Diseases and Conditions: Wilms tumor, pediatric

Wilms tumor, pediatric

Revised: April 5, 2019

Overview
Rare cancer involving the kidneys
Possibly affecting one or both kidneys
Also called nephroblastoma

Pathophysiology
Abnormal renal development leads to altered cellular function responsible for differentiation of the tubules
and glomerulus.
Several tumor suppressor genes appear to be involved, but the exact mechanism of how they play a role in
the development of Wilms tumor is unknown.

Causes
Unknown, possibly due to a genetic mutation

Incidence
It is the most common renal malignancy in children less than 15 years of age. Approximately 500 new
cases are diagnosed each year.
About two-thirds of the cases in the United States are diagnosed before age 5, with most cases diagnosed
before age 10.
African-Americans are at greater risk; there is a lower risk in Asian populations.
In some children, Wilms tumor occurs with other congenital syndromes.

Complications
Metastasis
Impaired renal function
Heart failure (due to chemotherapy)
Bowel obstruction (due to chemotherapy)
Pulmonary dysfunction (due to radiation)
Liver damage (due to chemotherapy)
Secondary malignancies
Hemorrhage
Wound infection
Recurrence of tumor (relapse)

Assessment

History
No signs or symptoms are common
Abdominal pain
Flank pain
Fever
Anorexia
Constipation
Nausea
Hematuria

Physical Findings
 Palpable or visible abdominal mass, frequently found initially during routine care of child
Abdominal asymmetry
Hypertension
Adventitious breath sounds (if metastasis)
Lymphadenopathy
Cryptorchidism and hypospadias may be found in children with associated syndromes

WARNING!

Abdominal palpation must be done carefully because the tumor is highly vascular and soft
and can easily rupture with excessive handling. When you've completed the initial palpation
and Wilms tumor is suspected, avoid further palpation.

Diagnostic Test Results

Laboratory
Urinalysis may show hematuria or leukocytes.
Liver function tests may be abnormal with metastasis.
Creatinine level (serum) is performed to check for reduced glomerular filtration rate.
Coagulations studies are done before surgery.
Chromosome studies may reveal a genetic abnormality.

Imaging
Ultrasonography (kidney and perirenal structures) and ultrasonography of the abdomen identifies the
tumor.
Computed tomography or magnetic resonance imaging of the abdomen and chest identify the tumor and
possible metastasis.

Treatment

General
Supportive care
Radiation therapy (typically for stages III and IV disease)
Surgical tumor removal

Diet
Well-balanced diet

Activity
As tolerated

Medications
Chemotherapeutic protocols with such agents as vincristine sulfate, dactinomycin doxorubicin
hydrochloride, cyclophosphamide, and etoposide phosphate

Surgery
Nephrectomy (simple, partial, or radical)
Central venous access device insertion
Nursing Considerations

Nursing Interventions
Avoid abdominal palpation.
Encourage verbalization of feelings and fears; allow time for the child and family to cope with the disorder
and the prognosis.
Assess level of pain utilizing an appropriate pain rating tool.
Provide comfort measures, as appropriate.
Provide adequate hydration and nutrition; offer a well-balanced diet with appropriate food choices and
snacks.
Obtain a daily weight.
Monitor fluid input and urine output.
Anticipate the need for insertion of a central venous access device for chemotherapy administration.
Prepare the child and family for insertion and provide central venous access site care, ensuring patency.
Institute safety and infection control precautions, as indicated.
Cluster nursing activities to ensure adequate rest periods, and allow time for uninterrupted rest. Gradually
increase the child's activity, as tolerated.
Provide care measures to address nausea, vomiting, hair loss, and other adverse reactions to
chemotherapeutic agents.
Obtain laboratory specimens to evaluate renal function preoperatively and postoperatively.
Prepare the child and family physically and psychologically for surgery, as appropriate. Explain preoperative
and postoperative procedures and equipment.
Provide postoperative care, including surgical site care, vital signs, renal and gastrointestinal functioning,
and pain management.

