Early diagnosis, complete surgical resection, and adjuvant chemotherapy can lead to favorable short-term outcomes in patients with highly aggressive and rare primary cardiac angiosarcoma.
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May support multimodal therapy in cardiac angiosarcoma; hypothesis-generating and should not yet change practice.
BACKGROUND: Primary cardiac tumors are exceptionally rare, with an autopsy incidence of only 0.001% to 0.03%. Angiosarcoma represents the most common subtype of cardiac sarcomas, which account for approximately 95% of malignant cases. CASE SUMMARY: A 30-year-old woman presented with chest tightness and dyspnea. Imaging revealed a 65 × 47 mm right atrial mass. She underwent complete surgical resection, with pathology confirming intimal sarcoma. Adjuvant paclitaxel/cisplatin chemotherapy was administered. Short-term follow-up showed no recurrence or metastasis. DISCUSSION: Cardiac angiosarcoma remains a therapeutic challenge with poor prognosis, necessitating multimodal management centered on complete surgical resection and adjuvant chemotherapy. Diagnosis relies heavily on advanced imaging, while definitive confirmation requires histopathological examination. Future research should prioritize targeted therapies and immunotherapies, supported by international collaboration to improve outcomes for this rare malignancy. TAKE-HOME MESSAGE: This case demonstrates that early diagnosis, complete resection, and adjuvant chemotherapy are crucial for managing this highly lethal disease with poor prognosis.
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Zhang et al. (2025) studied this question.
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