Long-term outcome of transcatheter embolization of renal angiomyolipomas due to tuberous sclerosis complex.
ABSTRACT Complications from renal angiomyolipomas (AMLs) are common in patients with tuberous sclerosis complex (TSC) and tumors greater than 4 cm are more likely to cause symptoms. AMLs are the most common cause of death in adults with TSC. We present our long-term experience with transcatheter tumor embolization as a definitive treatment for AMLs due to TSC.
A total of 16 patients with TSC between 7.5 and 47.2 years old with symptomatic or large (4 to 21 cm) AMLs underwent embolization. Followup consisted of periodic physician visits or telephone contacts and renal imaging.
The 16 patients underwent 18 treatment sessions to embolize 27 tumors. There were no intraoperative complications. The post-embolization syndrome occurred in 11 individuals but all responded to medical management. Two individuals had an arterial aneurysm within a tumor. The AML size decreased in the 13 patients who were imaged 3 months after treatment, and the 7 patients who were imaged 3 to 9 years after treatment have shown no tumor regrowth. No renal failure or hemorrhage has developed in patients following embolization.
Transcatheter embolization of symptomatic or large AMLs due to TSC prevents hemorrhage and renal loss. The treatment is minimally invasive, preserves renal function, and can be performed multiple times. All of the patients who underwent followup renal imaging after embolization showed decreased AML size, and none of the 16 patients has developed renal loss or renal insufficiency in these individuals. Embolization should be considered the initial treatment of choice for large or symptomatic AMLs.
- SourceAvailable from: John J Bissler[show abstract] [hide abstract]
ABSTRACT: Approximately 75% of patients with tuberous sclerosis complex develop renal angiomyolipomas. These hamartomatous lesions distort and damage renal parenchyma and can lead to hemorrhage. To reduce the risk of hemorrhage, transarterial embolization is used to necrose the angiomyolipoma while sparing normal renal tissue. Although an effective renal-sparing procedure, embolization most often is associated with an inflammatory response that causes significant fever and pain that can last for several days despite the use of acetaminophen. Reported cases show that 49 of 55 patients who underwent embolization developed this syndrome. The use of such nonsteroidal anti-inflammatory drugs as aspirin is contraindicated because of their adverse effects on platelet function. To reduce pain and fever associated with postembolization syndrome (PES), we changed our clinical management of patients postembolization to include a tapering dose of prednisone over a 2-week period. Nine patients underwent this pharmacological intervention, and one patient abstained. All patients were monitored for pain and fever. Only two patients treated with steroids developed fever, which was assuaged with acetaminophen, and no patient reported pain. The tapering dose of prednisone was well tolerated, and there were no postprocedure infections. The use of a short-term tapering dose of prednisone appeared to reduce PES compared with the reported literature and improved patient comfort.American Journal of Kidney Diseases 06/2002; 39(5):966-71. · 5.29 Impact Factor
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ABSTRACT: Renal lesions, including angiomyolipoma, renal cysts (simple and polycystic kidney disease) and renal cell carcinoma, develop in patients with tuberous sclerosis complex. While there is limited information that these lesions may grow in adults with tuberous sclerosis complex, the incidence, characterization and growth rate in children have not been reported. Also, the age at which these lesions first appear, thus providing insight into their natural history, is unknown. We present our data from a longitudinal renal surveillance study of children with tuberous sclerosis complex. Since 1985 children with tuberous sclerosis complex at our hospital have undergone periodic renal imaging by ultrasonography or computerized tomography to monitor renal lesions. A total of 35 girls and 25 boys 1 to 18 years old have undergone at least 2 or more annual renal ultrasounds. On initial evaluation 33 of 60 children (55%) (mean age 6.9 years) had an identifiable renal lesion, which increased to 48 of 60 (80%) at followup (mean age 10.5 years). Angiomyolipoma was the most frequent lesion (75%) followed by simple renal cysts (17%). Angiomyolipomas increased in size and/or number in 10 of 18 boys (56%) and 18 of 27 girls (66%). The largest growth rate in 1 year was from 0 to 4 cm. and from 5 to 9 cm. in diameter. The youngest patient demonstrated lesions at age 2 years. The average age at which a normal ultrasound became abnormal was 7.2 years. While a total of 27 patients had a normal ultrasound on entering the study, lesions had developed in 15 at followup (11 with angiomyolipomas, 4 with cysts). Five patients had cysts that had disappeared at followup. A 7-year-old boy had a 9 cm. renal cell carcinoma removed. One patient has renal lesions characteristic of autosomal dominant polycystic kidney disease. Renal involvement in patients with tuberous sclerosis complex begins in infancy, and angiomyolipoma is the most common lesion (75%). Angiomyolipomas are more likely to grow than remain stable, although the rate of growth varies. Simple renal cysts may appear or disappear with time but angiomyolipomas do not disappear. An initially normal renal ultrasound does not rule out future development of lesions. Periodic surveillance is indicated in children with tuberous sclerosis complex.The Journal of Urology 08/1998; 160(1):141-5. · 3.70 Impact Factor
- Journal of the American College of Surgeons 12/1998; 187(5):548-61. · 4.50 Impact Factor