A 40-year-old male is presented with dyspnea and cough 2 months before workup in another center. He came to our center in Babol University of Medical Sciences in April 2019, 6 months after his initial workup in the primary center. In the initial evaluation, the patient was a middle-aged man without a history of any disease or family history of a disease. He was a smoker and did not drink alcohol. On first physical examination, he had crackles in the base of the left lung with other findings being unremarkable. In para-clinical evaluation, on chest x-ray (figure 1A) and chest computed tomography (CT) scan, there was evidence of bronchiectasis and left lung lower lobe collapse. Coming from the Middle East, with all the symptoms described, the first differential diagnosis for the patient was tuberculosis (TB). Sputum evaluation for mycobacterium tuberculosis was negative and bronchoscopy and bronchoalveolar lavage were done for the patient and the pathology revealed positive Ziehl-Neelsen staining for TB but negative QuantiFERON. Considering the clinical setting and high probability of tuberculosis, treatment with anti-TB medications was started for the patient.
After 2 months of treatment, although his symptoms had improved a bit, he reported back to the hospital with increased dyspnea and weakness and easy fatigability. On physical examination, he had muffled heart sounds and so echocardiography was done for the patient, which revealed mild pericardial effusion (PE) and normal left ventricle (LV) and right ventricle (RV) size and function. Anti-TB medications were continued and anti-inflammatory drugs added. His symptoms did not improve over the following 2 months and he had to be admitted again with worsening dyspnea. On evaluation, he had mild to moderate PE and mild RV enlargement. There was no evidence for constrictive pericarditis on echocardiography. D-dimer was requested for the patient which was reported to be high. Pulmonary computed tomography angiography was done for the patient which showed no evidence of pulmonary embolism (figure 1B). Previous medications were continued for the patient and the patient was discharged. After another 2 months of treatment and near the end of his TB treatment, he was referred to our center due to respiratory distress and dyspnea. On physical examination, there was a diastolic murmur at the left lower sternal border and crackles at the base of the left lung. Other than his respiratory rate which was 24/min, his other vital signs were within normal limits. Trans-thoracic echocardiography was done for the patient which showed mild RV enlargement and dysfunction, dilated inferior vena cava without collapse and mild pulmonary artery hypertension. There was thick pericardium with small PE and compressive effect on tricuspid valve with probable extracardiac source which had resulted in a significant diastolic pressure gradient. A large sized mobile mass was also seen in RV outflow tract.
Transesophageal echocardiography was done for the patient which showed a very large size multilobulated nonhomogenous mass in anterolateral side of right atrium (RA) attached to the pericardium. One lobe of it was perforating the RA wall and protruding to RV outflow tract from tricuspid valve (figure 1C and supplementary video file 1). The patient had severe symptoms and so surgical consult was done for the patient to remove the mass (figure 2A). During surgery, after sternotomy, opening the pericardium was difficult because of the adhesions present. There was a large inflammatory mass without clear borders attached to the heart with especial attachment to the RA. It was removed to maximum extent and due to increasing blood oozing, the surgery was interrupted, mediastinal space temporarily packed and the patient was transferred to intensive care unit. After correcting the underlying coagulopathy, and repeating TEE, the patient was transferred to operating room and he was put on cardiopulmonary bypass. RA was surgically opened and there was fragile mass without capsule attached to the RA wall. The mass was excised to the maximum extent and after closing the chest wall, he was transferred to the ICU. On pathological evaluation, there was neoplastic tissue composed of fasciculated spindle cells being highly CD31 positive (figure 2B) and CD 34 positive with high Ki67 proliferation index (figure 2C). The final pathology report was in favor of high-grade angiosarcoma with massive necrosis. Abdominal and lung CT scan and whole-body bone scan was done for the patient which showed no evidence of metastasis.
After the surgery, transesophageal echocardiography showed some residual mass in the RA, for which chemotherapy with docetaxel and gemcitabine were started for the patient. After six cycles of chemotherapy, the cardiac mass in the RA regressed and the cardiac function improved. To see if there is extracardiac tumor present or not, cardiac magnetic resonance imaging (CMR) was done for the patient. CMR showed a non-homogenous mass around RA and RA appendage with a size of 64x14mm (figure 3 and supplementary video file 2). There was mild pericardial thickening without hemodynamic evidence for constrictive pericarditis. Due to the residual mass, the multidisciplinary team meeting decided the patient undergo radiotherapy and close monitoring. After 25 sessions of radiotherapy, the patient is in good condition with no sign or symptoms. He has been symptom-free more than 6 months now.