A rare case of primitive right atrium angio-sarcoma with favorable outcome, in a young female. Case report and literature review.

Abstract

Primary malignant cardiac tumors are extremely rare, but their associated mortality is very high. The clinical presentation is oñen variable and nonspecific; by the time symptoms appear, the tumor has usually evolved to a large intracardiac and paracardiac mass causing considerable hemodynamic involvement, regional invasión and distant dissemination. We report a 26 year-old female with a right atrium angiosarcoma with a surprisingly favorable outcome at 2 years follow-up after treatment with a combination of neoadjuvant chemotherapy and surgical resection. She presented with chest tightness, shortness of breath at effort, atypical chest pain, palpitations, asthenia, weight loss and profuse perspiration. Physical examination and irnaging diagnostic procedures, identified a pericardial effusion and a pathologic cardiac mass. The diagnosis of a malignant angiosarcoma was suggested afier computed tomography transthoracic and transesophageal echocardiography It was confirmed at surgery by a biopsy followed by histopathology and immunohistochemistry.