Primary pericardial angiosarcoma is extremely rare tumour with a poor prognosis which is tend to metastasis.The patients usually refer to the cardiology department with the complaints of dyspnea ,angina, exercise intolerance,tachycardia,hemoptysis and cough. These symptoms commonly occur during the further terms of the disease.The symptoms may imitate coronary artery disease. Here, we present an angiosarcoma that narrows the mid segment of the right coronary artery outside-in.
Case Report
39 year-old-female referred to our cardiology department with the complaints of exertional dyspnea and fatigue for two weeks. The patient had not suffered from any disease except for the pericardiosynthesis which was performed a year ago due to the pericardial effusion of uncertain cause. On physical examination, her arterial blood pressure was 100/60 mmHg, juguler venosus distension, hepatomegaly which is 8 cm below the costal margin and pretibial edema was observed. Chest X-Ray revealed an increased cardiothoracic ratio.The 12-lead electrocardiogram showed sinus tachycardia and negative T waves in the inferior and lateral leads. Left ventricle function was evaluated as normal in echocardiogram. Left ventricle was concentric hypertrophic and the pericardium was rather thick.In addition, hyperechogenic tissue density is seen in right ventricle wall neighbourhood and lateral wall neighbourhood. Coronary angiography showed normal coronary vessel except an outside-in narrowing in the right coronary artery from the proximal area to the mid area. CT-Angiography showed a medium density tissue formation of which the diameter may approach to 4.5-5 cm in the pericardial area. It was observed that the mentioned formation infiltrated and narrowing the proximal and medium segments of right coronary artery. Surgery was suggested,because of narrowing of right coronary artery and constrictive pericarditis. Pericardium was thickened and a diffuse tumour which was extensively adherent was present. Pericardium was mildly excised and released. Unfortunately the tumor was unresectable and just incisional biopsy was performed. The result of the pathological examination was positive for CD31, CD34 and factor 8 that supports the diagnosis of angiosarcoma.
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