PRIMARY RIGHT ATRIAL CARDIAC ANGIOSARCOMA INITIALLY PRESENTING AS SYNCOPE

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Primary cardiac Angiosarcomas are extremely rare, with incidence of about 0.056% (1). These tumors pose significant diagnostic dilemma due to rarity and varied clinical features at presentation. We report our experience while treating a patient with primary right atrial cardiac angiosarcoma who initially presented with syncope. CASE PRESENTATION: A 45-year-old Spanish woman presented to the emergency room after an episode of syncope. She reported having shortness of breath, sweating and fatigue for 2 weeks prior to the incident. A chest radiograph revealed an enlarged cardiac silhouette, and computed tomography (CT) showed an enhancing mass along the right cardiac border, measuring 4.6 cm by 5.9 cm (Image 1). An Echocardiogram showed a right atrial mass (Image 2) with significant pericardial effusion. Percutaneous CT guided biopsy showed a vascularized mass and the patient underwent an exploratory surgery and removal of tumor. Cytological analysis of the mass failed to provide a diagnosis. 11 months from the surgery, the patient presented with shortness of breath and intense right leg pain. A CT scan of the chest revealed aggressive recurrence of the tumor with cardiac/mediastinal mass invading the right atrium, the right ventricle, the inferior vena cava, the main pulmonary trunk, right middle lobe bronchus and the aorta. There was extensive mediastinal adenopathy as well as right sided pleural effusion (Image 3). There was another mass seen in the right femur and it was biopsied, with a histo-pathological analysis confirming metastatic angiosarcoma. She was started on paclitaxel with a dose of 80 mg/m2 weekly, and tolerated it well. She continues to follow up with Oncology. DISCUSSION: Primary cardiac Angiosarcomas are rare tumors and are usually metastatic at the time of diagnosis. Cardiac Angiosarcomas can present with varying symptoms, these clinical features depend on the tumor size, location, and the extent of the local or distant spread. This tumor affects the right atrium commonly, a feature that is distinct from Atrial Myxoma which usually affects the left atrium. This knowledge can aid clinicians in suspecting an aggressive disease early. Resection, in combination with adjuvant treatment including chemotherapy, radiotherapy, and molecular targeted therapies give the best chance for prolonging survival. Chemotherapeutic regimens include anthracyclines, taxanes or ifosfamide (2). Doxyrubicin based treatments although well known for their cardiotoxic adverse effects have been used as the standard treatment for locally advanced and metastatic disease (3) often with local radiotherapy. CONCLUSIONS: In conclusion, primary Cardiac Angiosarcomas have a rapidly progressive and fatal course with mean survival of 6-11 months from diagnosis. Early diagnosis, with multimodality approach tailored to each patient gives the best chance of prolonged survival. Reference #1: Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. 1993 Oct;117(10):1027-31 Reference #2: Schur S, Hamacher R, Brodowicz T. Pazopanib in Primary Cardiac Angiosarcoma of the Right Atrium: A Case Report. Case Reports in Oncology 2016;9(2):363–367. Reference #3: Penel N, Bui BN, Bay J-O, et al. Phase II Trial of Weekly Paclitaxel for Unresectable Angiosarcoma: The ANGIOTAX Study. Journal of Clinical Oncology 2008;26(32):5269–5274. DISCLOSURES: No relevant relationships by Yizhak Kupfer, source=Web Response No relevant relationships by Namrita Malhan, source=Web Response No relevant relationships by Ravikaran Patti, source=Web Response No relevant relationships by Vignesh Ponnusamy, source=Web Response No relevant relationships by Ankur Sinha, source=Web Response No relevant relationships by Parita Soni, source=Web Response