Review Question - QID 212391

This patient with fever, abdominal pain, dysuria, hypertension, and an abdominal mass that does not cross the midline most likely has Wilms tumor. Wilms tumor is associated with aniridia.

Wilms tumor is an embryonal malignancy of the kidney that is the most common renal tumor of childhood. It is seen in children under the age of 4 and presents with fever, abdominal pain, dysuria, and hypertension due to the ectopic production of renin. Physical exam in these patients will reveal a palpable abdominal mass that does not cross the midline, which distinguishes this tumor from neuroblastoma, which does cross the midline. Wilms tumors can be seen in syndromic forms such as WAGR syndrome, which also includes aniridia, genitourinary abnormalities, and mental retardation.

Incorrect Answers:
Answer 1: 11;22 chromosome translocation is associated with Ewing sarcoma, which is a childhood cancer that would present as a tumor in the bone rather than as an abdominal mass.

Answer 3: Ash leaf spots are associated with tuberous sclerosis, which can present with soft tissue tumors; however, this patient does not have any of the classic findings of tuberous sclerosis.

Answer 4: Epstein-Barr virus is associated with Burkitt lymphoma; however, this tumor is not associated with dysuria or hypertension.

Answer 5: Opsoclonus myoclonus is associated with neuroblastomas; however, this tumor would cross the midline as opposed to Wilms tumor which does not cross the midline.

Bullet Summary:
Wilms tumor presents in children under 4 years of age with fever, abdominal pain, dysuria, hypertension, and an abdominal mass and can be associated with aniridia, genitourinary abnormalities, and mental retardation in WAGR syndrome.