Wilms tumor is the most common childhood abdominal malignancy, usually occurring in children ages 3-4. It arises from mesodermal precursors in the kidney and presents as a large, heterogeneous mass that commonly invades the renal vein and inferior vena cava. Wilms tumor can be sporadic and unilateral or familial and bilateral. It is associated with congenital anomalies and genetic syndromes. Diagnosis involves imaging like CT or MRI to identify the renal mass along with any metastases.

1. Genito-Urinary
Tumors
Episode 5

2. Wilms Tumor
(Nephroblastoma)
• The most common childhood abdominal malignancy.
• 3–4 years of age.
• ♂ = ♀
• Bilateral in 4%–13% of children.

3. Wilms Tumor
(Nephroblastoma)
1- Sporadic form:
Common, unilateral, 3 Yrs.
2- Familial form:
Uncommon, bilateral, infants, autosomal dominant.

4. Wilms Tumor
(Nephroblastoma)
• May be associated with congenital anomalies
such as:
- Cryptorchidism (2.8% of cases).
- Hemihypertrophy (2.5%).
- Hypospadias (1.8%).
- Sporadic aniridia.

5. Wilms Tumor
(Nephroblastoma)
• WAGR syndrome (Wilms tumor, aniridia,
genitourinary abnormalities, mental retardation).
• Drash syndrome (male pseudohermaphroditism,
progressive glomerulonephritis)
• Beckwith-Wiedemann syndrome
(hemihypertrophy).

6. Wilms Tumor
(Nephroblastoma)
• Arises from mesodermal precursors of the
renal parenchyma (metanephric Blastema).

7. Wilms Tumor
(Nephroblastoma)
Gross Pathology:
• Large solid spherical mass with areas of
hemorrhage and necrosis surrounded with
pseudocapsule.

8. Wilms Tumor
(Nephroblastoma)
Gross Pathology:
Spread:
1. Extend into the renal vein and, subsequently, into the
inferior vena cava.
2. Direct local invasion of adjacent structures.
3. Local regional lymph node metastases.
4. Hematogenous metastases.

9. Wilms Tumor
(Nephroblastoma)
• Palpable mass.
• Hypertension may be present in up to 25% of
cases due to renin production by the tumor
• Hematuria and pain are infrequent clinical
findings.

10. Wilms Tumor
(Nephroblastoma)
Radiological Picture

11. Wilms Tumor
(Nephroblastoma)
Plain X-Ray:
Large soft tissue mass
displacing bowel gas

12. Wilms Tumor
(Nephroblastoma)
IVU:
Large soft tissue mass displacing
bowel gas and distorting and
displacing the collecting system.

13. Wilms Tumor
(Nephroblastoma)
IVU:
Large soft tissue mass displacing
bowel gas and distorting and
displacing the collecting system.

14. Wilms Tumor
(Nephroblastoma)
US:
• Large soft tissue mass
heterogeneous echogenicity, which
represents hemorrhage, necrosis,
or calcification.
• Vascular Invasion.

15. Wilms Tumor
(Nephroblastoma)
CT:
CT demonstrates a well-defined
heterogeneous mass with areas of
calcification, necrosis and
hemorrhage.

16. Wilms Tumor
(Nephroblastoma)
CT:
CT demonstrates a well-defined
heterogeneous mass with areas of
calcification, necrosis and
hemorrhage.

17. Wilms Tumor
(Nephroblastoma)
CT:
CT demonstrates a well-defined
heterogeneous mass with areas of
calcification, necrosis and
hemorrhage.

18. Wilms Tumor
(Nephroblastoma)
CT:
Nodal or hepatic metastases, tumor
extension into the renal vein or
inferior vena cava, contralateral
synchronous tumor, and associated
nephrogenic rests.

19. Wilms Tumor
(Nephroblastoma)
CT:
Nodal or hepatic metastases, tumor
extension into the renal vein or
inferior vena cava, contralateral
synchronous tumor, and associated
nephrogenic rests.

20. Wilms Tumor
(Nephroblastoma)
CT:
Nodal or hepatic metastases, tumor
extension into the renal vein or
inferior vena cava, contralateral
synchronous tumor, and associated
nephrogenic rests.

22. Wilms Tumor
(Nephroblastoma)
MRI:
Well-defined heterogeneous mass with low signal intensity
on T1-weighted images and high signal intensity on T2-
weighted images.

23. Wilms Tumor
(Nephroblastoma)
Angiography: