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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front. Med.    2009, Vol. 3 Issue (4) : 495-498
Research articles
Giant renal angiomyolipoma with tuberous sclerosis complex
Ouyan SHI PhD1,Guodong XU BM2,Chunxiang WANG BM2,
1.Faculty of Basic Medicine, Tianjin Medical University, Tianjin 300070, China; 2.Department of Pediatric Surgery, Tianjin Children’s Hospital, Tianjin 300070, China;
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Abstract Though rare, angiomyolipomas (AMLs) are the most common mesenchymal tumors of kidney. In general, AMLs can always be associated with two conditions affecting other organ systems: tuberous sclerosis complex (TSC) and sporadic lymphangioleiomyomatosis. This article presents a case of renal AML occurring in a 14-year-old girl with a definite diagnosis of TSC. She had been diagnosed with TSC at the age of three, and a schedule for close observation was disobeyed. At this time, she underwent a series of examinations: physical examination, ultrasonography, angiography, computed tomography (CT), and magnetic resonance imaging (MRI) scans. The physical examination showed adenoma sebaceum in a butterfly paranasal distribution, and a mass was palpated in the left upper quadrant. There were no neurological deficits. Imaging studies (including ultrasonography, angiography, CT, and MRI) of the abdomen showed a large heterogeneous mass arising from the left kidney. Partial nephrectomy was performed. The pathological diagnosis was hemorrhagic renal AML. No recurrence was found in the three-year follow-up. We concluded that schedule of close observation on patients with TSC should be strictly abided by for the high morbidity of AMLs. The specific risks of renal AMLs are spontaneous hemorrhage and rupture. Treatment options for AMLs include conservative and interventional (total/partial nephrectomy, cryoptherapy, and embolization) treatments.
Keywords renal angiomyolipoma      tuberous sclerosis complex      hemorrhage      
Issue Date: 05 December 2009
 Cite this article:   
Ouyan SHI PhD,Chunxiang WANG BM,Guodong XU BM. Giant renal angiomyolipoma with tuberous sclerosis complex[J]. Front. Med., 2009, 3(4): 495-498.
Curatolo P. Tuberoussclerosis complex: from basic science to clinical phenotypes. London: MackKeith Press, 2003
Napolioni V, Curatolo P. Genetics and molecular biologyof tuberous sclerosis complex. Curr Genomics, 2008, 9(7): 475―487

doi: 10.2174/138920208786241243
Nelson C P, Sanda M G. Contemporary diagnosis andmanagement of renal angiomyolipoma. J Urol, 2002, 168 (4 Pt 1): 1315―1325
Simmons J L, Hussain S A, Riley P, Wallace D M A. Management of renal angiomyolipoma in patients with tuberous sclerosiscomplex. Oncol Rep, 2003, 10(1): 237―241
Lesma E, Sirchia S M, Ancona S, Carelli S, Bosari S, Ghelma F, Montanari E, Di Giulio A M, Gorio A. The methylation of the TSC2 promoterunderlies the abnormal growth of TSC2 angiomyolipoma-derivedsmooth muscle cells. Am J Pathol, 2009, 174(6): 2150―2159

doi: 10.2353/ajpath.2009.080799
Astrinidis A, Henske E P. Tuberous sclerosis complex:linking growth and energy signaling pathways with human disease. Oncogene, 2005, 24(50): 7475―7481

doi: 10.1038/sj.onc.1209090
Curatolo P, Bombardieri R. Tuberous sclerosis. Handb Clin Neurol, 2007, 87: 129―151

doi: 10.1016/S0072-9752(07)87009-6
Steiner M S, Goldman S M, Fishman E K, Marshall F F. The natural history of angiomyolipoma. J Urol, 1993, 150(6): 1782―1786
Arenson A M, Graham R T, Shaw P, Srigley J, Herschorn S. Angiomyolipoma of the kidneyextending into the inferior vena cava: sonographic and CT findings. AJR Am J Roentgenol, 1988, 151(6): 1159―1161
Hélénon O, Merran S, Paraf F, Melki P, Correas J M, Chrétien Y, Moreau J F. Unusual fat-containing tumors of the kidney: a diagnostic dilemma. Radiographics, 1997, 17(1): 129―144
Lemaitre L, Claudon M, Dubrulle F. Imaging of angiomyolipomas. Semin Ultrasound CT, 1997, 18: 100―114

doi: 10.1016/S0887-2171(97)90054-8
Singh D, Kashyap S, Kaur S. Renal angiomyolipoma, a diagnostic dilemma: a case report. Indian J Pathol Microbiol, 2007, 50(3): 622―623
Handa U, Nanda A, Mohan H. Fine-needle aspiration of renal angiomyolipoma: a reportof four cases. Cytopathology, 2007, 18(4): 250―254

doi: 10.1111/j.1365-2303.2006.00356.x
Danforth T L, Lane B R, Novick A C. Conservative management of giant symptomatic angiomyolipomasin patients with the tuberous sclerosis complex. BJU Int, 2007, 100(4): 794―797

doi: 10.1111/j.1464-410X.2007.07059.x
Rakowski S K, Winterkorn E B, Paul E, Steele D J, Halpern E F, Thiele E A. Renal manifestations of tuberous sclerosis complex: Incidence,prognosis, and predictive factors. KidneyInt, 2006, 70(10): 1777―1782

doi: 10.1038/
Williams J M, Racadio J M, Johnson N D, Donnelly L F, Bissler J J. Embolization of renal angiomyolipomatain patients with tuberous sclerosis complex. Am J Kidney Dis, 2006, 47(1): 95―102

doi: 10.1053/j.ajkd.2005.09.028
Lee S Y, Hsu H H, Chen Y C, Huang C C, Wong Y C, Wang L J, Chuang C K, Yang C W. Evaluation of renal function of angiomyolipoma patientsafter selective transcatheter arterial embolization. Am J Med Sci, 2009, 337(2): 103―108

doi: 10.1097/MAJ.0b013e31817f6dd9
Zorn K C, Starks C L, Gofrit O N, Orvieto M A, Shalhav A L. Embolization of renal-arterypseudoaneurysm after laparoscopic partial nephrectomy for angiomyolipoma:case report and literature review. J Endourol, 2007, 21(7): 763―768

doi: 10.1089/end.2006.0332
Somani B K, Nabi G, Thorpe P, Hussey J, McClinton S. Therapeutic transarterialembolisation in the management of benign and malignant renal conditions. Surgeon, 2006, 4(6): 348―352
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