Cystic Nephroma In A 15-Month-Old Boy With Dicer1 Mutation

Cystic nephroma is a rare tumor in childhood. Seventy percent of all cases are associated with DICER1-anomaly and therefore cystic nephroma represents the second most common tumor of all patients with DICER1 mutation. We present a case of a 15-month-old boy with a DICER1 mutation and cystic nephroma. Organ-sparing surgery was not possible. Due to higher prevalence of germ cell tumors, follow-up after nephrectomy is necessary until adulthood.