Management of Renal Angiomyolipoma:Analysis of 15 Cases

Kessler, Oded; Gillon, Gabriel; Neuman, Menahem; Engelstein, Dov; Winkler, Harry; Baniel, J.

doi:10.1159/000019658

Cited by 62 publications

(27 citation statements)

References 19 publications

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“…1 c) that can rupture causing the hemorrhage [14][15][16][17] . The hemorrhage risk of renal angiomyolipomas in TSC patients is between 25 and 50% [18,19] , and between 20-30% of pa- tients with hemorrhages present to the emergency room in shock [20] . The hemorrhage risk is significantly increased for aneurysms larger than 5 mm [21] .…”

Section: Renal Angiomyolipomas In Tscmentioning

confidence: 99%

Tuberous sclerosis complex renal disease

Dixon¹,

Kingswood²,

Bissler³

2015

Oxford Medicine Online

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and intractable epilepsy), less than 40% of affected patients have all three features [3] . TSC most often presents with neurologic symptoms, and approximately 90% of affected individuals experience seizures and about half of patients experience cognitive impairment, autism, or other behavioral disorders. Renal manifestations are the second most common findings associated with TSC, with angiomyolipomas occurring in 80% and renal cystic disease in ϳ 50% of patients. Pulmonary involvement, specifically lymphangioleiomyomatosis (LAM), is the third most common cause of TSC-associated morbidity, occurring in approximately 35% of female TSC patients. The gender predilection in LAM is not understood.TSC is caused by mutations in either the TSC1 gene, located on chromosome 9, or the TSC2 gene, located on chromosome 16. Typically children with TSC are born with normal kidneys but develop cystic disease and angiomyolipomas as they age. Both renal cystic disease and angiomyolipomas cause chronic kidney disease (CKD), affecting approximately one million patients with TSC worldwide. Renal disease poses a significant burden on patients with TSC because of the relentless progression, morbidity, and mortality of CKD. In fact, using death certificate data Shepherd et al. [4] identified renal failure as the leading cause of death in their adult patients at the Mayo Clinic. Key WordsTuberous sclerosis complex ؒ Angiomyolipoma ؒ Renal cyst ؒ Oncocytoma ؒ Mammalian target of rapamycin complex 1 ؒ Rapamycin ؒ Sirolimus AbstractAlthough not as common as other genetic renal diseases such as autosomal dominant polycystic kidney disease, patients with tuberous sclerosis complex frequently have significant renal involvement. Recent revelations in the cell biology of these renal disease manifestations as well as effective therapies for tuberous sclerosis complex-related renal issues have heralded hope of improved renal survival and improved quality of life for the TSC patient. This review specifically addresses some of the major renal manifestations of this disease.

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Section: Renal Angiomyolipomas In Tscmentioning

confidence: 99%

Tuberous sclerosis complex renal disease

Dixon¹,

Kingswood²,

Bissler³

2015

Oxford Medicine Online

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“…The abnormal vasculature in angiomyolipomas is frequently associated with the development of micro-and macro-aneurysms, putting both children and adults with TSC at risk of spontaneous, possibly lifethreatening, hemorrhage [19][20][21][22][23]. This risk is estimated to be 25-50 % [16,24,25], with up to 20 % of patients presenting with hypovolemic shock [25]. Risk factors for bleeding from angiomyolipomas are tumor size (>4 cm), growth as assessed by serial measurements, and aneurysm size (>0.5 cm) [8,26,27].…”

Section: Angiomyolipomamentioning

confidence: 99%

Tuberous sclerosis complex: the past and the future

2014

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Renal lesions represent the second most significant cause of morbidity and mortality in patients with tuberous sclerosis complex (TSC). Recent advances in the understanding of the pathophysiology of TSC have led to the exploration of new potential therapeutic targets. Clinical trials with mammalian target of rapamycin (mTOR) inhibitors have demonstrated promising results for several indications, such as renal angiomyolipoma, subependymal giant cell astrocytoma, lymphangioleiomyomatosis and facial angiofibromas. Currently, there is a scarcity of natural history data and randomized, placebo-controlled clinical trials on TSC. Recently, however, recommendations for the diagnostic criteria, surveillance, and management of TSC patients have been updated. This review focuses on these novel recommendations and highlights the need for multidisciplinary follow-up of this multi-systemic disease.

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“…1c) that can rupture causing the hemorrhage [14,15,16,17]. The hemorrhage risk of renal angiomyolipomas in TSC patients is between 25 and 50% [18,19], and between 20–30% of patients with hemorrhages present to the emergency room in shock [20]. The hemorrhage risk is significantly increased for aneurysms larger than 5 mm [21].…”

Section: Renal Angiomyolipomas In Tscmentioning

confidence: 99%

Tuberous Sclerosis Complex Renal Disease

Dixon

Hulbert

Bissler

2010

Nephron Exp Nephrol

140

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Although not as common as other genetic renal diseases such as autosomal dominant polycystic kidney disease, patients with tuberous sclerosis complex frequently have significant renal involvement. Recent revelations in the cell biology of these renal disease manifestations as well as effective therapies for tuberous sclerosis complex-related renal issues have heralded hope of improved renal survival and improved quality of life for the TSC patient. This review specifically addresses some of the major renal manifestations of this disease.

show abstract

Management of Renal Angiomyolipoma:Analysis of 15 Cases

Cited by 62 publications

References 19 publications

Tuberous sclerosis complex renal disease

Tuberous sclerosis complex renal disease

Tuberous sclerosis complex: the past and the future

Tuberous Sclerosis Complex Renal Disease

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