Abstract：
BACKGROUND: Cardiac sarcomas are rare and aggressive tumors with little known about the demographics, genetics, or treatment outcomes.
OBJECTIVE: The objectives of this study were to characterize the demographics, treatment modality, and survival associated with cardiac sarcomas and evaluate the potential for mutation-directed therapies.
METHODS: All cases from 2000 to 2018 of cardiac sarcoma were extracted from the SEER database. Genomic comparison utilized The Cancer Genome Atlas (TCGA) database, as well as reviews and re-analysis of past applicable genomic studies.
RESULTS: Cardiac sarcomas occurred most often in White patients, compared with national census data cardiac sarcomas occurred at a significantly higher rate in Asians. The majority of cases were undifferentiated (61.7%) and without distant metastases (71%). Surgery was the most common primary treatment modality and offered survival benefit (HR 0.391 (p = 0.001) that was most pronounced and sustained as compared to patients who received chemotherapy (HR 0.423 (p = 0.001) or radiation (HR 0.826 (p = 0.241) monotherapy. There was no difference in survival when stratified by race or sex; however, younger patients (< 50) had better survival. Genomics data on histologically undifferentiated cardiac sarcomas revealed a significant number were likely poorly differentiated pulmonary intimal sarcomas and angiosarcomas.
CONCLUSIONS: Cardiac sarcoma is a rare disease with surgery continuing to be a cornerstone of therapy followed by traditional chemotherapy. Case studies have indicated the potential for therapies directed to specific genetic aberrations to improve survival for these patients and utilization of next-generation sequencing (NGS) will help improve both classification and these therapies for cardiac sarcoma patients.
OBJECTIVE: The objectives of this study were to characterize the demographics, treatment modality, and survival associated with cardiac sarcomas and evaluate the potential for mutation-directed therapies.
METHODS: All cases from 2000 to 2018 of cardiac sarcoma were extracted from the SEER database. Genomic comparison utilized The Cancer Genome Atlas (TCGA) database, as well as reviews and re-analysis of past applicable genomic studies.
RESULTS: Cardiac sarcomas occurred most often in White patients, compared with national census data cardiac sarcomas occurred at a significantly higher rate in Asians. The majority of cases were undifferentiated (61.7%) and without distant metastases (71%). Surgery was the most common primary treatment modality and offered survival benefit (HR 0.391 (p = 0.001) that was most pronounced and sustained as compared to patients who received chemotherapy (HR 0.423 (p = 0.001) or radiation (HR 0.826 (p = 0.241) monotherapy. There was no difference in survival when stratified by race or sex; however, younger patients (< 50) had better survival. Genomics data on histologically undifferentiated cardiac sarcomas revealed a significant number were likely poorly differentiated pulmonary intimal sarcomas and angiosarcomas.
CONCLUSIONS: Cardiac sarcoma is a rare disease with surgery continuing to be a cornerstone of therapy followed by traditional chemotherapy. Case studies have indicated the potential for therapies directed to specific genetic aberrations to improve survival for these patients and utilization of next-generation sequencing (NGS) will help improve both classification and these therapies for cardiac sarcoma patients.
摘要：
背景：心脏肉瘤是罕见的侵袭性肿瘤，对人口统计学知之甚少，遗传学,或治疗结果。
目的：本研究的目的是表征人口统计学，治疗方式，以及与心脏肉瘤相关的生存率，并评估突变指导治疗的潜力。
方法：从SEER数据库中提取2000年至2018年的所有心脏肉瘤病例。基因组比较利用癌症基因组图谱(TCGA)数据库，以及对过去适用的基因组研究的回顾和重新分析。
结果：心脏肉瘤最常见于白人患者，与全国人口普查数据相比，亚洲人的心脏肉瘤发生率明显更高。大多数病例未分化（61.7％），无远处转移（71％）。手术是最常见的主要治疗方式，与接受化疗（HR0.423（p=0.001）或放疗（HR0.826（p=0.241））的患者相比，其生存获益（HR0.391（p=0.001）最明显且持续。按种族或性别分层时，生存率没有差异；然而，年轻患者（<50）有更好的生存。组织学上未分化的心脏肉瘤的基因组学数据显示，很大一部分可能是低分化的肺内膜肉瘤和血管肉瘤。
结论：心脏肉瘤是一种罕见疾病，手术继续是传统化疗后的治疗基础。案例研究表明，针对特定遗传畸变的疗法有可能改善这些患者的生存率，并且利用下一代测序（NGS）将有助于改善心脏肉瘤患者的分类和这些疗法。
目的：本研究的目的是表征人口统计学，治疗方式，以及与心脏肉瘤相关的生存率，并评估突变指导治疗的潜力。
方法：从SEER数据库中提取2000年至2018年的所有心脏肉瘤病例。基因组比较利用癌症基因组图谱(TCGA)数据库，以及对过去适用的基因组研究的回顾和重新分析。
结果：心脏肉瘤最常见于白人患者，与全国人口普查数据相比，亚洲人的心脏肉瘤发生率明显更高。大多数病例未分化（61.7％），无远处转移（71％）。手术是最常见的主要治疗方式，与接受化疗（HR0.423（p=0.001）或放疗（HR0.826（p=0.241））的患者相比，其生存获益（HR0.391（p=0.001）最明显且持续。按种族或性别分层时，生存率没有差异；然而，年轻患者（<50）有更好的生存。组织学上未分化的心脏肉瘤的基因组学数据显示，很大一部分可能是低分化的肺内膜肉瘤和血管肉瘤。
结论：心脏肉瘤是一种罕见疾病，手术继续是传统化疗后的治疗基础。案例研究表明，针对特定遗传畸变的疗法有可能改善这些患者的生存率，并且利用下一代测序（NGS）将有助于改善心脏肉瘤患者的分类和这些疗法。
