WILMS TUMOR

• Most common renal malignancy in children <15 years old

• Two-thirds cases are diagnosed before 5 years of age and 95% before 10 years of age.

• Children with bilateral disease are diagnosed at an earlier age

• In 10% cases, part of a multiple malformation syndrome

• WAGR syndrome refers to Wilms tumor, aniridia, genitourinary (GU) anomalies, and intellectual disability
(mental retardation).

• The Denys-Drash syndrome is a triad of progressive renal disease, male pseudohermaphroditism, and
Wilms tumor.

• BWS include macrosomia, macroglossia, omphalocele, prominent eyes, ear creases, large kidneys,
pancreatic hyperplasia, and hemihypertrophy.
PATHOLOGY

• The tumor is typically surrounded by a pseudocapsule, which may help distinguish it from other renal
tumors, which have an infiltrative border.

• Histologically, the classic favorable histology Wilms tumor is comprised of three cell types:
• Blastemal cells – Undifferentiated cells
• Stromal cells – Immature spindle cells and heterologous skeletal muscle, cartilage, osteoid, or fat
• Epithelial cells – Glomeruli and tubules
• Tumor histology is linked to patient outcome. Anaplasia is associated with poor outcome.

• Blastemal type histology, associated with poor outcome.

CLINICAL PRESENTATION
• Most children present with an abdominal mass or swelling, without other
signs or symptoms.

• Other symptoms include abdominal pain (30 to 40%), hematuria (12 to

25%), fever, and hypertension (25%).

• Patients with sub capsular hemorrhage can present with rapid abdominal
enlargement, anemia, hypertension, and sometimes fever.
• Although the lung is the most common metastatic site, children rarely
present with respiratory symptoms.

• Physical examination reveals a firm, nontender, smooth mass that is

eccentrically located and rarely crosses the midline.

• Examination should include assessment for associated anomalies, such as

aniridia, hemihypertrophy, and genitourinary anomalies.
DIAGNOSIS

• Abdominal USG is typically the initial study performed for evaluation of

abdominal mass.

• In patients with a suspected renal tumor, Doppler can be performed to

detect tumor infiltration of the renal vein and IVC and to assess patency of
blood flow.

• CT or MRI is recommended to further evaluate the nature and extent of the

mass, including evidence of preoperative rupture or ascites.
• CT & MRI have similar ability to detect lymph node metastasis and
capsular penetration.

• Either modality can be used for initial locoregional staging of pediatric

renal tumors.
• Patients with any of the following findings or circumstances generally
undergo an upfront biopsy and receive prenephrectomy
chemotherapy rather than initial primary nephrectomy:

1. Tumor thrombus above the level of the hepatic veins

2. Pulmonary compromise from massive tumor or extensive
pulmonary metastases
3. Resection requiring removal of contiguous structures (other than
adrenal gland)
4. Surgeon judges that attempting nephrectomy would result in
significant morbidity, tumor spill, or residual tumor
• Imaging of the chest is needed to determine whether there are lung
metastases.
Staging
• There are two major systems currently in use:

• National Wilms Tumor Study (NWTS) – The NWTS system is based upon surgical
evaluation prior to the administration of chemotherapy. It is used throughout the United
States and Canada.

• International Society of Pediatric Oncology (SIOP) – The SIOP system is based upon
post-chemotherapy surgical evaluation and is used extensively in Europe.
Treatment (NWTS)
• Stages I and II – Primary surgical resection followed by 19 weeks
chemotherapy. No radiation therapy required.

• Stage III – Primary surgical resection is followed by 25 weeks of three-

drug chemotherapy.

• Radiation therapy based on lymph node involvement or the extent of

the peritoneal contamination.
• Stage IV- Primary surgical resection is followed by 25 weeks of triple
drug chemotherapy.

• Radiation therapy is administered to the whole lung field only if lung

metastases do not completely resolve after six weeks of
chemotherapy.
• In patients with evidence of spread of disease to the regional lymph
nodes radiation therapy is administered to the loco-regional area
including the appropriate one-half of the abdomen

• For patients with metastatic sites other than the lung, radiation dose
varies according to the site.
Loss of heterozygosity (LOH) at Chr 1p, Very low risk tumors:
11p15, and 16q and 1q gain in tumor -Age <2 years
cells -Stage I favorable histology
-Tumor weighs <550 g