Renal Angiomyolipoma: A Review of the Literature and a Report of 4 Cases

Wong, Adelaine; McGeorge, A; Clark, A. H.

doi:10.1111/j.1464-410x.1981.tb03218.x

Cited by 37 publications

(22 citation statements)

References 21 publications

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“…The contribution of each of the different cell components can vary from lesion to lesion, and some lesions can exhibit a dominant cell type [30,31] . Although very rare for the TSC patient population, epithelioid angiomyolipomas can exhibit an aggressive phenotype [32] , recur after resection and even be fatal [25,33,34] .…”

Section: Fat-poor Angiomyolipomamentioning

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: Fat-poor Angiomyolipomamentioning

confidence: 99%

Tuberous sclerosis complex renal disease

Dixon¹,

Kingswood²,

Bissler³

2015

Oxford Medicine Online

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show abstract

“…The contribution of each of the different cell components can vary from lesion to lesion, and some lesions can exhibit a dominant cell type [30,31]. Although very rare for the TSC patient population, epithelioid angiomyolipomas can exhibit an aggressive phenotype [32], recur after resection and even be fatal [25,33,34].…”

Section: Fat-poor Angiomyolipomamentioning

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.

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“…The contribution of each different cell component can vary from lesion to lesion, and some lesions can exhibit a dominant cell type [53,54]. Phenotypically, epithelioid cells can range from polygonal with a slight degree of nuclear atypia to atypical with variable size; these epithelioid cells also can exhibit significant mitotic activity (Fig.…”

Section: Fat-poor Renal Lesionsmentioning

confidence: 97%

Clinical and Molecular Insights into Tuberous Sclerosis Complex Renal Disease

2011

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Patients with tuberous sclerosis complex are at great risk of developing renal lesions as part of their disease. These lesions include renal cysts and tumors. Significant advances in understanding the cell biology of these renal lesions has already led to clinical trials demonstrating that pharmacological interventions are likely possible. This review focuses on the pathology of these renal lesions, their underlying cell biology, and the possible therapeutic strategies that may prove to significantly improve care for these patients.

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Renal Angiomyolipoma: A Review of the Literature and a Report of 4 Cases

Abstract: Summary— Four cases of renal angiomyolipoma are presented in patients with no other stigmata of tuberous sclerosis. The presentation, radiological and pathological features are reviewed.

Cited by 37 publications

References 21 publications

Tuberous sclerosis complex renal disease

Tuberous sclerosis complex renal disease

Tuberous Sclerosis Complex Renal Disease

Clinical and Molecular Insights into Tuberous Sclerosis Complex Renal Disease

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