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

Anil Kapoor; Louis Girard; Jean-Baptiste Lattouf; York Pei; Ricardo Rendon; Paul Card; Alan So

doi:10.1016/j.urology.2015.12.009

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

Urology. 2016 Mar:89:19-26. doi: 10.1016/j.urology.2015.12.009. Epub 2015 Dec 23.

Authors

Anil Kapoor¹, Louis Girard², Jean-Baptiste Lattouf³, York Pei⁴, Ricardo Rendon⁵, Paul Card⁶, Alan So⁷

Affiliations

¹ Division of Urology, McMaster University, Hamilton, Ontario, Canada.
² Department of Medicine, The University of Calgary, Alberta, Canada.
³ Department of Surgery, University of Montreal, Montreal, Quebec, Canada.
⁴ University Health Network, Toronto General Hospital, Toronto, Ontario, Canada.
⁵ QEII Health Sciences Centre-Department of Urology, Dalhousie University, Halifax, Nova Scotia, Canada.
⁶ Kaleidoscope Strategic, Toronto, Ontario, Canada.
⁷ University of British Columbia, BCCA, Prostate Centre at Vancouver General Hospital, Vancouver, British Columbia, Canada. Electronic address: dralanso@mail.ubc.ca.

PMID: 26723178
DOI: 10.1016/j.urology.2015.12.009

Abstract

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.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Angiomyolipoma / therapy*
Asymptomatic Diseases
Decision Trees
Humans
Neoplasms, Multiple Primary / therapy*
Practice Guidelines as Topic
Tuberous Sclerosis*