Key result
Radical surgical resection with CABG and reconstruction achieves R0 margins and short-term clinical stability.
Why the study?
Primary cardiac angiosarcoma is a rare, highly aggressive malignancy with non-specific manifestations, frequent local invasion or distant metastasis at diagnosis, a median natural survival of 3–6 months, and exceedingly rare occurrence in young patients.
Case Report (n=1)
No
Active comprehensive surgical treatment (tumor resection, CABG, valvuloplasty, and atrial reconstruction) in a young patient with locally invasive primary right atrial angiosarcoma and negative preoperative biopsy can effectively alleviate symptoms, establish a definitive diagnosis, and bridge to adjuvant chemotherapy.
Supports aggressive surgical management for symptom control in rare young-onset right atrial angiosarcoma; leaves open long-term survival benefits.
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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Wu et al. (2026) conducted a case report in Primary right atrial angiosarcoma (n=1). Radical tumor resection, CABG, tricuspid valvuloplasty, right atrial reconstruction, and adjuvant doxorubicin plus ifosfamide chemotherapy was evaluated on R0 surgical resection, symptom resolution, and short-term clinical stability. Radical surgical resection with CABG, tricuspid valvuloplasty, and atrial reconstruction achieved R0 margins, resolved symptoms, and maintained stability at 1-month follow-up in a 22-year-old male.
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