Evidence is strong for a genetic/developmental etiology.
The tumor may arise as a result of failure of blastemal tissue to differentiate into normal renal structures.,
Occurs in children, usually under age 5.
Peak incidence between ages 1 and 3.
Occurs in approximately 7 in a million children per year in the U.S.
Occurs as part of WARG syndrome (Wilms tumor, aniridia, retardation, genital abnormalities).
Occurs as part of Denys-Drash syndrome (gonadal dysgenesis and nephropathy leading to renal failure).
Occurs as part of Beckwith-Wiedemann syndrome (organomegaly, hemihypertrophy, renal cysts, adrenal cytomegaly).
|General Gross Description|
At the time of detection, Wilms tumors are usually large and dwarfs the native kidney.
The cut surface is usually soft, fleshy and brain-like.
Irregular areas of hemorrhage and necrosis may be present.
|General Microscopic Description|
The classic Wilms tumor is triphasic showing areas that are blastemal (non-descript undifferentiated small round cells), areas that show epithelial differentiation (tubules, glomeruloid structures) and areas that show stromal differentiation (spindle shaped cells, skeletal muscle).
Mitotic figures are usually plentiful.
Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 462-464.
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||Synopsis by: Harold Yamase M.D. (T71000M89603)