"skipMainNavigation"
Search
Quick Search in Journals
Quick Search anywhere
Advanced Search
Radiology
Menu
Previous
Next
Pediatric Imaging

Tuberous Sclerosis Complex: Renal Imaging Findings

Published Online:Nov 1 2002https://doi.org/10.1148/radiol.2252011584

Abstract

PURPOSE: To review the renal imaging findings and changes over time in a large series of young patients with tuberous sclerosis complex (TSC).

MATERIALS AND METHODS: One hundred thirty-nine renal imaging studies (113 ultrasonographic scans, 15 computed tomographic scans, and 11 magnetic resonance images) were identified in 59 patients with TSC (mean age, 11.4 years; age range, 3 days to 36 years). There were 31 male and 28 female patients. Angiomyolipomas and cysts were identified and characterized according to the following features: number, unilateral versus bilateral, largest diameter, and change over time. In patients whose initial examination results were normal, the age at onset of lesions was noted. The χ2 test was used to determine whether cysts and angiomyolipomas occurred in different subpopulations.

RESULTS: Angiomyolipomas were identified in 47 (80%) patients and were too numerous to count in 36 (76%), focal in 38 (81%), and bilateral in 42 (89%). The mean largest diameter was 21 mm. Cysts were identified in 28 (47%) patients, 18 (64%) of whom had fewer than five cysts. Cysts were bilateral in 17 (61%) patients. The mean largest diameter was 20 mm. In patients with initially normal examination results, the mean age at presentation was 9.0 years for those with cysts and 9.2 years for those with angiomyolipomas. In 80 follow-up examinations, size and/or number increased in 32 (40%) angiomyolipomas and 21 (26%) cysts. Cysts and angiomyolipomas did not occur in significantly different subpopulations (P = .13).

CONCLUSION: Both angiomyolipomas and cysts occur commonly in pediatric patients with TSC and tend to increase in size and number with increasing age. Angiomyolipomas are more common than cysts and tend to be numerous.

© RSNA, 2002

References

  • 1 Ewalt DH, Sheffield E, Sparagana SP, Delgado MR, Roach ES. Renal lesion growth in children with tuberous sclerosis complex. J Urol 1998; 160:141-145.
    Medline Google Scholar
  • 2 Chonko AM, Weiss SM, Stein JH, Ferris TF. Renal involvement in tuberous sclerosis. Am J Med 1974; 56:124-132.
    Medline Google Scholar
  • 3 Bernstein J, Evan AP, Gardner KD, Jr. Epithelial hyperplasia in human polycystic kidney diseases: its role in pathogenesis and risk of neoplasia. Am J Pathol 1987; 129:92-101.
    Medline Google Scholar
  • 4 Kernelly MJ, Grossman HB, Cho KJ. Outcome analysis of 42 cases of renal angiomyolipoma. J Urol 1994; 152:1988-1991.
    Medline Google Scholar
  • 5 Lemaitre L, Claudon M, Dubrulle F, Mazeman E. Imaging of angiomyolipomas. Semin Ultrasound CT MR 1997; 18:100-114.
    Medline Google Scholar
  • 6 Narla LD, Slovis TL, Watts FB, Nigro M. The renal lesions of tuberosclerosis (cysts and angiomyolipoma): screening with sonography and computerized tomography. Pediatr Radiol 1988; 8:205-209.
    Google Scholar
  • 7 Lemaitre L, Robert Y, Dubrulle F, et al. Renal angiomyolipoma: growth followed up with CT and/or US. Radiology 1995; 197:598-602.
    Google Scholar
  • 8 Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993; 150:1782-1786.
    Medline Google Scholar
  • 9 Adler J, Greweldinger J, Litzky G. “Macro” aneurysm in renal angiomyolipoma: two cases, with therapeutic embolization in one point. Urol Radiol 1984; 6:201-203.
    Medline Google Scholar
  • 10 Moorhead JD, Fritzsche P, Hadley HL. Management of hemorrhage secondary to renal angiomyolipoma with selective arterial embolization. J Urol 1997; 117:112-123.
    Google Scholar
  • 11 Ou YC, Wu HC, Yang CR, Chang CL, Hwang TI, Chang CH. Renal angiomyolipoma: experience of 23 patients. Chin Med J 1991; 48:217-223.
    Google Scholar
  • 12 Lee W, Kim TS, Chung JW, Han JK, Kim SH, Park JH. Renal angiomyolipoma: embolotherapy with a mixture of alcohol and iodized oil. J Vasc Interv Radiol 1998; 9:255-261.
    Medline Google Scholar
  • 13 Hartmut PH, Schwarzkopf G, Henske EP. Renal angiomyolipomas, cysts, and cancer in tuberous sclerosis complex. Semin Pediatr Neurol 1999; 5:269-275.
    Google Scholar
  • 14 Clarke A, Hancock E, Kingswood C, Osborne JP. End-stage renal failure in adults with the tuberous sclerosis complex. Nephrol Dial Transplant 1999; 14:988-991.
    Medline Google Scholar
  • 15 Shepherd CW, Gomez MR, Lie JT, Crowson CS. Causes of death in patients with tuberous sclerosis. Mayo Clin Proc 1991; 66:792-796.
    Medline Google Scholar
  • 16 Obuz F, Karabay N, Secil M, Igci E, Kovanlikaya A, Yorukoglu K. Various radiological appearances of angiomyolipomas in the same kidney. Eur Radiol 2000; 10:897-899.
    Medline Google Scholar
  • 17 Povey S, Burley MW, Attwood J, et al. Two loci for tuberous sclerosis: one on 9q34 and one on 16p13. Ann Hum Genet 1994; 58:107-127.
    Medline Google Scholar
  • 18 Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol 1993; 150:1782-1786.
    Medline Google Scholar
  • 19 Koike H, Muller SC, Hohenfellner R. Management of renal angiomyolipoma: a report of 14 cases and review of the literature—is nonsurgical treatment adequate for this tumor? Eur Urol 1994; 25:183-188.
    Medline Google Scholar
  • 20 Osterling JE, Fishman EK, Goldman SM, Marshall FF. The management of renal angiomyolipoma. J Urol 1986; 135:1121-1124.
    Medline Google Scholar
  • 21 Brook-Carter PT, Peral B, Ward CJ, et al. Deletion of the TSC2 and PKD1 genes associated with severe infantile polycystic kidney disease: a contiguous gene syndrome. Nat Genet 1994; 8:328-332.
    Medline Google Scholar
  • 22 Sampson JR, Maheshwar MM, Aspinwall R, et al. Renal cystic disease in tuberous sclerosis: role of the polycystic kidney disease 1 gene. Am J Hum Genet 1997; 61:843-851.
    Medline Google Scholar
  • 23 Dabora SL, Jozwiak S, Franz DN, et al. Mutational analysis in a cohort of 224 tuberous sclerosis patients indicates increased severity of TSC2, compared with TSC1, disease in multiple organs. Am J Hum Genet 2001; 68:64-80.
    Medline Google Scholar
  • 24 Kleymenova E, Ibraghimov-Beskrovnaya O, Kugoh H, et al. Tuberin-dependent membrane localization of polycystin-1: a functional link between polycystic kidney disease and the TSC2 tumor suppressor gene. Mol Cell 2001; 7:823-832.
    Medline Google Scholar

Article History

Published in print: Nov 2002