Medical MCQ-A 4 year old boy diagnosed of having a malignancy. On physical examination, he found to have aniridia. Which of the following tumor is associated with aniridia?

A 4 year old boy diagnosed of having a malignancy. On physical examination, he found to have aniridia. Which of the following tumor is associated with aniridia?

Hepatoblastoma

Medulloblastoma

Nephroblastoma

Retinoblastoma

Question Category:

Pediatrics

Correct Answer:

Nephroblastoma

Description:

Wilms Tumor (Nephroblastoma): After neuroblastoma, this is the second most common abdominal tumor in children. Symptoms consist of abdominal enlargement, pain, hematuria, malaise, weakness, anorexia, weight loss and fever. Malformations or syndromes associated with Wilms tumor: Aniridia Hemihyperophy Genitourinary malformations Cryptorchidism Hypospadias Gonadal dysgenesis Pseudohermaphroditism Horseshoe kidney Beckwith-Wiedemann syndrome Denys-Drash syndrome WAGR syndrome (Wilms tumor, aniridia, ambiguous genitalia, mental retardation) Ref: Graham D.K., Quinones R.R., Keating A.K., Maloney K., Foreman N.K., Giller R.H., Greffe B.S. (2012). Chapter 31. Neoplastic Disease. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

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