Long-term Clinical Morbidity in Patients With Renal Angiomyolipoma Associated With Tuberous Sclerosis Complex

Bissler, John J.; Cappell, Katherine; Charles, H.; Song, Xue; Liu, Zhimei; Prestifilippo, Judith; Gregory, Christopher; Hulbert, John C.

doi:10.1016/j.urology.2016.04.027

Cited by 31 publications

(19 citation statements)

References 28 publications

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“…Treatment options for AMLs are often complicated by the presence of bilateral and multiple lesions, which particularly affect the TSC subgroup. 20 In the present study, 71% of patients had multiple lesions, rendering traditional surgical techniques for preserving renal function, such as partial or total nephrectomy, a less favourable option. 6 TSC is an autosomal dominant disease affecting approximately 2 million people globally.…”

Section: Discussionmentioning

confidence: 54%

Transarterial embolization for renal angiomyolipomas: A single centre experience in 79 patients

et al. 2017

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ObjectiveTo evaluate the long-term efficacy and safety of selective arterial embolization (SAE) in the treatment of renal angiomyolipomas (AMLs).MethodsThis was a retrospective review of medical records and imaging findings from patients with renal AMLs who attended our clinic and received SAE between January 2007 and January 2014. Only patents with complete medical records, preoperative computed tomography scans using typical imaging and follow-up data were included.ResultsA total of 79 patients were enrolled in the study. Technical and clinical success rates were 100% and 91% (n = 72), respectively. Only two patients experienced major complications. Post-embolization syndrome (i.e. fever, abdominal pain, nausea or vomiting) was reported in 68 (86%) patients, but all symptoms were mild and resolved with conservative measures. Mean radiological and clinical follow-up periods were 16.8 and 35.9 months, respectively. In 75 (95%) patients, tumours decreased in size; mean ± SD tumour size significantly decreased from 8.4 ± 3.5 cm pre-embolization to 6.7 ± 3.0 cm post-embolization.ConclusionsThis study provides long-term evidence that SAE is a safe and effective method in the treatment of patients with renal AMLs.

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Section: Discussionmentioning

confidence: 54%

Transarterial embolization for renal angiomyolipomas: A single centre experience in 79 patients

et al. 2017

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“…The most common renal manifestations of TSC are angiomyolipomas, which are found in up to 80% of TSC patients [ 4 ]; the presence of two or more renal angiomyolipomas is a major diagnostic criterion for a TSC diagnosis [ 5 ]. Another frequent kidney lesion in TSC is renal cysts, which are found in 14–32% of patients [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Diagnosis and follow-up of TSC-associated renal angiomyolipomas are based mainly on imaging studies. Although renal angiomyolipomas are still the main cause of morbidity and mortality in adults with TSC [ 4 ], mTOR inhibitors can reduce the size of angiomyolipomas and have become first-line therapy for tumours with diameters >3 cm [ 5 ]. There are no controlled trials comparing therapy with mTOR inhibitors and embolization to prevent bleeding and recurrence and preserve renal function [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

Recommendations for imaging-based diagnosis and management of renal angiomyolipoma associated with tuberous sclerosis complex

Pradilla

Ballesté

Torra

et al. 2017

Clinical Kidney Journal

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Renal angiomyolipomas are found in up to 80% of tuberous sclerosis complex (TSC) patients. Although these tumours are usually asymptomatic, lesions >3 cm in diameter are prone to bleeding and up to 10% of TSC patients may experience a massive and potentially fatal retroperitoneal haemorrhage. Diagnosis can be complicated because of the initial lack of symptoms and the fat-poor content of atypical renal angiomyolipomas. After diagnosis, tumour growth and the emergence of new tumours must be monitored. Treatment with mammalian target of rapamycin (mTOR) inhibitors can reduce tumour size and is indicated in patients with TSC-associated renal angiomyolipomas >3 cm in diameter. Imaging-based assessment of kidney disease is essential to the diagnosis and management of patients with TSC. The aims of imaging studies in this context are to detect and characterize tumours, assess and detect the risk of complications and evaluate the response to treatment, especially in patients treated with mTOR inhibitors. A multidisciplinary expert panel developed a series of recommendations based on current evidence and professional experience for imaging studies in adults and children with TSC-associated renal angiomyolipoma. The recommendations cover radiological diagnosis and follow-up of the classic and atypical or fat-poor TSC-associated renal angiomyolipomas, biopsy indications, minimal requirements for radiological requests and reports and recommended technical features and protocols for computed tomography and magnetic resonance imaging.

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“…Unlike sporadic AML, TSC-associated AML develops at multiple sites on the bilateral sides. 7 It develops at a younger age, and tends to exhibit a much faster growth rate over time than sporadic AML 22,23 . When urolithiasis is comorbid with TSC-associated AML, ESWL and PNL cannot be performed because of the risk of bleeding from the AML.…”

Section: Discussionmentioning

confidence: 99%

Management of urolithiasis associated with tuberous sclerosis complex

Hatano

Imai

Mori³

et al. 2019

Preprint

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The most common renal symptoms of tuberous sclerosis complex (TSC) are angiomyolipomas (AMLs) and renal cysts; however, some patients with TSC also develop urolithiasis. In this study, we evaluated the incidence, risk factors and clinical courses of TSC-associated urolithiasis. We analyzed a total of 102 patients who met the diagnostic criteria for TSC, of whom 15 (14.7%) had urolithiasis. We compared urinary specific gravity, urine pH, serum calcium, intact parathyroid hormone and the use of antiepileptic agents of the urolithiasis and non-urolithiasis groups. The urine specific gravity and urine pH were higher in the urolithiasis group than in the non-urolithiasis group (P = 0.005, P = 0.042, respectively). No significant difference was found between the two groups with regard to the serum corrected calcium and intact parathyroid hormone levels. The antiepileptic drugs topiramate and zonisamide were more frequently administered to the urolithiasis group than the non-urolithiasis group (P <0.001, P = 0.039, respectively). Four patients in the urolithiasis group underwent surgery. Three patients could not receive extracorporeal shock wave lithotripsy due to the risk of bleeding from the AML, and therefore underwent transurethral lithotripsy. If urolithiasis is comorbid with TSC-associated AML, the treatment options are more limited in cases with multiple AMLs around the stone due to an increased risk of hemorrhage. Prevention and early detection of urolithiasis are beneficial to patients with TSC.

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Long-term Clinical Morbidity in Patients With Renal Angiomyolipoma Associated With Tuberous Sclerosis Complex

Abstract: TSC-renal AML patients are at significantly higher risk for renal morbidity relative to the general population.

Cited by 31 publications

References 28 publications

Transarterial embolization for renal angiomyolipomas: A single centre experience in 79 patients

Transarterial embolization for renal angiomyolipomas: A single centre experience in 79 patients

Recommendations for imaging-based diagnosis and management of renal angiomyolipoma associated with tuberous sclerosis complex

Management of urolithiasis associated with tuberous sclerosis complex

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