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Cardiac Angiosarcoma Management and Outcomes in the Modern Era
*Nicole J Look Hong1, *Prakash K Pandalai2, *Jason L Hornick3, *James E Butrynski4, *Elizabeth H Baldini5, Chandrajit P Raut1
1Division of Surgical Oncology, Brigham and Women’s Hospital/ Dana-Farber Cancer Institute, Center for Sarcoma and Bone Oncology, Division of Surgical Oncology, Boston, MA;2Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD;3Division of Pathology, Brigham and Women’s Hospital/Dana-Farber Cancer Institute, Center for Sarcoma and Bone Oncology/Harvard Medical School, Boston, MA;4Division of Medical Oncology, Brigham and Women’s Hospital/Dana-Farber Cancer Institute, Center for Sarcoma and Bone Oncology/Harvard Medical School, Boston, MA;5Division of Radiation Oncology,Brigham and Women’s Hospital/Dana-Farber Cancer Institute, Center for Sarcoma and Bone Oncology/Harvard Medical School, Boston, MA
Objective: To identify the clinicopathologic characteristics, treatments, and outcomes of a series of patients with primary cardiac angiosarcomas (AS).
Design: Retrospective case series.
Setting: Tertiary referral center with dedicated multidisciplinary sarcoma clinic.
Patients: Consecutive patients with institutionally confirmed pathologic diagnosis of cardiac AS from January 1990 - December 2009 were reviewed.
Interventions: Multidisciplinary cancer care.
Main Outcome Measures: Patient demographics, histologic tumor characteristics, management strategies, disease response, and survival were extracted.
Results: Seventeen patients (82% male, mean age at diagnosis 37 years (range 24-58 years)) were reviewed. Sixteen patients (94%) had AS originating in the right atrium. The predominant presenting symptom was pericardial effusion and tamponade (65%). At diagnosis, six patients (35%) had locally advanced disease and 11 patients (65%) had distant metastatic disease. Initial treatment strategies included attempted surgical resection (41 %), chemotherapy (47%), and radiation therapy (6%). Of the 6 patients with locally advanced AS at diagnosis, 3 underwent macroscopically complete resection, 1 underwent macroscopically incomplete resection, 1 underwent chemotherapy followed by surgery and intraoperative radiation therapy, and 1 did not survive until planned radiation. Two of 5 patients with locally advanced disease and attempted resection developed local recurrences at 14 and 50 months, respectively, and all progressed to distant metastatic disease. Median follow-up was 8 months. Median overall survival was 14 months. Patients with locally advanced and metastatic AS at presentation had median survivals of 23 and 12 months, respectively (p-value 0.081).
Conclusions: Cardiac AS should be considered a systemic disease. Cardiac AS presenting with distant metastatic disease portends a poor prognosis. Patients with localized AS at presentation trend towards longer survival but are still at high risk for distant failure.
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