The incidence of primary cardiac neoplasm is approximately 0.0001% to 0.030%, 25% are malignant, and cardiac angiosarcoma is the most common pathological type. Cardiac angiosarcomas usually originate in the right atrium, and the most common sites for metastasis are the lung and the pericardium. Transthoracic echocardiography is usually the initial diagnostic tool for cardiac tumors, which has a sensitivity of 93% for detecting cardiac masses. Kupsky et al have reported that the absence of a stalk was a universal finding that may help distinguish angiosarcoma from benign, primarily pedunculated such as myxom and papillary fibroelastoma.[10,11] CT scanning is a valuable supplement tool to echocardiography. Magnetic resonance imaging (MRI) is often used to detect tissue characterization and tumor infiltration.18F-FDG PET-CT could be used to noninvasively determine malignant tumors with a sensitivity of 100%. However, MRI, CT, or PET-CT cannot replace biopsy for the final diagnosis. Literatures have ever been reported that pericardial fluid analysis in 15 cases (88%), and the results were negative for malignancy in all. Pericardiocentesis frequently yields bloody or necrotic tissues, cytology is often negative involvement.[14,15] Just as we have done, pericardiocentesis and CT-guided biopsy were all failure. Cardiac angiosarcoma was diagnosed after exploratory thoracotomy. Immunohistochemistry staining of CD31 and CD34 confirmed endothelial origin. Ki67 was used as a prognostic factor. High values (≥10%) have been statistically correlated with poor survival outcome.
Surgical resection remains the first-line treatment of choice. Unfortunately, many patients lost their operating opportunities when found it. Adjuvant therapies, including chemotherapy and radiotherapy are all debatable. No randomized controlled trials have been performed to provide evidence to these adjuvant therapies. With regard to targeted therapy, Imatinib is not found to be an active agent against advanced sarcoma in phase II trials. Sorafenib exerts activity against angiosarcoma, with 3.8 and 14.9 months progression-free survival and overall survival, respectively. Bevacizumab is effective for angiosarcoma with 57% (13/23) patients showing a response and no progression. This patient experienced chemotherapy with docetaxel which has been reported antitumor activity in angiosarcoma.[5–7] The efficacy was evaluated as progression after 2 cycles. Bevacizumab was combined with chemotherapy for 3 months to this patient. Unfortunately, the tumor was still in progress. She survived for 10 months and died. The literatures have reported that the prognosis of cardiac angiosarcoma is generally poor, with survival ranging from 6 to 12 months after the diagnosis has been established. Although, this case responds poorly to chemotherapy and targeted therapy, some of limited cases, after radical resection, a multidisciplinary approach with adjuvant chemotherapy, radiotherapy, and targeted therapy may improve overall survival even reaching 33 months.[21–23]
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