REVIEW OF PEDIATRIC RENAL NEOPLASMS
John Hicks
Texas Children's Hospital, Houston, TX

Associated with Tuberous Sclerosis (50%)
80% of Tuberous Sclerosis Patients with Tumor
May Be Discovered in Childhood and Adolescence
(Mean Age 41 years)

Sporadic Tumors (50%)
Von Recklinghaus Disease, Autosomal Dominant
Polycystic Kidney Disease

Gender Ratio 2.0F: 1.0M

Symptom:
Flank Pain Due to Intra-tumoral Hemorrhage

Hemorrhage, Necrosis and Thrombosis Common

Unique Phenotypic Features of Smooth Muscle Cells
HMB-45: Perimembranous Pattern
PAS-Positive, Diastase-Resistant Crystalloid Material

Tuberous Sclerosis More Likely If:
Multiple Hamartomas in Adjacent Kidney
Calcified Spicules
Tubular Inclusions

Single Documented Case of SarcomatousTransformation