PDF(Pubmed)
Abstract：
Primary cardiac sarcomas are rare and their clinicopathologic features are heterogeneous. Among them, particularly intimal sarcoma is a diagnostic challenge due to nonspecific histologic features. Recently, MDM2 amplification reported to be a characteristic genetic event in the intimal sarcoma. In this study, we aimed to identify the types and incidence of primary cardiac sarcomas that occurred over 25 years in tertiary medical institutions, and to find clinicopatholgical significance through reclassification of diagnoses using additional immunohistochemistry (IHC).
We reviewed the primary cardiac sarcoma cases between January 1993 and June 2018 at Asan Medical Center, South Korea, with their clinicopathologic findings, and reclassified the subtypes, especially using IHC for MDM2 and then, analyzed the significance of prognosis.
Forty-eight (6.8%) cases of a primary cardiac sarcoma were retrieved. The tumors most frequently involved the right atrium (n = 25, 52.1%), and the most frequent tumor subtype was angiosarcoma (n = 23, 47.9%). Seven cases (53.8%) were newly reclassified as an intimal sarcoma by IHC for MDM2. Twenty-nine (60.4%) patients died of disease (mean, 19.8 months). Four patients underwent a heart transplantation and had a median survival of 26.8 months. This transplantation group tended to show good clinical outcomes in the earlier stages, but this was not statistically significant (p = 0.318). MDM2 positive intimal sarcoma showed the better overall survival (p = 0.003) than undifferentiated pleomorphic sarcoma. Adjuvant treatment is beneficial for patient survival (p < 0.001), particularly in angiosarcoma (p < 0.001), but not in intimal sarcoma (p = 0.154).
Our study supports the use of adjuvant treatment in primary cardiac sarcoma, as it was associated with a significantly better overall survival rate. Further consideration of tumor histology may be important in determining the optimal use of adjuvant treatment for different types of sarcomas. Therefore, accurate diagnosis by MDM2 test is important condsidering patient\'s prognosis and treatment.
We reviewed the primary cardiac sarcoma cases between January 1993 and June 2018 at Asan Medical Center, South Korea, with their clinicopathologic findings, and reclassified the subtypes, especially using IHC for MDM2 and then, analyzed the significance of prognosis.
Forty-eight (6.8%) cases of a primary cardiac sarcoma were retrieved. The tumors most frequently involved the right atrium (n = 25, 52.1%), and the most frequent tumor subtype was angiosarcoma (n = 23, 47.9%). Seven cases (53.8%) were newly reclassified as an intimal sarcoma by IHC for MDM2. Twenty-nine (60.4%) patients died of disease (mean, 19.8 months). Four patients underwent a heart transplantation and had a median survival of 26.8 months. This transplantation group tended to show good clinical outcomes in the earlier stages, but this was not statistically significant (p = 0.318). MDM2 positive intimal sarcoma showed the better overall survival (p = 0.003) than undifferentiated pleomorphic sarcoma. Adjuvant treatment is beneficial for patient survival (p < 0.001), particularly in angiosarcoma (p < 0.001), but not in intimal sarcoma (p = 0.154).
Our study supports the use of adjuvant treatment in primary cardiac sarcoma, as it was associated with a significantly better overall survival rate. Further consideration of tumor histology may be important in determining the optimal use of adjuvant treatment for different types of sarcomas. Therefore, accurate diagnosis by MDM2 test is important condsidering patient\'s prognosis and treatment.
摘要：
背景：原发性心脏肉瘤是罕见的，其临床病理特征是不均匀的。其中,由于非特异性组织学特征,特别是内膜肉瘤是一种诊断挑战.最近,据报道，MDM2扩增是内膜肉瘤中的特征性遗传事件。在这项研究中,我们的目的是确定在三级医疗机构中发生超过25年的原发性心脏肉瘤的类型和发病率，并通过使用额外的免疫组织化学（IHC）对诊断进行重新分类来发现临床病理意义。
方法：我们回顾了1993年1月至2018年6月在Asan医学中心的原发性心脏肉瘤病例，韩国,根据他们的临床病理发现，并重新分类了亚型，特别是使用IHC对MDM2，然后，分析预后的意义。
结果：检索到48例（6.8％）原发性心脏肉瘤。肿瘤最常累及右心房（n=25，52.1%），最常见的肿瘤亚型是血管肉瘤（n=23，47.9％）。通过IHC对MDM2新将7例（53.8％）重新分类为内膜肉瘤。29名(60.4%)患者死于疾病(平均，19.8个月）。四名患者接受了心脏移植，中位生存期为26.8个月。该移植组倾向于在早期阶段显示良好的临床结果，但这没有统计学意义(p=0.318).MDM2阳性内膜肉瘤显示出比未分化多形性肉瘤更好的总生存期（p=0.003）。辅助治疗对患者生存有益（p<0.001），特别是在血管肉瘤中（p<0.001），但不在内膜肉瘤中（p=0.154）。
结论：我们的研究支持在原发性心脏肉瘤中使用辅助治疗，因为它与明显更好的总体生存率相关。进一步考虑肿瘤组织学对于确定不同类型肉瘤的辅助治疗的最佳使用可能很重要。因此,MDM2检测的准确诊断是考虑患者预后和治疗的重要因素。
方法：我们回顾了1993年1月至2018年6月在Asan医学中心的原发性心脏肉瘤病例，韩国,根据他们的临床病理发现，并重新分类了亚型，特别是使用IHC对MDM2，然后，分析预后的意义。
结果：检索到48例（6.8％）原发性心脏肉瘤。肿瘤最常累及右心房（n=25，52.1%），最常见的肿瘤亚型是血管肉瘤（n=23，47.9％）。通过IHC对MDM2新将7例（53.8％）重新分类为内膜肉瘤。29名(60.4%)患者死于疾病(平均，19.8个月）。四名患者接受了心脏移植，中位生存期为26.8个月。该移植组倾向于在早期阶段显示良好的临床结果，但这没有统计学意义(p=0.318).MDM2阳性内膜肉瘤显示出比未分化多形性肉瘤更好的总生存期（p=0.003）。辅助治疗对患者生存有益（p<0.001），特别是在血管肉瘤中（p<0.001），但不在内膜肉瘤中（p=0.154）。
结论：我们的研究支持在原发性心脏肉瘤中使用辅助治疗，因为它与明显更好的总体生存率相关。进一步考虑肿瘤组织学对于确定不同类型肉瘤的辅助治疗的最佳使用可能很重要。因此,MDM2检测的准确诊断是考虑患者预后和治疗的重要因素。
