Long-Term Survival after Repeat Resection and Multimodal Therapy for Primary Cardiac Angiosarcoma: A Case Report

Primary cardiac angiosarcoma (PCA) is an exceptionally rare and aggressive malignant tumor characterized by a poor prognosis. Although complete surgical resection is the most effective treatment for survival, recurrence and metastasis occur frequently even after radical excision.

We report a rare case of PCA in a 40-year-old woman in whom long-term survival of 68 months was achieved through aggressive multimodal therapy. She underwent urgent complete resection of a right atrial tumor, followed by preoperative chemotherapy and repeat resection with tricuspid valve replacement for recurrent disease and pulmonary metastasis. Postoperative adjuvant chemotherapy resulted in disease-free survival of 41 months. The subsequent hepatic and pulmonary metastases were managed with chemotherapy and radiotherapy before the patient succumbed to multiple organ failure.

Individualized, aggressive, and multimodal treatment-including repeat surgical resection and sustained systemic therapy-can achieve durable disease control and prolonged survival in selected patients with PCA, a malignancy otherwise associated with extremely poor outcomes.