Background/Objectives:Primary cardiac sarcomas are rare and aggressive tumors. Management is often guided by evidence from other sarcoma types due to limited disease-specific data. This study aimed to analyze the clinical characteristics, pathology, treatment, and outcomes of primary cardiac sarcomas at a national referral center in Spain.Methods:We conducted a retrospective, observational, single-center study from February 2017 to December 2024. Patient data were collected from medical records, and a descriptive analysis was performed.Results:Twelve patients were identified (58% female; median age 43 years, range 13–76). Dyspnea was the most common symptom (8/12, 67%), and the right atrium was the most frequent tumor site (6/12, 50%). Angiosarcoma was the predominant histologic subtype (6/12, 50%). Seven patients had localized disease at diagnosis. Surgery was performed in six patients, with complete (R0) resection in two. Two patients received adjuvant chemotherapy, one underwent cardiac transplantation, and one received sequential chemo- and radiotherapy. All patients experienced tumor recurrence, with a median recurrence-free survival of 5 months (95% CI, 1.5–8.6). Median overall survival for localized disease was 22 months (95% CI, 16–28). Five patients were metastatic at diagnosis, and 11 of 12 developed metastases. Median progression-free survival for first-line therapy was 5.9 months (95% CI, 1.8–9.9), and median overall survival for advanced disease was 12 months (95% CI, 10–13.6).Conclusions:Complete surgical resection was rarely achieved, and recurrence was universal. Outcomes remained poor even for localized disease, highlighting the limited efficacy of current therapies and the need for improved multimodal treatment strategies.
背景/目的：原发性心脏肉瘤是一种罕见且侵袭性强的肿瘤。由于疾病特异性数据有限，其治疗通常参考其他类型肉瘤的证据。本研究旨在分析西班牙一家国家转诊中心收治的原发性心脏肉瘤患者的临床特征、病理学表现、治疗及预后。 方法：我们于2017年2月至2024年12月期间开展了一项回顾性、观察性、单中心研究。从医疗记录中收集患者数据并进行描述性分析。 结果：共纳入12例患者（女性占58%；中位年龄43岁，范围13-76岁）。呼吸困难是最常见的症状（8/12，67%），右心房是最常见的肿瘤部位（6/12，50%）。血管肉瘤是主要的组织学亚型（6/12，50%）。7例患者在诊断时为局限性病变。6例患者接受了手术治疗，其中2例实现了完全（R0）切除。2例患者接受了辅助化疗，1例接受了心脏移植，1例接受了序贯放化疗。所有患者均出现肿瘤复发，中位无复发生存期为5个月（95% CI，1.5-8.6）。局限性病变患者的中位总生存期为22个月（95% CI，16-28）。5例患者在诊断时已发生转移，12例患者中有11例最终出现转移。一线治疗的中位无进展生存期为5.9个月（95% CI，1.8-9.9），晚期疾病的中位总生存期为12个月（95% CI，10-13.6）。 结论：完全手术切除很少能够实现，且复发普遍存在。即使是局限性病变，预后仍然较差，这凸显了当前治疗方法的疗效有限，亟需改进多模式治疗策略。
肿瘤(癌症)患者之家