A rare case of superior mesenteric artery aneurysm caused by segmental arterial mediolysis with concomitant left renal angiomyolipoma and pulmonary lymphangioleiomyomatosis

Abstract

We report here in an extraordinary case of superior mesenteric artery (SMA) aneurysm caused by segmental arterial mediolysis (SAM) with concomitant left renal angiomyolipoma (AML) and pulmonary lymphangioleiomyomatosis (LAM). A 32-year-old woman presented at the urological outpatient clinic of our hospital with erythrocyturia. Abdominal computed tomography (CT) and abdominal angiography examination revealed a saccular aneurysm arising from the base of the SMA and a tiny aneurysm from the posterior inferior pancreaticoduodenal artery. A 14 cm left renal AML was also observed. Chest CT showed multiple cysts suggesting LAM. We surgically removed the aneurysm from the bifurcation region of the SMA and concurrently performed left nephrectomy including the AML. Pathological findings from the resected aneurysm showed SAM and the huge left renal tumor was histologically diagnosed as AML.