O1-10-2 - Patient Characteristics and Management of Primary Cardiac Angiosarcoma: National Cancer Center Hospital Experience

Abstract

Background: Primary cardiac angiosarcomas are extremely rare and poorly understood. We reviewed the National Cancer Center Hospital (NCCH) experience of 7 patients with primary cardiac angiosarcoma to identify a preferred diagnostic and management strategy and evaluate the role of adjunctive therapy.Methods: We reviewed the patient database of NCCH to identify patients with primary cardiac angiosarcoma diagnosed in between 2008 and 2012. We identified 7 patients and reviewed the details of their symptoms, treatments and outcomes.Results: Of the 7 patients, 3 were male and 4 were female. The median age was 42 years old (range 34-60). The most common symptom of the patients at the first visit was dyspnea on exertion (n = 5) and peripheral coldness (n = 2), and all patients had pericardial effusion. In all cases, tumor originated in the right atrium. Four patients had localized disease and 3 patients had distant metastasis. Surgical resection was performed in 3 patients with localized disease. Complete resection was achieved in 2 patients, but all patients who received surgical resection had disease recurrence within 1 year. All patients with unresectable, metastatic or recurrent disease received systemic chemotherapy. They received weekly paclitaxel as first-line chemotherapy. Two of 7 patients (28.6%) achieved partial response (PR) and 4 of 7 patients presented stable disease (SD) with weekly paclitaxel. Median progression free survival was 4.8 months and median overall survival was 13 months.Conclusion: Primary cardiac angiosarcomas remain rare but lethal disease. The treatment response with paclitaxel for primary cardiac angiosarcoma in our institution is similar to the previously reported response in patients with angiosarcoma. Multidisciplinary therapy containing surgical resection and systemic chemotherapy may improve the clinical outcome of primary cardiac angiosarcoma.