Primary B-cell Lymphoma of the Paravisceral Aorta with Clinical Presentation Mimicking Mycotic Aneurysm
Lauren E. Orr, M.D., Manju Kalra, M.B.B.S., Young Erben, M.D., Melanie C. Bois, M.D., Joseph J. Maleszewski, M.D., Randall R. DeMartino, M.D..
Mayo Clinic, Rochester, MN, USA.
OBJECTIVE - Lymphoma arising primarily from the aorta is an exceedingly rare disease entity. The purpose of this report is to describe the diagnosis, management, and outcomes of two cases of diffuse large B-cell lymphoma of the aorta associated with rapidly expanding, symptomatic abdominal aortic aneurysms.
METHODS - A retrospective review of two patients undergoing surgery at a single institution for lymphoma of the aorta was performed.
RESULTS - Two Caucasian male patients, ages 74 and 75 years, presented with symptoms of abdominal/back pain of 6 months and 3 weeks duration, along with constitutional symptoms of chills and weight loss in the former patient. Computerized tomography (CT) in both revealed paravisceral abdominal aortic aneurysms that had rapidly expanded over a period of months to 4.8 cm and 4.1 cm, respectively. Leukocytes, sedimentation rate, and C-reactive protein (CRP) were normal, and blood cultures were negative. One patient had a PET scan performed, which showed mild FDG uptake in the aneurysm. A presumptive diagnosis of mycotic aneurysm was made in both cases, and they underwent open repair with debranching of the visceral and renal arteries using antegrade rifampin-soaked Dacron grafts arising from the supraceliac aorta, followed by aortic replacement. The excised aortic tissue and thrombus revealed EBV-positive diffuse large B-cell lymphoma, confirmed with immunoperoxidase studies and in situ hybridization. One patient had an uncomplicated postoperative course and underwent adjuvant R-CHOP chemotherapy. At 20 months, he had no evidence of disease on surveillance imaging. The second patient's course was complicated by colonic perforation and multiple organ dysfunction, and he died of sepsis two weeks after hospital discharge.
Fig 1. (A) Preoperative CT scan shows a paravisceral aortic aneurysm with irregular mural thrombus extending into the right renal artery. (B) Postoperative CT angiography in the same patient depicts the repair with a quadrangulated visceral/renal and straight aortic graft.
CONCLUSIONS - Although rare, aortic lymphoma should remain in the differential diagnosis for symptomatic, rapidly expanding abdominal aortic aneurysms. In suspicious cases, aortic tissue should be sent for pathologic analysis. Surgical resection and definitive chemotherapy is the current standard of treatment for this complex lymphoma.
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