[size=5]An 18-month-old child with Beckwith-Weidemann syndrome is found on screening imaging to have a left renal mass that is 50% of the left renal volume. There is no tumor thrombus or right sided renal mass. The most appropriate next step in management is:

 

a Chemotherapy

 

b Tumor biopsy

 

c Left radical nephrectomy

 

d Left radical nephrectomy with right renal exploration

 

e Nephron-sparing resection of the left renal mass[/size]

A

[size=5]An 18-month-old child with Beckwith-Weidemann syndrome is found on screening imaging to have a left renal mass that is 50% of the left renal volume. There is no tumor thrombus or right sided renal mass. The most appropriate next step in management is:

 

a Chemotherapy

 

b Tumor biopsy

 

c Left radical nephrectomy

 

d Left radical nephrectomy with right renal exploration

 

e Nephron-sparing resection of the left renal mass[/size]

c

A followed by E

correct answer

a Chemotherapy

 

Patients with Beckwith-Weidemann syndrome are at risk for developing Wilms tumor and should undergo screening ultrasound every 3 months until 5 years of age. Due to their high risk for developing metachronous tumors, nephron-sparing nephrectomy is appropriate when tumors are identified. However, neoadjuvant chemotherapy should be administered to reduce tumor volume and allow for salvage of more kidney. Radical nephrectomy is not appropriate as it would place the patient at increased risk for renal insufficiency if a contralateral Wilms tumor developed later. Contralateral exploration is not indicated with contemporary imaging and there is no role for renal biopsy at diagnosis.

 

A