Intramural Great Vessel Lymphoma in a Patient with Left Ventricular Assist Device

Abstract

<h3>Introduction</h3> Intramural Great Vessel lymphoma (IMGVL) is an extremely rare lymphoma proliferating within the lumina of large vessels like Superior vena cava and Aorta. We present a case of an IMGVL presenting in a patient with a Left Ventricular Assist Device (LVAD). <h3>Case Report</h3> A 63-year-old male with ischemic cardiomyopathy and advanced systolic failure supported by an LVAD as a bridge to transplant, presented to the clinic complaining of claudication symptoms. He has known peripheral vascular disease and underwent Aortobifemoral stenting in the past. Coronary Tomography with Angiography (CTA) showed a large intraluminal thrombus above the iliac limbs with severe narrowing of the lumen thought to be flow-limiting (Figure 1). He underwent a mechanical thrombectomy. Intra-operatively, a large mural thrombus with near occlusion of the descending aorta was noted (Figure 2). Pathology revealed diffuse large B-cell lymphoma (DLBCL) with immunohistochemistry positive for BCL2, MYC, Ki-67 and CD20. EBV status was negative. A whole-body Positron Emission Tomography (PET) scan was negative for malignancy elsewhere. He was initiated on chemotherapy. <h3>Summary</h3> Primary IMGVL is a rare subtype of extranodal lymphoma with only 4 cases reported in literature. It is distinct from Intravascular Large B-cell Lymphoma (IVLBCL) which is limited to small vessels. Furthermore, only a single case of aortic IMGVL found on autopsy of an EBV positive patient, has been reported. DLBCL is known to be an aggressive subtype of lymphoma often requiring a combination of chemotherapy and immune therapy. Long term prognosis in our patient awaiting heart transplant is rather uncertain.