WILMS TUMOR, OR NEPHROBLASTOMA

is the most common childhood abdominal malignancy.

• Wilms tumor is an adenosarcoma in the kidney region.

• The tumor arises from bits of embryonic tissue that remain after birth.
• This tissue can spark rapid cancerous growth in the area of the kidney.

Stages
The Children’s Oncology Group has identified the staging of Wilms tumor as:

• Stage 1. The tumor is limited to kidney and is completely resected; the renal
capsule is intact; the tumor was not ruptured or biopsied prior to removal; the
vessels of the renal sinus are not involved, and no evidence of tumor is present
at or beyond the margins of resection.
• Stage 2. The tumor is completely resected; no evidence of tumor at or beyond
the margins of resection is noted; and the tumor extends beyond the kidney
(penetration of renal capsule, involvement of renal sinus).
• Stage 3. A residual, nonhematogenous tumor is present following surgery and is
confined to the abdomen; positive lymph nodes in the abdomen or pelvis are
noted; penetration through the peritoneal surface is observed; peritoneal implants
are present; gross or microscopic tumor remains postoperatively, including
positive margins of resection; tumor spillage is noted, including biopsy; the tumor
is treated with preoperative chemotherapy; and the tumor is removed in more
than 1 piece.
• Stage 4. Hematogenous metastases (eg, lung, liver, bone, brain) or lymph node
metastases beyond the abdomen or pelvis are noted.
• Stage 5. Bilateral renal involvement by the tumor is present at diagnosis.

Pathophysiology

Wilms tumor is a malignant mixed tumor containing metanephric blastoma, stromal, and
epithelial derivatives.

• There are mutations of WT1 gene on chromosome 11 and nephroblastematosis

(persistence of renal blastema in kidney tissue.
• The tumor consists of tumor epithelial component (abortive tubules and glomeruli)
surrounded by metanephric blastema and tumor immature spindle cell stroma.

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 • The stroma may include differentiated (muscle, cartilage, bone, fat tissue, fibrous
tissue) or anaplastic elements.
• The tumor compresses the normal kidney parenchyma.

Statistics and Incidences

The occurrence of Wilms tumor in the United States and around the world are as follows:

• Wilms tumor affects approximately 10 children and adolescents per 1 million

before age 15 years.
• Therefore, it accounts for 6-7% of all childhood cancers in North America.
• As a result, about 450-500 new cases are diagnosed each year on this continent.
• Wilms tumor appears to be relatively more common in Africa and least common
in East Asia.
• Wilms tumor is relatively more common in blacks than in whites and is rare in East
Asians.
• