Blackmon Shanda H, Reardon Michael J
Methodist DeBakey Heart & Vascular Center, Houston, Texas, USA.
Methodist Debakey Cardiovasc J. 2010 Jul-Sep;6(3):38-43. doi: 10.14797/mdcj-6-3-38.
Primary heart tumors are rare, and malignant primary heart tumors are only a small subset of these. Most primary malignant tumors are sarcomas arising from the cells of the structural elements of the heart such as blood vessels, muscle, connective tissue, fat and even bone. Unlike most malignancies, where cell type often dictates treatment choices and prognosis and is used for classification, the histology in primary cardiac sarcoma plays little role in determining therapeutic options or prognosis. We have found that anatomic location within the heart is the major determining factor in clinical presentation, treatment options and prognosis in cardiac sarcoma. Therefore, we accordingly classify primary cardiac sarcomas into right heart sarcomas, left heart sarcomas and pulmonary artery (PA) sarcomas. Since the first autopsy report of a primary PA sarcoma in 1923, there have been fewer than 250 cases reported in the English literature. Most of these reports have been single autopsy or case reports, and patient prognosis has generally been dismal. Since few institutions and even fewer individual physicians acquire much exposure to this disease, the diagnostic and treatment approaches have remained unresolved. Our cardiac sarcoma group working at the Methodist DeBakey Heart & Vascular Center and the MD Anderson Cancer Center has undertaken a systematic study of this disease, and operated on 9 patients using a radical resection with curative intent and multimodality approach. Based on this work, we have suggested a diagnostic strategy, treatment approach and staging system for primary PA sarcoma. A substantial improvement in patient survival over historical controls has also been demonstrated and will be discussed in this review.
原发性心脏肿瘤较为罕见，而恶性原发性心脏肿瘤只是其中的一小部分。大多数原发性恶性肿瘤是肉瘤，起源于心脏结构元件的细胞，如血管、肌肉、结缔组织、脂肪甚至骨骼。与大多数恶性肿瘤不同，细胞类型通常决定治疗选择和预后并用于分类，而原发性心脏肉瘤的组织学在确定治疗方案或预后方面作用不大。我们发现心脏内的解剖位置是心脏肉瘤临床表现、治疗方案和预后的主要决定因素。因此，我们相应地将原发性心脏肉瘤分为右心肉瘤、左心肉瘤和肺动脉（PA）肉瘤。自1923年首次报道原发性PA肉瘤的尸检报告以来，英文文献中报道的病例不到250例。这些报告大多是单次尸检或病例报告，患者预后通常很差。由于很少有机构甚至很少有医生接触到这种疾病，其诊断和治疗方法仍未得到解决。我们在卫理公会德巴基心脏与血管中心和MD安德森癌症中心工作的心脏肉瘤研究小组对这种疾病进行了系统研究，并对9例患者进行了根治性切除并采用多模式方法进行手术。基于这项工作，我们提出了原发性PA肉瘤的诊断策略、治疗方法和分期系统。与历史对照相比，患者生存率也有显著提高，本文将对此进行讨论。
