右心房原发性上皮样血管肉瘤伴症状性心动过缓：病例报告
Primary Epithelioid Angiosarcoma of the Right Atrium Presenting With Symptomatic Bradycardia: A Case Report.
摘要(中文译文)
原发性心脏血管肉瘤是一种罕见且侵袭性强的恶性肿瘤，主要累及心脏右心房。本报告介绍一种罕见变异型——心脏上皮样血管肉瘤。由于其罕见性，该病的流行病学和发病机制尚不清楚。我们报道一例44岁女性患者，表现为心悸、胸痛及症状性心动过缓。多模态影像学检查发现右心房大型广基肿块。肿块位于窦房结区域附近，可能导致了患者的心动过缓。患者接受了手术切除及牛心包补片重建。组织病理学倾向于上皮样血管肉瘤，由于标本碎裂，切缘不明确。术后病程并发交界性心律缓慢性心律失常，需要起搏治疗。包括脑部MRI及胸部、腹部和盆腔CT在内的全面分期检查排除了远处转移。由于肿瘤位于心脏，患者被认为不适合接受放射治疗。尽管肿瘤科建议辅助AIM化疗（多柔比星、异环磷酰胺、美司钠），但鉴于化疗的预期获益有限，患者选择观察。本病例凸显了原发性心脏血管肉瘤管理的复杂性，尤其是当标准治疗选择有限时，并强调了个体化多学科诊疗的必要性。
Abstract
Primary cardiac angiosarcoma is a rare and aggressive malignancy that mainly affects the right atrium of the heart. In this report, we present a rare variant known as cardiac epithelioid angiosarcoma. Due to its rarity, the epidemiology and pathogenesis of this disease are not well understood. We present the case of a 44-year-old woman who presented with palpitations, chest pain, and symptomatic bradycardia. Multimodal imaging identified a large, sessile right atrial mass. The location of the mass near the sinoatrial node region likely contributed to the patient's bradycardia. The patient underwent surgical resection with bovine pericardial patch reconstruction. Histopathology favored epithelioid angiosarcoma, and the margins were unclear due to specimen fragmentation. The postoperative course was complicated by junctional rhythm bradyarrhythmia requiring pacing. Comprehensive staging, including brain MRI and CT of the chest, abdomen, and pelvis, ruled out distant metastasis. The patient was deemed not to be a candidate for radiation therapy due to the tumor's location in the heart. Although adjuvant AIM chemotherapy (doxorubicin, ifosfamide, mesna) was recommended by oncology, the patient opted for observation, given the limited expected benefit of chemotherapy. This case highlights the complexity of managing primary cardiac angiosarcoma, particularly when standard therapeutic options are limited, and underscores the need for individualized, multidisciplinary care.
如何引用
AMA
Omar Sadaqah, Bahy Abofrekha, Ahmed Zayed, Abdelrahman Abouelnas, Ekrem Yetiskul, Gil Garber, et al. Primary Epithelioid Angiosarcoma of the Right Atrium Presenting With Symptomatic Bradycardia: A Case Report.. Cureus. 2026; doi:10.7759/cureus.108865.
APA
Omar Sadaqah, Bahy Abofrekha, Ahmed Zayed, Abdelrahman Abouelnas, Ekrem Yetiskul, Gil Garber, et al (2026). Primary Epithelioid Angiosarcoma of the Right Atrium Presenting With Symptomatic Bradycardia: A Case Report.. Cureus. https://doi.org/10.7759/cureus.108865
GB/T 7714
Omar Sadaqah, Bahy Abofrekha, Ahmed Zayed, Abdelrahman Abouelnas, Ekrem Yetiskul, Gil Garber, et al. Primary Epithelioid Angiosarcoma of the Right Atrium Presenting With Symptomatic Bradycardia: A Case Report.[J]. Cureus, 2026 doi:10.7759/cureus.108865.
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