Magnetic resonance imaging of renal involvement in genetically studied patients with tuberous sclerosis complex

Eur J Radiol. 2009 Nov;72(2):335-41. doi: 10.1016/j.ejrad.2008.07.046. Epub 2008 Oct 2.

Abstract

Purpose: To evaluate renal involvement in tuberous sclerosis complex (TSC).

Materials and methods: A series of 24 TSC patients (19 with genetically demonstrated disease), underwent abdominal 1.0-T MR imaging with axial/coronal T1- and T2-weighted scans, with/without fat saturation. We looked for angiomyolipomas (AMLs) and cysts in 47 kidneys of 24 patients. We evaluated the percentage of parenchymal involvement by manual contouring on the coronal scans in 39 kidneys of 20 patients.

Results: We detected AMLs in 15/24 (63%) patients and in 27/47 (57%) kidneys, cysts in 14/24 (58%) and in 26/47 (55%); respectively. AMLs were found in 2/4 TSC1 and in 11/15 TSC2 patients, cysts were found in 2/4 TSC1 and in 9/15 TSC2 patients. The global renal involvement ranged from 0 to 32% (median, 18%) in TSC1 and from 0 to 100% (median, 39%) in TSC2 patients. A fair correlation (r=.464) was found between patient's age and renal involvement, a good correlation (r=0.655) between renal involvement and creatinine clearance.

Conclusion: Renal parenchyma of TSC patients can be evaluated with MR imaging. A detectable renal involvement was found in 83% of patients, higher in TSC2 than in TSC1. Renal function seems to correlate with renal involvement quantified with MR imaging.

MeSH terms

  • Adolescent
  • Adult
  • Angiomyolipoma / complications
  • Angiomyolipoma / diagnosis*
  • Angiomyolipoma / genetics*
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Liver Neoplasms / complications
  • Liver Neoplasms / diagnosis*
  • Liver Neoplasms / genetics*
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged
  • Tuberous Sclerosis / complications
  • Tuberous Sclerosis / diagnosis*
  • Tuberous Sclerosis / genetics*
  • Young Adult