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Int J Clin Exp Pathol 2013;6(6):1180-1186
A case of renal angiomyolipoma with intracardiac extension and asymptomatic
Xiaoman Li, Qingchang Li, Yuan Miao, Hongtao Xu, Yang Liu, Xueshan Qiu, En-Hua Wang
Key Laboratory of Medical Cell Biology, Ministry of Education, China Medical University, Shenyang, 110001, China; Department of Pathology, the
First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China
Received March 24, 2013; Accepted March 29, 2013; Epub May 15, 2013; Published June 1, 2013
Abstract: Angiomyolipoma (AML) is the most common benign tumor of the kidney, which is composed of a mixture of three tissue components:
blood vessels, smooth muscle and adipose cells. Occasionally, AML may extend into the renal vein or the vena cava, but so far at least,
intracardiac extension was rarely reported. We herein present one case of renal AML with intracardiac extension and pulmonary embolism
simultaneously in a 52-year-old Chinese female patient. Contrast-enhanced computed tomography revealed a well-demarcated
heterogeneous mass in the right kidney which extended into the right atrium through the right renal vein and inferior vena cava and resulted in
embolization in the right pulmonary artery. The renal mass together with the thrombus was resected. The renal mass and thrombus in vena
cava and right atrium shared the similar histological features: mature adipose tissue, smooth muscle and thick-walled vessels. The thrombus
in the right pulmonary artery was mainly composed of mature adipose tissue. These histological features and the result of positive
immunostaining for HMB-45, Melan-A, and smooth muscle actin supported the diagnosis of AML. The component of epithelioid cells was less
than 5% and mitosis was rarely seen. Intracardiac extension is often observed in the malignant tumor and only seldom seen in benign tumors.
Our case reminds the rare possibility of intracardiac extension in renal AML, which may potentially result in fatal complications if not
appropriately managed. (IJCEP1303051).
Keywords: Angiomyolipoma, perivascular epithelioid cell, intracardiac extension, pulmonary embolism, kidney
Address correspondence to: Dr. En-Hua Wang, Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences,
China Medical University, Shenyang, 110001, China. Tel: +86 24 23261638; Fax: +86 24 23261638; E-mail: firstname.lastname@example.org