BERKELEY, CA (UroToday.com) - Tuberous Sclerosis Complex (TSC) is an autosomal dominant genetic syndrome, where renal manifestations include renal cystic disease, renal cell carcinoma, and angiomyolipomas (AML). Eighty percent of older children and adults with TSC develop multifocal AML's, and the most common cause of death in adults with TSC is due to AML's with resultant hemorrhage or renal failure. While certainly TSC is a rare genetic syndrome, the practicing urologist can gain some insight into the outcomes for embolization of AML's of the kidney, through the experience reported by Ewalt and colleagues in the TSC population.

The authors report on 16 patients with TSC who presented with AML's ranging from 4 - 21 cm in size. These 16 patients underwent 18 procedures to embolize a total of 27 tumors. Three patients had embolization of tumors in a solitary kidney and two patients required 2 separate embolization sessions to complete the therapy. Eight patients had more than one tumor embolized in a single session. Following embolization therapy, no patient had renal failure requiring dialysis, and no patient required blood transfusion due to hemorrhage. Following embolization, tumors were noted to regress significantly in the 13 patients who received post-embolization imaging at 3 months, and there was no regrowth of the embolized lesions in the 7 patients who had imaging 3 - 9 years post-therapy. Following embolization, 11 patients required hospitalization for 2-7 days due to post-embolization syndrome, which consisted of fever, pain, nausea, and vomiting, all of which was treated with supportive care. Of note, the incidence of the post-embolization syndrome was not related to the size or number of lesions treated.

Angioembolization of AML's remains a viable treatment option for patients with TSC and also for the population at large. It is minimally invasive, associated with minimal toxicity, results in nephron conservation, and adequately controls even large AML's that are threatening and at risk for hemorrhagic complications.

J Urol 174: 1764-1766, November, 2005