Introduction: Renal angiomyolipoma (AML) is a mesenchymal tumor benign composed by vascular, smooth muscle and adipose tissue. There are two types of AMLs: The classic variant is the most common and is characterized by thick walled vessels, spindle smooth-muscle-like cells and adipose tissue.
The epithelioid variant has an epithelial cell component to it with minimal adipose tissue and has the potential to undergo malignant transformation.
The objective of this video is presented the multidisciplinary approach from surgical technique to medical intensive care during and after the surgery.
Methods: A 44-year-old woman was referred by his primary care physician with symptoms characterized by shortness of breath, tachycardia, intermittent hematuria and right lumbar pain. Physical examination with holosystolic murmur.
Computerized tomography (CT) scan demonstrated a 6.1x5.1cm right renal tumor, the attenuation was - 83 Hounsfield Units (HU) with tumor thrombus invasion into the inferior vena cava, which extended from renal vein ostium to right atrium. Magnetic resonance imaging (MRI) showed non-infiltration of the vena cava wall. Echocardiogram presented systolic protrusion of the thrombus towards the right ventricle, developing moderate tricuspid regurgitation.
Results: With multidisciplinary management (Urology, cardiology, cardiothoracic surgery, perfusionists and intensive care therapy) were performed an open right radical nephrectomy, sternotomy, cavotomy with extraction of intra-atrial thrombus and primary cavorrhaphy. With support of cellular recuperator and extracorporeal circulation pump (pump time 100 min) trans-surgical transfusions were: 3 erythrocyte concentrates, 2 fresh frozen plasmas and 1 platelet apheresis. Surgical time was 380 min. During the surgery a real-time transesophageal echocardiogram was used. Bleeding was 2500 ml and no intraoperative complications were reported.
Hospital stay of 20 days, discharged without complications with serum creatinine 1.0 mg / dl and hemoglobin 11.5 g / dl.
Pathology report was renal classic AML, size 8.0x6.0x4.0cm, immunohistochemistry: HMB45 (+) and smooth muscle actin (+).
Conclusions: This is a rare case of a classic AML presenting with a venous tumor thrombus involving the inferior vena cava from renal vein ostium to right atrium. Radical nephrectomy with tumor thrombectomy is the most widely accepted surgical treatment following established vascular principles of venous tumor thrombectomy.
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