Renal Angiomyolipoma: Relationships between Tumor Size, Aneurysm Formation, and Rupture

Yamakado, Koichiro; Tanaka, Naoshi; Nakagawa, Toshio; Kobayashi, Shigeki; Yanagawa, Makoto; Takeda, Kan

doi:10.1148/radiol.2251011477

Cited by 394 publications

(280 citation statements)

References 18 publications

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“…The characteristic feature of renal AML is thick-walled blood vessels, which do not have normal elastin and are prone to rupture (11). Aneurysm size (close to 5 mm) and tumor size (close to 4 cm) significantly account for rupture and hemorrhage (12). Likewise, another study revealed that tumors with serial changes in volumes were associated with higher probability of hemorrhagic lesions (13).…”

Section: Methodsmentioning

confidence: 99%

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

et al. 2017

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Recent prophylactic treatment options and diagnostic tools show a suboptimal improvement in patients with renal angiomyolipoma (AML). This study was an attempt to review management strategies applied before invasive options. An extensive research on medical databases such as PubMed and Scopus was performed from 1999 to 2016 using the following keywords: 'kidney', 'renal', and 'angiomyolipoma'. All related studies on patients treating with conservative or minimally-invasive procedures were included. However, reports on surgical treatments were excluded. Treatment strategies have been selected based on outcomes resulting from computed tomography (CT) and magnetic resonance (MR) imaging. Fat content and tumor size are the most common used indications for AMLs. Unenhanced CT and chemical shift imaging provide good evidence in case of fat-poor AMLs. Chemical analysis and percutaneous biopsy are recommended in case of a diagnostic challenge. A tumor size of 6 cm or larger in diameter might necessitate invasive treatment, while AMLs patients with tumors around 4 cm may require prophylactic treatment. Of all treatment options, embolization indicated a high risk of reintervention. Moreover, everolimus and sirolimus as 2 inhibitors of mammalian target of rapamycin (mTOR) have been evaluated in clinical trials and demonstrated promising outcomes. In conclusion, advances in imaging techniques along with mTOR inhibitors and embolic materials potentiate functional outcomes in AMLs.

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Section: Methodsmentioning

confidence: 99%

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

et al. 2017

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“…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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“…Yamakado et al reported a significant positive association between aneurysm formation and active retroperitoneal hemorrhage. 29 There may be a role for angiography, either conventional renal angiography or renal CTA, in informing treatment decisions, particularly in smaller, asymptomatic angiomyolipoma where surveillance may be considered.…”

Section: Selecting Patients Who May Benefit From Treatmentmentioning

confidence: 99%

Transarterial Embolization of Angiomyolipoma: A Systematic Review

2015

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Renal Angiomyolipoma: Relationships between Tumor Size, Aneurysm Formation, and Rupture

Abstract: Aneurysm formation appears to be related to tumor size, and large aneurysms confer a higher probability of rupture.

Cited by 394 publications

References 18 publications

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

Recent Strategies for the Management of Renal Angiomyolipoma: A Review of Diagnostic and Therapeutic Approaches

Tuberous sclerosis complex renal disease

Transarterial Embolization of Angiomyolipoma: A Systematic Review

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