CONGRESS ABSTRACT

Cardiac angiosarcoma is a rare endothelial cell tumor, the most common primary malignant tumor of the heart, characterized by an aggressive permeating growth within the surrounding myocardial wall. In most cases, it occurs in the right atrium and can infiltrate into neighboring structures and spread distantly. It is associated with a poor prognosis due to its aggressive nature and high rates of local recurrence and systemic metastases. There is currently no standard treatment approach. When localized, surgical resection leads to the best chance of long-term survival. However, surgery is often technically challenging, particularly since these tumors occur within the heart or in close proximity to the great vessels. Combined modality therapies have emerged as a potential way to achieve better survival outcomes: adjuvant radiotherapy, as well as neoadjuvant or adjuvant chemotherapy. Upfront chemotherapy can shrink the tumor mass, allowing for radical surgery. We report a case of a primary angioscarcoma in a 53-year-old man, localized at the level of the right atrium with axial dimensions of at least 5 cm, which extended into the superior vena cava occupying its lumen and at the pericardial level, with reactive pericardial effusion (see panels a and b). The patient also had secondary pulmonary and bone lesions. The subjects underwent transthoracic and transesophageal echocardiography, cardiac magnetic resonance, chest computed tomography with and without contrast medium and total body position emission tomography (see panels). The patient underwent a right atrial biopsy via the jugular venous route with transoesophageal echocardiography guidance, and the samples were sent for histological analysis. The morphological and immunohistochemical findings were consistent with a diagnosis of right atrial angiosarcoma. Given the loco-regional and distant extension of the lesion, the patient underwent chemotherapy with gemcitabine for 8 days every 21 days with evidence of notable reduction in cardiac mass.