLE1) confirmed the diagnosis of angiosarcoma. The eyelid edema completely resolved spontaneously after relief of pericardial effusion and radical tumor resection. The patient recovered uneventfully postoperatively, remained stable at 1-month follow-up, and was undergoing chemotherapy at an outside hospital. In patients with cardiac angiosarcoma featuring extensive invasion, lymph node metastasis, and negative preoperative biopsy, active comprehensive surgical treatment (tumor resection + CABG + valvuloplasty + atrial reconstruction) effectively alleviates symptoms, establish a definitive diagnosis, and create favorable conditions for postoperative adjuvant chemotherapy. Early imaging screening, precise surgical intervention, and standardized postoperative adjuvant therapy are key to improving the short-term prognosis of such young patients.

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Journal
Journal of Cardiothoracic Surgery
Published
2026-10-27
DOI
https://doi.org/10.1186/s13019-026-04691-9
Primary Topic
Cardiac tumors and thrombi
Type
article
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article

Surgical treatment of primary cardiac angiosarcoma: a case report

Gang Liu, Qi Miao, Jie Wu, Shaojie Liang
1 links
Journal of Cardiothoracic Surgery
Cardiac tumors and thrombi
article

Surgical treatment of primary cardiac angiosarcoma: a case report

Gang Liu, Qi Miao, Jie Wu, Shaojie Liang
article en

Abstract

Primary cardiac angiosarcoma is a rare and highly aggressive vascular malignant neoplasm, with an autopsy detection rate of approximately 0.0017%–0.28% and accounting for 30%–40% of primary cardiac malignant tumors. The disease predominantly affects males aged 40–60 years, with a male-to-female ratio of about 2:1. Approximately 80% of lesions are located in the right atrium, and cases occurring in young patients are exceedingly rare. Clinical manifestations are non-specific, and most patients present with local tissue invasion or distant metastasis at the time of diagnosis, with a median natural survival of only 3–6 months. We report a 22-year-old male patient with primary right atrial angiosarcoma who presented with left eyelid edema as the initial symptom.Pericardial effusion analysis was unremarkable, and transvenous endomyocardial biopsy conducted at an external hospital yielded negative pathological results. The false-negative biopsy result is further discussed in the Discussion section.Subsequent examinations suggested a malignant tumor with possible mediastinal lymph node metastasis, accompanied by intermittent fever.The patient underwent surgical resection of the cardiac mass, coronary artery bypass grafting (CABG), tricuspid valvuloplasty, and right atrial reconstruction under cardiopulmonary bypass in our hospital. Postoperative pathological and immunohistochemical examinations (positive CD31, positive CD34, positive TLE1) confirmed the diagnosis of angiosarcoma. The eyelid edema completely resolved spontaneously after relief of pericardial effusion and radical tumor resection. The patient recovered uneventfully postoperatively, remained stable at 1-month follow-up, and was undergoing chemotherapy at an outside hospital. In patients with cardiac angiosarcoma featuring extensive invasion, lymph node metastasis, and negative preoperative biopsy, active comprehensive surgical treatment (tumor resection + CABG + valvuloplasty + atrial reconstruction) effectively alleviates symptoms, establish a definitive diagnosis, and create favorable conditions for postoperative adjuvant chemotherapy. Early imaging screening, precise surgical intervention, and standardized postoperative adjuvant therapy are key to improving the short-term prognosis of such young patients.

Journal of Cardiothoracic Surgery
Tianjin University (CN), TEDA International Cardiovascular Hospital (CN)
Good health and well-being
Openalex Percentile: Top 11%
Cardiac tumors and thrombi
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