An 4-year-old male is brought to the urgent care in respiratory distress. The parents state symptoms began gradually over several days. The child has been otherwise healthy.

On physical exam, vital signs show blood pressure 100/70, pulse 95, temperature 100, respiratory rate 35, and oxygen saturation of 94% on room air. The patient is in moderate distress and utilizing accessory muscles of respiration. There is dullness to percussion over the lower and middle lobes of the right lung, absent breath sounds, and decreased tactile fremitus. Abdominal exam is notable for a large, hard, irregular mass palpated along the right mid-axillary line from the bottom of the liver down to the level of the umbilicus. There is no hepatosplenomegaly or tenderness to palpation.

CBC shows mild normochromic normocytic anemia. Electrolytes are significant for decreased serum calcium and increased serum phosphate levels. Creatinine and BUN are elevated. Urinalysis, cytogenetics, and coagulation studies are pending.

Chest x-ray shows a large right pleural effusion. EKG is normal.

Supplemental oxygen is given. Thoracentesis of the right pleural effusion is performed. Cytology and chemistry of the fluid is pending.

What is the best next step in management?

  1. Abdominal and renal ultrasound
  2. Serial abdominal examinations
  3. Fine needle aspiration of the mass and cytology
  4. Contrast CT of the chest, abdomen and pelvis
  5. T1, T2 MRI with and without contrast
Explanation

Answer: A - In pediatric patients with a painless sizeable abdominal mass that has caused malignant pleural effusion, a Wilms tumor is suspected. Abdominal and renal ultrasound will establish the size of the tumor, and whether it involves the inferior vena cava.

Over-manipulation and fine needle aspiration of a Wilms tumor is contraindicated due to the possibility of rupturing the capsule and seeding of the abdomen with tumor cells. Contrast CT can help confirm the diagnosis, establish the involvement of the inferior vena cava, and ascertain the presence of metastatic disease. MRI is indicated for cases where inferior vena cava involvement and patency is equivocal on CT, which is superior to CT for that.

Wilms' tumor is a rare kidney cancer that primarily affects children. Also known as nephroblastoma, Wilms' tumor is the most common cancer of the kidneys in children. Wilms' tumor most often affects children ages 3 to 4 and becomes much less common after age 5.

Wilms' tumor most often occurs in just one kidney, though it can sometimes be found in both kidneys simultaneously.

Sources: Mayo Clinic

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