Peds onc Flashcards
Which syndromes associates with Wilms?
Denys-Drash
WAGR
Beckwith-Wideman
What genetic mutation is associated with Wilms?
WT-1 and WT-2
Of kids with Denys-Drash syndrome, what percentage will get Wilms?
80%
Describe a typical Denny’s Drash child
Usually born with ambiguous genitalia. Soon thereafter kidney nephrotic syndrome develops and HTN ensues. Ultimately almost all will develop both ESRD requiring dialysis, and bilateral Wilms requiring nephrectomy. This all typically occurs before age 5.
Describe the typical child with Beckwith Widemann?
It is not usually recognized until after birth. These children have abnormal overgrowth usually on one side of their body along with omphalocele, a large tongue, and can develop bilateral Wilms tumors. The risk is MUCH lower than Denys Drash - only 4-10%.
What is aniridia and why is it associated with Wilms?
It is a congenital condition where you are missing all or part of the iris. It gives the pupil a strange misshapen appearance. It is associated with Wilms because some of these people have an abnormal WT-1 gene. But not all. Typically, these people are screened until age 7 for Wilms tumor.
What is the differential diagnosis of an abdominal mass in a child?
Neuroblastoma Wilms Clear cell sarcoma Congenital mesoblastic nephroma RCC Rhabdoid Tumor
What are the worst two types of pediatric abdominal tumors?
Rhabdoid Tumor
Clear cell sarcoma
Both metastasize to brain so brain imaging needed. Clear cell also goes to bone.
Is tumor spillage bad during Wilms Tumor resection?
Yes, results in upstaging from Stage 2 to Stage 3
What is typical upfront treatment for Wilms?
Usually surgery (unless bilateral Wilms)
After surgical resection for Wilms tumor, what is the mainstay of therapy?
Chemotherapy and radiation based on stage
