[size=20px]Children with which of the following conditions should receive screening renal ultrasounds for nephroblastoma (Wilms tumor)?

 

A a teenager who had unilateral Wilms’ at four years of age

 

B Denys-Drash syndrome

 

C end stage renal failure

 

D cryptorchidism

 

E isolated Wilms’ tumor in a sibling[/size]

B Denys-Drash syndrome

B Denys-Drash syndrome

Correct

 

Wilms tumor usually presents as an asymptomatic abdominal mass in an otherwise healthy child. Up to one third of patients will have associated symptoms of abdominal pain, fever, anemia, hematuria or hypertension.

 

Current theories of tumor development suggest a genetic mutation that predisposes to the persistence of nephrogenic rests. Nephrogenic rests are also present in the predisposition syndromes of WAGR (Wilms tumor- aniridia - genital anomalies - retardation), Denys-Drash (nephropathy, intersex disorders), Beckwith-Wiedemann (hemihypertrophy, macroglossia, hypoglycemia) and isolated hemihypertrophy. These patients may benefit from screening ultrasound. The frequency of screening is dependent on the syndrome.

 

Current recommendations suggest surveillance abdominal ultrasound examinations for the above patients, siblings of patients with familial Wilms and offspring of patients with bilateral Wilms.

 

Patients who have finished therapy for Wilms should have ultrasound examinations every three months until the age of seven years.

B