Monitoring
Vital signs
Renal function
Pain rating
Coping strategies
Adverse effects of treatment
Hydration and nutritional status
Daily weight
Surgical site (postoperatively)
Signs and symptoms of bleeding and infection (postoperatively)

Associated Nursing Procedures

Axillary temperature assessment, pediatric
Blood pressure assessment, pediatric
Cardiopulmonary status monitoring, pediatric
Central venous access catheter flushing and locking, pediatric
Central venous access catheter needleless connector change, pediatric
Central venous access catheter insertion, assisting, pediatric
Central venous access catheter removal, pediatric
Central venous access device declotting
Central venous access device dressing change
Chemotherapy administration, intravascular (IV), pediatric
IV bag preparation
IV pump use
IV secondary line drug infusion
IV administration set (tubing) change, pediatric
Oral drug administration, older child
Oral drug administration, toddler
Oral temperature assessment, pediatric
Preoperative care, pediatric
Pulse assessment, pediatric
Pulse oximetry, pediatric
 Respiratory assessment, pediatric
Venipuncture, pediatric

Patient and Family Teaching

General
Include the child's family or caregiver in your teaching, when appropriate. Be sure to cover:
disorder, diagnosis, and treatment, including protocols for chemotherapy administration and possible
radiation therapy (depending on the stage of the disease)
prescribed medications, including drug names, dosages, rationales for use, schedule of administration, and
frequency and duration of therapy
possible adverse effects of chemotherapeutic regimen
danger signs and symptoms to report to a practitioner
measures to minimize or prevent possible adverse effects of chemotherapy, such as the need to use a soft
toothbrush and to avoid hot spicy foods and commercial mouthwashes
signs and symptoms of infection
care of the surgical site, as indicated
importance of protecting the remaining kidney from injury, such as avoiding such sports as boxing and
hockey
signs and symptoms of abnormal bleeding
importance of planned rest periods during the day
positive coping strategies
infection control precautions and measures to reduce the risk of infection
recommended dietary plan
importance of adhering to recommended follow-up, including visits, screening for treatment-related
complications, and diagnostic testing, such as chest X-rays and abdominal ultrasounds every 6 to 8 weeks
during therapy, then every 3 months for 2 years, and then every 6 months for an additional 2 years.

Discharge Planning
Participate as part of a multidisciplinary team to coordinate discharge planning efforts. The team may
include a bedside nurse, care manager, nutritionist, oncologist, nephrologist, surgeon, and primary care
practitioner.
Assess the child's (if appropriate) and family or guardian's understanding of the diagnosis, treatment,
follow-up, and warning signs for which to seek medical attention.
Explain the importance of scheduling and attending all follow-up appointments.
Confirm arrangements for transportation to initial follow-ups.
Ensure that the parents understand the treatment regimen, including dosage, administration, expected
results, duration, and possible adverse effects of the prescribed medication.
Ensure that the parents or caregivers (or whoever has been designated as the responsible party) are able to
obtain medications.
Ensure that the child and caregivers have been given the proper medical contact information.
Assess the child's and family or guardian's understanding of teaching by using the teach-back method,
when possible.
Document the discharge planning evaluation in the patient's clinical record, including who was present and
involved in discharge planning and teaching and their understanding of the teaching provided.

Resources
American Academy of Pediatrics: www.aap.org
American Cancer Society: www.cancer.org
Guide to Internet Resources for Cancer: www.cancerindex.org
National Cancer Institute: www.cancer.gov
National Wilms Tumor Study: www.nwtsg.org
Selected References
1. Chintagumpala, M., & Muscal, J. A. Presentation, diagnosis, and staging of Wilms tumor. (2017). In: UpToDate,
Pappo, A. S. (Ed.).
2. Chintagumpala, M., & Muscal, J. A. Treatment and prognosis of Wilms tumor. (2018). In: UpToDate, Pappo, A.
S. (Ed.).
3. Dumba, M., et al. (2015). Neuroblastoma and nephroblastoma: A radiological review. Cancer Imaging, 15(1), 5.
4. Irtan, S., et al. (2016). Wilms tumor: “State-of-the-art” update, 2016. Pediatric Surgery, 25(5), 250-256.
5. Kieran, K., & Ehrlich, P. F. (2016). Current surgical standards of care in Wilms tumor. Urologic Oncology, 34(1),
13-23.
6. Millar, A. J. W., et al. (2017). Management of bilateral Wilms tumours. Pediatric Surgery International, 33(7),
737-745.
7. Paulino, A. C. (2016). “Wilms tumor” [Online]. Accessed March 2019 via the Web at
http://emedicine.medscape.com/article/989398-overview
8. Shrestha, A., et al. (2014). Prenatal exposure to air toxics and risk of Wilms' tumor in 0- to 5-year-old children.
Journal of Occupational & Environmental Medicine, 56(6), 573-578. (Level II).
9. Smith, N., & Ashley, T. J. (2018). Wilms' tumor. CINAHL Nursing Guide.