title: Tuberous sclerosis complex-associated renal angiomyolipomas: A single center study of 17 consecutive cases.
abstract: The aim of the present study was to investigate the treatment options for patients with tuberous sclerosis complex (TSC)-associated renal angiomyolipomas (AMLs). A total of 17 patients who were consecutively diagnosed with TSC-associated renal AMLs at the Department of Urology of Zhongshan Hospital between 1998 and 2012 were included in the study. The patient cohort included 7 males and 10 females with a mean age of 37.6 years (range, 18-62 years). A total of 12 patients were diagnosed with renal AML with TSC during physical examination (PE), while 5 patients were admitted to the Emergency Department of Zhongshan Hospital due to spontaneous rupture of renal AMLs. All renal lesions were examined by ultrasonography and abdominal computed tomography prior to treatment. The primary outcome measure was the kidney reservation rate (patients that had not received nephrectomies) in the rupture group and PE group. Both abdominal ultrasonography and CT revealed AMLs in all patients and the mean tumor size was 10.0±4.0 cm (range, 3.0-17.5 cm). Overall, 9 patients underwent surgery, which included unilateral nephrectomy in 4 patients and unilateral partial nephrectomy/tumor enucleation in 5 patients. The remaining 8 patients received medical treatment. All patients were followed-up for between 10 and 67 months. One patient succumbed as a result of multiple organ failure, which was caused by hypovolemic shock due to the spontaneous rupture of renal AML. The kidney reservation rate during surgery was 87.5% (7/8) in the PE group and 25% (1/4) in the spontaneous rupture group. The management of TSC-associated renal AMLs differs from that of solitary sporadic AMLs. Surgical therapy is recommended following careful risk-benefit analysis.
authors.affiliation: Department of Urology, Zhongshan Hospital, Fudan University, Shanghai 200032, P.R. China.
authors.forename: Hang
authors.initials: H
authors.lastname: Wang
authors.affiliation: Department of Urology, Zhongshan Hospital, Fudan University, Shanghai 200032, P.R. China.
authors.forename: Qilai
authors.initials: Q
authors.lastname: Long
authors.affiliation: Department of Urology, Zhongshan Hospital, Fudan University, Shanghai 200032, P.R. China.
authors.forename: Yiwei
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Urology, Zhongshan Hospital, Fudan University, Shanghai 200032, P.R. China.
authors.forename: Li
authors.initials: L
authors.lastname: Liu
authors.affiliation: Department of Urology, Zhongshan Hospital, Fudan University, Shanghai 200032, P.R. China.
authors.forename: Lin
authors.initials: L
authors.lastname: Zhou
authors.affiliation: Department of Urology, Zhongshan Hospital, Fudan University, Shanghai 200032, P.R. China.
authors.forename: Jianming
authors.initials: J
authors.lastname: Guo
keywords: PN; TSC; mTORi; renal AMLs; surgical therapy