Primary Intra-aortic Epstein-Barr Virus–Positive Large B-Cell Lymphoma Presenting as Aortic Mural Thrombosis: An Entity Distinct From Intravascular Large B-Cell Lymphoma

Abstract

Intravascular selective growth of neoplastic B lymphocytes is a characteristic finding of intravascular large B-cell lymphoma (IVLBCL). However, because neoplastic B cells of IVLBCL grow merely in the lumina of capillaries or small vessels, primary IVLBCL of the great vessels is considered exceptional. To our knowledge, only 2 primary B-cell lymphomas in the lumina of the vena cava have been reported. However, there has been no report of primary B-cell lymphoma with intra-aortic growth. We describe a novel manifestation of primary Epstein-Barr virus-positive large B-cell lymphoma mainly affecting the lumina of the aorta and its major branches in a 76-year-old man. He had a long-term fever that was refractory to antibiotics and aortic mural thrombosis with visceral embolization. Because he had no detectable mass suggesting a malignancy, it was difficult to diagnose while he was alive. He died without anticancer treatment, and the confirmed diagnosis was made at autopsy.

EBV-Positive Intravascular Large B-Cell Lymphoma of the Small Intestine: A Case Report and Literature Review

“…30 There have been 3 cases of EBV-positive IVLBCL reported in the literature. [33][34][35] One case (1 of 3) was associated with HIV infection, while the other 2 cases (2 of 3) involved elderly adults without EBV infection or acquired immunodeficiency syndrome (AIDS). Burkitt's lymphoma, classic Hodgkin's lymphoma, lymphoma-like granulomatosis, primary exudative lymphoma, plasmablastic lymphoma, and lymphoproliferative diseases after transplantation are primarily associated with EBV infection.…”

Intravascular lymphoma forming massive aortic tumors complicated with sarcoidosis and focal segmental glomerulosclerosis: a case report and literature review

“…Evaluating cases with similar presentation may help to further investigate the etiology of IVLBCL with aortic tumor formation. There have been case reports of intra-aortic B-cell lymphoma confirmed by pathological evaluation [ 19 ] or suspected from history [ 20 , 21 ]. None of them were proven to have IVLBCL, but there may be a common underlying etiology that promotes intra-aortic tumor formation.…”