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PEDIATRIC RENAL ANGIOMYOLIPOMAS IN TUBEROUS SCLEROSIS COMPLEX
Katie BRODIE
1
, Erin GRANTHAM
1
, Jason WARNCKE
2
, Salvatore CATARRINICHIA
2
, Suhong TONG
3
, Kimi KONDO
4
and
Nicholas COST
1
1) Children's Hospital Colorado, Department of Pediatric Urology, Aurora, USA - 2) University of Colorado, Department
of Urology, Aurora, USA - 3) Children's Hospital Colorado, Child Health Research Biostatistics Core, Aurora, USA - 4)
University of Colorado, Department of Radiology, Aurora, USA
PURPOSE
Tuberous Sclerosis Complex (TSC) is a multisystem, genetic disorder. Renal manifestations are a major cause
of morbidity. We describe the natural history of renal angiomyolipomas (AML) in children with TSC.
MATERIAL AND METHODS
We conducted an IRB approved chart review. Demographics, renal function, imaging, extrarenal manifestations,
genetics, and treatments were recorded.
RESULTS
We found 193 (89 female) with TSC; 145 had renal imaging studies (range 1-18 studies per patient).
AMLs were seen in 23 (11.9%) 0-6 year olds, 29 (15%) 7-11 year olds, 20 (10.4%) 12-16 year olds, and
12 (6.2%) 17+ year olds. Measurable (>5mm) AMLs were seen in 11 (6%) 0-6 year olds, 20 (10.4%) 7-11 year olds,
13 (6.7%) 12-16 year olds, and 8 (4%) 17+ year olds.
One nine-year-old had an AML >4 cm, no other young patients had concerning AMLs. AML growth increased in
patients >11 years, and in AMLs >1cm. Median AML growth in 12-16 year olds was 2.5mm/year (maximum 28.6).
Median growth of AMLs 1-1.9cm was 1.4mm/year (maximum 4.6mm/year). Median growth of AMLs 2-2.9cm was
4.28mm/year (maximum 114.1).
Seven patients (3.6%) underwent a total of 13 angioembolizations or surgeries. Thirty-five patients (18.1%)
took mTor inhibitors. Ninety-four of 96 with creatinine measured had normal glomerular filtration rates.
CONCLUSIONS
Larger AMLs occur mostly in patients > 11 years. Growth can be rapid and unpredictable. We recommend annual renal
ultrasounds of pediatric patients with TSC, with closer follow up or MRI for patients with measurable AML >11 years, or
with larger AMLs at any age.