Genito-Urinary Tumors

Episode 5

Wilms Tumor(Nephroblastoma)

• The most common childhood abdominal malignancy.

• 3–4 years of age.

• ♂ = ♀• Bilateral in 4%–13% of children.

Wilms Tumor(Nephroblastoma)

1- Sporadic form:

Common, unilateral, 3 Yrs.

2- Familial form:

Uncommon, bilateral, infants, autosomal dominant.

Wilms Tumor(Nephroblastoma)

• May be associated with congenital anomalies

such as: - Cryptorchidism (2.8% of cases). - Hemihypertrophy (2.5%). - Hypospadias (1.8%). - Sporadic aniridia.

Wilms Tumor(Nephroblastoma)

• WAGR syndrome (Wilms tumor, aniridia,

genitourinary abnormalities, mental retardation).

• Drash syndrome (male pseudohermaphroditism,

progressive glomerulonephritis)

• Beckwith-Wiedemann syndrome

(hemihypertrophy).

Wilms Tumor(Nephroblastoma)

• Arises from mesodermal precursors of the

renal parenchyma (metanephric Blastema).

Wilms Tumor(Nephroblastoma)

Gross Pathology:

• Large solid spherical mass with areas of

hemorrhage and necrosis surrounded with

pseudocapsule.

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.

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.

Wilms Tumor(Nephroblastoma)

Radiological Picture

Wilms Tumor(Nephroblastoma)

Plain X-Ray:

Large soft tissue mass

displacing bowel gas

Wilms Tumor(Nephroblastoma)

IVU:

Large soft tissue mass displacing

bowel gas and distorting and

displacing the collecting system.

Wilms Tumor(Nephroblastoma)

IVU:

Large soft tissue mass displacing

bowel gas and distorting and

displacing the collecting system.

Wilms Tumor(Nephroblastoma)

US:

• Large soft tissue mass

heterogeneous echogenicity, which

represents hemorrhage, necrosis,

or calcification.

• Vascular Invasion.

Wilms Tumor(Nephroblastoma)

CT:

CT demonstrates a well-defined

heterogeneous mass with areas of

calcification, necrosis and

hemorrhage.

Wilms Tumor(Nephroblastoma)

CT:

CT demonstrates a well-defined

heterogeneous mass with areas of

calcification, necrosis and

hemorrhage.

Wilms Tumor(Nephroblastoma)

CT:

CT demonstrates a well-defined

heterogeneous mass with areas of

calcification, necrosis and

hemorrhage.

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.

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.

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.

Wilms Tumor(Nephroblastoma)

MRI:

Well-defined heterogeneous mass with low signal intensity

on T1-weighted images and high signal intensity on T2-

weighted images.

Wilms Tumor(Nephroblastoma)

Angiography: