Vaporciyan Ara, Reardon Michael J
The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Methodist Debakey Cardiovasc J. 2010 Jul-Sep;6(3):44-8. doi: 10.14797/mdcj-6-3-44.
Primary cardiac tumors are unusual, and primary cardiac sarcomas constitute a rare subset of these. In cardiac sarcoma, unlike many malignancies, the histologic cell type does appear to affect the treatment options or prognosis in a significant way. The presenting symptoms, treatment options and, indeed, prognosis are largely controlled by the tumor's anatomic location. We have proposed a classification system based on anatomic location that divides cardiac sarcoma into left heart, right heart and pulmonary artery sarcomas. In our experience, right heart sarcoma tends to be bulky, grow in a more exophitic manner, be more infiltrative, and metastasize earlier than left heart or pulmonary artery sarcoma. Right heart sarcoma also presents less often in congestive heart failure or with compromised hemodynamic status than left heart and pulmonary artery sarcoma, which are usually highly symptomatic at presentation. The prognosis for right heart sarcoma without surgery is dismal. Complete surgical resection remains the goal of therapy and the only treatment modality shown to increase survival. Complete surgical resection is complicated both by the bulky infiltrative nature of right heart sarcoma and the high incidence of metastatic disease at presentation. The current approach of our cardiac sarcoma group to right heart sarcoma has been to begin neoadjuvant chemotherapy once a definitive tissue diagnosis of sarcoma is achieved. After 4 to 6 rounds of chemotherapy, the patient is considered for surgical resection. This standardized treatment has been approved in our IRB protocol: a clinical trial to assess the safety and Efficacy of a novel radical tumor reSection Procedure used in conjunction with nEoadjuvant chemotheRapy to treat malignant primary right heart cardiac tumOrs - the ESPERO trial. This protocol is designed to compare our existing 24 index cases of surgical resection of right heart sarcoma using a nonstandardized treatment plan, with routine neoadjuvant chemotherapy, and a standardized treatment plan to see if the rate of microscopically complete resection can be improved from its current level of 33% and if this will improve patient survival. In this reveiw, we will discuss the experience with right heart sarcoma.
原发性心脏肿瘤较为罕见，原发性心脏肉瘤则是其中更为罕见的一类。在心脏肉瘤中，与许多恶性肿瘤不同，组织学细胞类型似乎并未对治疗方案或预后产生显著影响。其临床表现、治疗方案以及预后在很大程度上受肿瘤解剖位置的控制。我们提出了一种基于解剖位置的分类系统，将心脏肉瘤分为左心、右心和肺动脉肉瘤。根据我们的经验，右心肉瘤往往体积较大，呈更外向性生长，浸润性更强，且比左心或肺动脉肉瘤更早发生转移。与通常在就诊时具有高度症状性的左心和肺动脉肉瘤相比，右心肉瘤在充血性心力衰竭或血流动力学状态受损时的表现也较少见。未经手术治疗的右心肉瘤预后很差。完整的手术切除仍然是治疗的目标，也是唯一被证明能提高生存率的治疗方式。右心肉瘤体积大且具有浸润性，同时就诊时转移疾病的发生率高，这使得完整的手术切除变得复杂。我们心脏肉瘤治疗团队目前针对右心肉瘤的方法是，一旦获得肉瘤的确切组织诊断，就开始进行新辅助化疗。经过4至6轮化疗后，考虑对患者进行手术切除。这种标准化治疗已在我们的机构审查委员会方案中获得批准：一项评估一种新型根治性肿瘤切除手术联合新辅助化疗治疗原发性右心恶性心脏肿瘤的安全性和有效性的临床试验——ESPERO试验。该方案旨在将我们现有的24例采用非标准化治疗方案并结合常规新辅助化疗进行右心肉瘤手术切除的索引病例，与标准化治疗方案进行比较，以观察显微镜下完全切除率是否能从目前 的33%提高，以及这是否会改善患者生存率。在本综述中，我们将讨论右心肉瘤的治疗经验。
