BERKELEY, CA (UroToday.com) - Tuberous sclerosis complex (TSC) is an autosomal dominant disease of variable penetrance that clinically manifests with sebaceous adenoma, mental retardation and renal lesions. The renal lesions can be cysts, carcinoma or, most commonly angiomyolipomas (AMLs).

The renal AMLs are composed of tortuous blood vessels, muscle and fat. They usually are multiple, bilateral and at risk for spontaneous hemorrhage when larger than 4 cm in size. As renal failure is a major cause of death in TSC patients, renal sparing approaches are critical. Dr. Ewalt and associates at The University of Texas, Southwestern Medical Center and collaborating institutions report their experience using transcatheter embolization of AMLs in the November 2005 issue of The Journal of Urology.

The study includes 16 TSC patients treated between 1994 and 2002. Mean age of first embolization was 23 years. Except for 3 lesions, all were asymptomatic and treated due to size greater than 4cm. Selective embolization was performed via femoral artery access and consisted of injection of polyvinyl alcohol or microspheres with placement of a fibered coil. Repeat CT or MRI imaging was obtained at 3 months and periodically thereafter.

A total of 27 AMLs were embolized in the 16 patients with no instances of intraoperative hemorrhage. Eleven patients were hospitalized for 2 to 7 days with post-embolization syndrome (fever, pain, nausea and vomiting). Fever typically resolved after 48 hours.

All of the 13 patients who had renal imaging 3 months after embolization had decreased size of the embolized AML. Follow-up imaging in 7 patients done 3 to 9 years after treatment did not demonstrate regrowth of the AML. Serum creatinine remained stable in 9 individuals with information available.

This study although with sporadic follow-up, supports the role for selective renal embolization as part of nephron-sparing approaches to long-term management of TSC patients.

J Urol 2005; 174:1764-66