Evolving Strategies in the Treatment of Tuberous Sclerosis Complex-associated Angiomyolipomas (TSC-AML)

Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder characterized by the development of numerous benign tumors that occur in multiple organ systems throughout the lifetime of the affected individuals. Renal angiomyolipomas occur in up to 80% of TSC patients, and chronic ki...

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Bibliographic Details
Published in:Urology (Ridgewood, N.J.) N.J.), 2016-03, Vol.89, p.19-26
Main Authors: Kapoor, Anil, Girard, Louis, Lattouf, Jean-Baptiste, Pei, York, Rendon, Ricardo, Card, Paul, So, Alan
Format: Article
Language:English
Subjects:
Angiomyolipoma - therapy
Asymptomatic Diseases
Decision Trees
Humans
Neoplasms, Multiple Primary - therapy
Practice Guidelines as Topic
Tuberous Sclerosis
Urology
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Summary:Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder characterized by the development of numerous benign tumors that occur in multiple organ systems throughout the lifetime of the affected individuals. Renal angiomyolipomas occur in up to 80% of TSC patients, and chronic kidney disease from increasing tumor burden is the primary cause of TSC-related mortality. Our review evaluates evidence for localized and systemic therapy in the management of TSC-angiomyolipomas. Urologists or nephrologists experienced in TSC disease should coordinate the care of TSC patients with renal involvement to improve care and reduce costs.
ISSN:0090-4295
1527-9995
DOI:10.1016/j.urology.2015.12.009