RGD Reference Report - Nephron-sparing resection of angiomyolipoma after sirolimus pretreatment in patients with tuberous sclerosis. - Rat Genome Database

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Nephron-sparing resection of angiomyolipoma after sirolimus pretreatment in patients with tuberous sclerosis.

Authors: Staehler, M  Sauter, M  Helck, A  Linsenmaier, U  Weber, L  Mayer, K  Fischereder, M 
Citation: Staehler M, etal., Int Urol Nephrol. 2012 Dec;44(6):1657-61. doi: 10.1007/s11255-012-0292-z. Epub 2012 Sep 28.
RGD ID: 7245506
Pubmed: PMID:23054313   (View Abstract at PubMed)
DOI: DOI:10.1007/s11255-012-0292-z   (Journal Full-text)

PURPOSE: Renal angiomyolipoma in patients with tuberous sclerosis can cause life-threatening bleeding. Embolization or resection is recommended, but either intervention may result in substantial loss of renal function. Recently, regression of angiomyolipoma size has also been achieved with mTOR inhibitor therapy, but recurrent lesion growth after treatment cessation has to be expected. This is the first report on a multimodal therapeutic approach facilitating a nephron-sparing, definitive resection. METHODS: Three patients with renal angiomyolipoma not amenable to nephron-sparing surgery were treated with sirolimus aiming trough levels of 4-8 ng/ml. Treatment was well tolerated. RESULTS: mTOR inhibitor treatment resulted in a regression of angiomyolipoma volume by 38-95 %. Thereafter, nephron-sparing angiomyolipoma resection conserved normal renal function. CONCLUSION: Neoadjuvant use of mTOR inhibitor pretreatment may represent a novel approach facilitating nephron-sparing resection.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
MTORHumankidney angiomyolipoma treatmentIMP associated with Tuberous SclerosisRGD 
MtorRatkidney angiomyolipoma treatmentISOMTOR (Homo sapiens)associated with Tuberous SclerosisRGD 
MtorMousekidney angiomyolipoma treatmentISOMTOR (Homo sapiens)associated with Tuberous SclerosisRGD 

Objects Annotated

Genes (Rattus norvegicus)
Mtor  (mechanistic target of rapamycin kinase)

Genes (Mus musculus)
Mtor  (mechanistic target of rapamycin kinase)

Genes (Homo sapiens)
MTOR  (mechanistic target of rapamycin kinase)


Additional Information