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Abstract：
BACKGROUND: Primary cardiac angiosarcoma(PCA) has a low incidence rate and poor prognosis. Currently, no unified clinical treatment standards are available.
METHODS: We report the case of a 48-year-old man presenting chest tightness, breathlessness, and dyspnea. Imaging and postoperative histopathologic studies confirmed PCA and that the tumor had invaded the entire right atrium. The patient developed progressive disease (PD) during postoperative radiotherapy. We used immunotherapy combined with targeted therapy based on the results of molecular profile and evaluation of tertiary lymphoid structures (TLSs) and programmed cell death-ligand 1 (PD-L1). After treatment, the metastatic lymph nodes of the patient were reduced to a certain extent, indicating that combination therapy was effective.
CONCLUSIONS: To the best of our knowledge, this is the first report of radiotherapy combined with anti-PD-1 and tyrosine kinase inhibitors(TKI) for PCA. In addition, this is the first report on immunotherapy for PCA based on new evaluation methods, including TLSs, PD-L1, and genomic profile.
METHODS: We report the case of a 48-year-old man presenting chest tightness, breathlessness, and dyspnea. Imaging and postoperative histopathologic studies confirmed PCA and that the tumor had invaded the entire right atrium. The patient developed progressive disease (PD) during postoperative radiotherapy. We used immunotherapy combined with targeted therapy based on the results of molecular profile and evaluation of tertiary lymphoid structures (TLSs) and programmed cell death-ligand 1 (PD-L1). After treatment, the metastatic lymph nodes of the patient were reduced to a certain extent, indicating that combination therapy was effective.
CONCLUSIONS: To the best of our knowledge, this is the first report of radiotherapy combined with anti-PD-1 and tyrosine kinase inhibitors(TKI) for PCA. In addition, this is the first report on immunotherapy for PCA based on new evaluation methods, including TLSs, PD-L1, and genomic profile.
摘要：
背景：原发性心脏血管肉瘤（PCA）发病率低，预后差。目前,没有统一的临床治疗标准。
方法：我们报告一例48岁男性胸闷，呼吸困难,和呼吸困难。影像学和术后组织病理学研究证实了PCA，并且肿瘤已侵入整个右心房。患者在术后放疗期间发展为进行性疾病（PD）。我们根据分子谱和三级淋巴结构（TLSs）和程序性细胞死亡配体1（PD-L1）的评估结果，将免疫治疗与靶向治疗相结合。治疗后,患者的转移性淋巴结在一定程度上减少，表明联合治疗是有效的。
结论：据我们所知，这是放疗联合抗PD-1和酪氨酸激酶抑制剂(TKI)治疗PCA的首次报道.此外,这是关于基于新评估方法的PCA免疫治疗的第一份报告，包括TLS，PD-L1和基因组谱。
方法：我们报告一例48岁男性胸闷，呼吸困难,和呼吸困难。影像学和术后组织病理学研究证实了PCA，并且肿瘤已侵入整个右心房。患者在术后放疗期间发展为进行性疾病（PD）。我们根据分子谱和三级淋巴结构（TLSs）和程序性细胞死亡配体1（PD-L1）的评估结果，将免疫治疗与靶向治疗相结合。治疗后,患者的转移性淋巴结在一定程度上减少，表明联合治疗是有效的。
结论：据我们所知，这是放疗联合抗PD-1和酪氨酸激酶抑制剂(TKI)治疗PCA的首次报道.此外,这是关于基于新评估方法的PCA免疫治疗的第一份报告，包括TLS，PD-L1和基因组谱。
