Recurrent Ebstein-Barr Virus–Associated Diffuse Large B-Cell Lymphoma in an Ascending Aorta Graft

A 51-year-old man with Marfan syndrome and multiple previous surgical interventions presented with an enlarging aortic root pseudoaneurysm. His past history was significant for type A aortic dissection at age 25 years, treated with composite root replacement with a Bjork-Shiley valved conduit. Revision of the left coronary button was performed 1 year later as a result of pseudoaneurysm formation. At age 48 years, he had documented ventricular tachycardia with an echocardiogram showing inferolateral hypokinesis. His cardiac enzymes were negative, and he received an implantable cardioverter defibrillator (ICD). Although asymptomatic, he was referred to the Marfan Clinic at our institution a few months later. Transthoracic echocardiography showed mobile density in the ascending aortic graft (Figure 1), and computed tomography (CT) angiography showed a large amount of intraluminal thrombus in the ascending aorta graft, as well as coronary button aneurysms (Figure 2). Magnetic resonance imaging of the head showed infarcts in multiple vascular distributions. The patient underwent redo aortic root replacement with a 25-mm CarboMedics composite graft that required a short segment of a saphenous vein interposition graft to the small left coronary button. A large amount of intraluminal thrombus within the ascending aorta was noted at the time of surgery (Figure 3), and histological evaluation of this thrombus revealed Ebstein-Barr virus (EBV)-associated diffuse large B-cell lymphoma (DLBCL). The neoplastic lymphocytes expressed CD20, MUM1, and BCL2 and in situ hybridization studies …