Wilms' Tumor

Wilms’ Tumor
Medical definition
Wilms' tumor or nephroblastoma is cancer of the kidneys that typically occurs

in children, rarely in adults. Its common name is an eponym, referring to Dr. Max Wilms, the
German surgeon (1867–1918) who first described this kind of tumor.
Risk Factors
Wilms' tumor occurs almost exclusively in young children. The average patient is about three
years old, although cases have also been reported in infants younger than six months and adults
in their early twenties. Females are only slightly more likely than males to develop Wilms'
tumor. In the United States, Wilms' tumor occurs in 8.3 individuals per million in white children
under the age of 15 years. The rate is higher among African-Americans and lower among Asian-
Americans. Wilms' tumors are found more commonly in patients with other types of congenital
conditions. These conditions include:
 absence of the colored part (the iris) of the eye (aniridia)
 enlargement of one arm, one leg, or half of the face (hemihypertrophy)
 certain birth defects of the urinary system or genitals
 certain genetic syndromes (WAGR syndrome, Denys-Drash syndrome, and Beckwith-
Wiedemann syndrome)
Diagnostic Exams
Children with Wilms' tumor generally first present to physicians with a swollen abdomen
or with an obvious abdominal mass. The physician may also find that the child has fever, bloody
urine, or abdominal pain. The physician will order a variety of tests before imaging is performed.
These tests mostly involve blood analysis in the form of a white blood cell count, complete blood
count, platelet count, and serum calcium evaluation. Liver and kidney function testing will also
be performed as well as a urinalysis.
Initial diagnosis of Wilms' tumor is made by looking at the tumor using various imaging
techniques:
- Ultrasound
- Computed Tomography scans (CT scans)
- Magnetic Resonance Imaging (MRI scans)
- Intravenous pyelography in which a dye injected into a vein helps show the structures
of the kidney, can also be used in diagnosing this type of tumor
Final diagnosis, however, depends on obtaining a tissue sample from the mass (biopsy)
and examining it under a microscope in order to verify that it has the characteristics of a Wilms'
tumor. This biopsy is usually done during surgery to remove or decrease the size of the tumor.
Other studies (chest x rays, CT scan of the lungs, bone marrow biopsy) may also be done in
order to see if the tumor has spread to other locations.
Therapeutic Modalities
Staging is determined by combination of imaging studies and pathology findings if the tumor is
operable (adapted from www.cancer.gov). Treatment strategy is determined by the stage:
Stage I (43% of patients)

For stage I Wilms' tumor, 1 or more of the following criteria must be met:
 Tumor is limited to the kidney and is completely excised.
 The surface of the renal capsule is intact.
 The tumor is not ruptured or biopsied (open or needle) prior to removal.
 No involvement of renal sinus vessels.
 No residual tumor apparent beyond the margins of excision.
Treatment: Nephrectomy +/- 18 weeks of chemotherapy depending on age of patient and weight
of tumor. EG: less than 2 years old and less than 550g only requires Nephrectomy with
observation
Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic
Stage II (23% of patients)

For Stage II Wilms' tumor, 1 or more of the following criteria must be met:
 Tumor extends beyond the kidney but is completely excised.
 No residual tumor apparent at or beyond the margins of excision.
 Any of the following conditions may also exist:
 Tumor involvement of the blood vessels of the renal sinus and/or outside the renal
parenchyma.
 The tumor has been biopsied prior to removal or there is local spillage of tumor
during surgery, confined to the flank.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy
Stage III (23% of patients)

For Stage III Wilms' tumor, 1 or more of the following criteria must be met:
 Unresectable primary tumor.
 Lymph node metastasis.
 Positive surgical margins.
 Tumor spillage involving peritoneal surfaces either before or during surgery, or
transected tumor thrombus.
Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor
shrinkage
Stage IV (10% of patients)

Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone,
or brain), or lymph node metastases outside the abdomenopelvic region.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of
metastatic site as appropriate
Stage V (5% of patients)
Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis.
Note: For patients with bilateral involvement, an attempt should be made to stage each side
according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy.
The 4-year survival was 94% for those patients whose most advanced lesion was stage I or stage
II; 76% for those whose most advanced lesion was stage III.
Treatment: Individualized therapy based on tumor burden
Stage I-IV Anaplasia

Children with stage I anaplastic tumors have an excellent prognosis (80-90% five-year
survival). They can be managed with the same regimen given to stage I favorable histology
patients.
Children with stage II through stage IV diffuse anaplasia, however, represent a higher-risk
group. These tumors are more resistant to the chemotherapy traditionally used in children with
Wilms’ tumor (favorable histology), and require more aggressive regimens.
Chemotherapy used
Information about the tumor cell type and the spread of the tumor is used to decide the
best kind of treatment for a particular patient. Treatment is usually a combination of surgery,
medications used to kill cancer cells (chemotherapy), and x rays or other high-energy rays used
to kill cancer cells (radiation therapy). These therapies are called adjuvant therapies, and this
type of combination therapy has been shown to substantially improve outcome in patients with
Wilms' tumor. It has long been known that Wilms' tumors respond to radiation therapy.
Likewise, some types of chemotherapy have been found to be effective in treating Wilms' tumor.
Three Prioritized Nursing Diagnosis
- Acute Pain
- Constipation
- Risk for hemorrhage
Pathology/physiology
Hyperplastic
Incipient or Dormant Regressing Wilms’ Tumor
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Wilms' Tumor

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Wilms’ Tumor

Wilms' tumor or nephroblastoma is cancer of the kidneys that typically occurs

Stage I (43% of patients)

Stage II (23% of patients)

Stage III (23% of patients)

Stage IV (10% of patients)

Stage I-IV Anaplasia

Three Prioritized Nursing Diagnosis

Incipient or Dormant Regressing Wilms’ Tumor

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