Renal angiomyolipoma (RAML), a rare benign mesenchymal tumor, consists of variable proportions of thick-walled blood vessels admixed with adipose tissue and bundles of smooth muscle. Rarely, RAML angiomyolipoma may be associated with pseudoaneurysm, spontaneous rupture and hemorrhage. we herein present an exceptional case of fat-poor RAML containing a large pseudoaneurysm in a 48-yearold woman with known pulmonary lymphangioleiomyomatosis (PLAM), who was admitted to hospital due to right flank pain for about two months. To avoid risk of rupture of the pseudoaneurysms, transcatheter arterial embolization (TAE) procedure was performed, at the same time taking into account of the possibility of malignancy in such a large neoplasm, right radical nephrectomy was taken five days later. This case was benign and the 1-year follow-up ultrasonic examination showed no recurrence. This is the first report that the patient had a sporadic giant RAML with pseudoaneurysm which was not associated with the TSC, and to our knowledge, such cases have not been explored in literature. This case reminds us that giant sporadic RAML with pseudoaneurysm, although rare, does exist, and we should improve the understanding of the neoplasm. Early detection and therapeutic interventions of pseudoaneurysm in an AML patient can reduce serious complications.
Keywords: Renal angiomyolipoma (RAML); case report; pseudoaneurysm; pulmonary lymphangioleiomyomatosis (PLAM); ultrasonography.