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Virtual Pediatric Hospital: Paediapaedia: Wilms Tumor Paediapaedia: Genitourinary Diseases

Wilms Tumor

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Most kids are asymptomatic, with an abdominal mass being found incidentally. Can also present with hypertension or hematuria. May be associated with aniridia, hemihypertrophy, and horseshoe kidney. It is the most common primary malignant abdominal neoplasm in children, comprising 22% of all abdominal masses beyond the newborn age and 10% of all malignant tumors in the pediatric age group. The peak incidence is at 3 years old.

Etiology/Pathophysiology:
Originates in the kidney, displaces and distorts the pelvicalyceal system, and replaces most of the involved kidney, and has central hemorrhage and necrosis. Can invade the renal vein and IVC (12-20% ), and can spread via blood, lymphatics, and direct extension. The most common site of metastases is the lung, liver metastases are seen in 8-10% , and bone metastases are rare. Five to ten percent are bilateral and this can be due to a malignant progression of nephroblastomatosis, multicentric tumor, or metastases. Nephroblastomatosis has no capsule, hemorrhage, or necrosis unlike Wilms which has all three.

Pathology:
Fairly well differentiated renal tissue with embryonic glomeruli and tubules.

Imaging Findings:
Abdominal film shows a non calcified abdominal mass.

Ultrasound shows a solid mass arising from the kidney with a heterogenous echotexture due to hemorrhage and necrosis that deforms the renal collecting system.

Imaging work up should include an ultrasound for initial imaging of the abdominal mass to include a color Doppler ultrasound to assess for renal vein invasion and thrombosis, a MR or CT to assess for disease extent, and a bone scan to look for bone metastases.

DDX:
Renal Masses

References:

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