Abstract 12847: Left Atrial Cardiac Angiosarcoma: A Systematic Review of Case Reports

Abstract

Background: Primary cardiac angiosarcoma is a rare endothelial cell tumor, most commonly (78-93%) found in the right atrium. However, rare instances of left atrial angiosarcoma have been noted with varied clinical presentation and fatal prognosis. Methods: A systematic search using PUBMED, SCOPUS and WEB OF SCIENCE was undertaken to identify case reports on left atrial angiosarcomas through June 2021. The data on patient demographics, presentation, diagnostics imaging, management, and outcomes were analyzed using descriptive statistics. Results: Of 19 case reports (Table 1) , left atrial angiosarcoma was more prevalent among females (63%, 12/19). The mean age of cases was 59.26± 12.21 years. Progressive exertional dyspnea (11/19, 58%) and chest pain (5/19, 26%) were the most common presenting symptoms. Myocardial ischemia was the initial presentation in 11% of cases (2/19), congestive heart failure in 26%(5/19) while cerebrovascular events were reported in 11% of cases (2/19). Transesophageal echocardiography and transthoracic echocardiography were the mainstay imaging modalities used for diagnoses. Most cases were diagnosed intraoperatively (16/19, 84.2%) with one case being diagnosed through skin biopsy of a cutaneous metastatic nodule (Table 2) . Surgical excision followed by chemotherapy was the treatment of choice in most cases. With 26% (5/19) metastatic prevalence, death was reported in 58% of the patients (11/19). Conclusions: Primary left atrial cardiac angiosarcoma remains an elusive diagnosis with nonspecific constitutional symptoms. Even with advanced diagnostic modalities, the tumor is mostly detected at an advanced and incurable stage with a grave prognosis and high mortality rate. A deeper understanding of the spectrum of clinical presentation with a high index of suspicion can help clinicians in early diagnosis, which is imperative to prolonged survival.