Nokea
You receive a phone call from a community pediatrician who is caring for a 2-year-old toddler with a cancer predisposition syndrome. The pediatrician describes a child at the 95th percentile for height and weight with a history of corrective oral surgery to reduce a large tongue and a history of an omphalocele in infancy. The pediatrician is currently performing ultrasound of the abdomen and laboratory evaluation for this patient every 3 months. Which tumor is this patient most at risk of developing?
Correct Answer: D
Rationale: The correct answer is D: Nephroblastoma. This patient most likely has Beckwith-Wiedemann Syndrome (BWS) due to the large tongue (macroglossia) and omphalocele. BWS is associated with an increased risk of nephroblastoma (Wilms tumor). The 95th percentile for height and weight is also a characteristic feature of BWS. Ultrasound and laboratory evaluation are appropriate monitoring tools for nephroblastoma in this patient due to the increased risk associated with BWS.
A: Pleuropulmonary blastoma is more commonly associated with DICER1 syndrome, not BWS.
B: Hepatocellular carcinoma is not commonly associated with BWS.
C: Cystic nephroma is not typically associated with BWS, and nephroblastoma is a more common tumor in this context.