%0 Journal Article
%T Nephron Sparing Surgery for Renal Angiomyolipoma with Inferior Vena Cava Thrombus in Tuberous Sclerosis
%A Adrien Riviere
%A Thomas Bessede
%A Jean-Jacques Patard
%J Case Reports in Urology
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/285613
%X Introduction. Angiomyolipoma is a common benign renal tumor. It is associated with Tuberous Sclerosis Complex (TSC) in 20% of patients. Angiomyolipomas are classically multiple, bilateral, and growing; they may lead to complications such as Wunderlich syndrome or, in rare cases, to venous extension. Observation. a 74-year-old woman with TSC presented with an angiomyolipoma of the right kidney with inferior vena cava (IVC) fatty thrombus. She underwent partial nephrectomy and thrombectomy. After a 7-year follow-up there was no evidence of recurrence or metastasis and her renal function was preserved. Review of Literature. It is the 44th reported angiomyolipoma associated with IVC thrombus. The mean size of angiomyolipomas was 86.1£żmm and 67.4% of patients were symptomatic. Pulmonary embolism was found in 6 patients. There were 2 cases of recurrence/metastatic outcome after radical nephrectomy and thrombectomy. They were associated with epithelioid form. The mean size of epithelioid tumors was significantly bigger than in classical angiomyolipomas (127.1£żmm versus 82.6£żmm, ). With a median follow-up of 12 months, 91.3% of patients were recurrence and metastasis free, with 3 cases of nephron sparing surgery. Conclusion. Nephron sparing surgery for angiomyolipoma with IVC fatty thrombus can be safely performed in TSC, even in sporadic angiomyolipoma. 1. Introduction Angiomyolipomas (AMLs) represent 0.3% of all renal tumors. The sex ratio is 4 men for 11 women. Most of the time, AMLs are sporadic but in 20% of patients AMLs are associated with Tuberous Sclerosis Complex. Thus they are classically multiple, bilateral, and growing [1]. They may become symptomatic and may require active management which has to be as conservative as possible. Many cases of inferior vena cava thrombus associated with AML have been reported [2¨C43]. We report a new clinical case of AML with inferior vena cava thrombus in a TSC patient that raises the question of the best surgical approach regarding the necessity of preserving renal function. 2. Materials and Methods 2.1. Observation A 74-year-old woman with tuberous sclerosis and multiple bilateral AML who had undergone partial polar superior nephrectomy for a renal cell carcinoma in 1990 came after 7 years of surveillance with the evidence of an inferior vena cava thrombus developed from the right renal vein (Figure 1). Figure 1: Sonography of the IVC thrombus originated from the right renal vein. Computerized tomography showed multiple renal tumors with spontaneous density inferior toĦŞ20 Hounsfield Units (HU) without contrast
%U http://www.hindawi.com/journals/criu/2014/285613/