Primary tumors of the heart are uncommon, with roughly 75% benign and 25% malignant. Most of the malignant tumors are sarcomas and historically have had a very poor prognosis. These tumors tend to occur in young patients with a mean age of 40 years. Making a diagnosis of cardiac sarcoma can be difficult due to its rarity and the nature of the symptoms. For left heart sarcomas, almost all patients are symptomatic by the time the diagnosis has been made. Symptoms are dependent on the location and the extent of the tumor and are not related to tumor histology; similarly, histologic cell type has not been found to be related to prognosis in most studies. We therefore previously proposed a classification system for primary cardiac sarcoma based on anatomic location, dividing primary cardiac sarcoma into right heart, pulmonary artery and left heart sarcomas.
Left heart sarcoma presents a technical anatomic challenge: the left atrium, being the posterior heart chamber, allows somewhat limited access using routine surgical approaches. The role of chemotherapy or radiotherapy remains unclear and unproven, leaving complete surgical resection as the only mode of therapy with a proven survival benefit. Our review of the published literature showed frequent local recurrence and poor long-term survival in left heart sarcomas. Our hypothesis was that the left atrium had limited anatomic accessibility for large complex resections and reconstructions, and this led cardiac surgeons to do a more limited tumor removal with an increased chance of local recurrence and a detrimental effect on survival. To address this technical challenge, our group introduced the surgical technique of cardiac explantation, ex vivo tumor resection, cardiac reconstruction, and subsequent cardiac reimplantation or cardiac autotransplantation for left heart sarcoma in an attempt to improve the completeness of local resection, decrease local recurrence, and extend patient survival. This review discusses the approach of the cardiac sarcoma group at the Methodist DeBakey Heart & Vascular Center and the MD Anderson Cancer Center to the diagnosis and treatment of left heart sarcoma, as well as our current patient outcomes.