|
|
|
|
|
|
| Wilms tumor |
| Etiology •Evidence is strong for a genetic/developmental etiology. |
| Pathogenesis •The tumor may arise as a result of failure of blastemal tissue to differentiate into normal renal structures., |
| Epidemiology •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. |
| Clinical Correlation •The clinical presentation is that of a large abdominal mass in a child, usually 1 to 5 years old. •The neoplasm is aggressive and metastasizes widely, but is responsive to combination therapy. |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 462-464. |
| Wilms tumor |
| Synopsis by: Harold Yamase M.D. (T71000M89603)[198] |
| Search Medline at National Library of Medicine Please be patient during transfer. Medline will open in a new window. To return, close the Medline Window |
|
|
|
|
|
|