The incidence of primary cardiac tumor is reporter as 0.0017%-0.033% and sarcomas make up for the majority of them. However, the recognition of a primary cardiac sarcoma can be hard and progress rapidly in a short time. The prognosis for patients with surgery only is poor with a mean survival of 3 months to 1 year. Although most angiosarcomas are resistant to both chemotherapy the radiation, it’s meaningful to adopt them due to the high possibilities of the further metastases. It should be treated as early as possible if suspected in imaging examinations and symptoms, however there’s no specific clinical symptoms to detect it on time, dyspnea is the most common feature followed by chest pain as we mentioned in the case above. Cardiac angiosarcoma can grow in any part of heart, usually originates from the right atrium, rarely occurs in pericardium, and the most common sites of metastasis are the lungs and bones.
Non-invasive imaging examinations such as echocardiography, CT and MRI, has contributed to the early diagnosis or detection of the tumors. The common manifestation of pericardial angiosarcoma in echo including the hemorrhagic pericardial effusion, abnormal mass attaching to the pericardium which usually thickened. The echo could not only provide the specific location, morphology, internal echo and attachment site of the tumor, but also observe the movement of the mass in the cavity and the degree of obstruction dynamically, the relationship between the neoplasm and other cavity structures, evaluate the secondary hemodynamic changes to supply the reliable first-hand information for clinical diagnosis and treatment. The identify of benign and malignant tumors was preliminary based on the image characteristics, such as inhomogeneous masses with poor border definition, invasion into extracardiac structures, or the presence of a pericardial effusion, but it’s still difficult to differentiate benign and malignant tumors by ultrasound only, and the large amount of pericardial effusion may lead to missed diagnosis of the lesion occasional.
CT and enhanced CT are the most practical and valuable methods to show the size and location of the tumor. Primary cardiac angiosarcoma often presents as homogeneous or inhomogeneous density on unenhanced CT scans and heterogeneous centripetal enhancement on enhanced images. Delayed imaging could offer more information about the visualization of the mass given late contrast enhancement. CT imaging can also provide more detailed information such as pericardial thickening and effusion, the scope of tumor invasion, and the relationship between the lesion and surrounding structures to guide the clinical operation. With percardial involvement, there is ussally “sheet-like” thickening due to the distribution of mass cells. In cases with percardial diffusion, linear contrast material enhancement along vascular lakes have described as a “sunray” appearance. But it is not reliable for prediction of the degree of malignancy of mass comparing with MRI due to the poorer histological resolution.
MRI examination has better tissue specificity and soft tissue contrast rate, more accuracy in the size, location and shape of cardiac tumor, which has great significance in judging the degree of benign and malignant invasion of neoplasm. The lesion often shows as heterogeneous intensity, with hyperintense hemorrhage foci on both T1-and T2-weighted images compared with the myocardium. It is recommended as the evaluation of heart function, surgical assessment, and postoperative follow-up. PET/CT reveals abnormal FDG activity in part of the pericardium. It may help in the diagnosis in differentiating the benign from a malignant lesion with a 100% sensitivity and about 86% specificity, using a cutoff of SUVmax of 3.5. So it can be used for early detection of tumor and determining whether metastasis occurs.
In principle, cardiac tumor should be operated within a time limit once diagnosed to avoid arterial embolism and (or) sudden death, and sometimes emergency operation is needed. Surgical indications should be strictly grasped for patients with poor preoperative cardiac function and distant metastasis. Generally, the tumor can be resected under cardiopulmonary bypass, the mass should be completely resected as well the endocardium and myocardial tissue at the root and attachment of the tumor pedicle. For the defects caused by surgery, the autologous pericardium or artificial patch can be used for repair, and valve replacement when necessary. At present, the treatment of cardiac malignant tumors tends to the multidisciplinary comprehensive treatment of surgery with radiotherapy and chemotherapy followed by.