Wallach's Interpretation of Diagnostic Tests: Pathways to Arriving at a Clinical Diagnosis (558 page)

Suggested Reading

Rini BI, Campbell SC, Escudier B. Renal cell carcinoma.
Lancet.
2009;373(9669):1119–1132.

WILMS TUMOR

   Definition

   It is the most common renal malignancy in childhood (95% of cases are diagnosed before 10 years of age).

   This tumor has been associated with loss-of-function mutations of a number of tumor suppressor genes including the
WT1, FTW1, FTW2,
and
p53
genes.

   Diagnosis is by histologic examination of a renal biopsy or the tumor removed surgically.

   Who Should Be Suspected?

Candidates are children between the ages of 3 and 10 years, with an abdominal mass, hematuria, abdominal pain, or hypertension.

   Laboratory Findings

   Urinalysis may show proteinuria in some cases.

   Serum creatinine may be elevated. Liver function tests, if abnormal, may indicate the presence of hepatic metastasis. Hypercalcemia may accompany other associated syndromes.

   Studies for Von Willebrand disease (vWD) are indicated since up to 8% of affected children have acquired vWD disease and may bleed at surgery.

   Genetic studies of tumor suppressor genes can be helpful.

   
RENAL DISORDERS IN SELECTED DISEASES

AMYLOIDOSIS-ASSOCIATED KIDNEY DISEASE

   Overview

   See primary amyloidosis.