Wieder's 2 - Wilms' & Peds Renal Tumors

jahen86's version from 2015-05-25 03:37


Question Answer
avg age of presentation?4yo, earlier if b/l
if a newborn has a renal mass, what is it likely?nephroma or neuroblastoma, usually Wilms forms much later
most common presenting symptom?abd/flank mass (doesn't cross midline), also a/w hypertension; rarely hematuria
arise from what cells?abnormal persistence of metanephric blastema (embryologic structure containing nephrogenic cells, i.e. nephrogenic rests)
defn nephroblastomatosisdiffuse multi-focal nephrogenic rests (premalignant lesions)
chrm location of Wilms' tumor genes WT1 & WT211p13 and 11p15 respectively, loss of 16q heterozygosity also results in Wilms'
3 most common met sitespulm, liver, bone
WAGR syndromeWilms', aniridia, GU anomalies, retardation; a/w WT1
Denys-Drash syndromeWilms, intersex d/o, glomerulopathy; a/w WT1
Beckwith-Wiedemann syndromegrowth abnormalities, gigantism, hemihypertrophy, omphalocele; a/w WT2
Why is it important to test coags (plts/PT/PTT) in Wilms'?5-10% pts develop acquired von Willebrand dz
Initial H&P w/u?eye exam, hypertrophy?, hypospadia?, cryptorchidism?, blood pressure?
Initial imaging w/u?ultrasound
Initial therapy?usually surgical exploration; sometimes neoadjuvant chemo
Indications for neoadjuvant chemob/l tumors; unresectable at presentation; extensive caval involvement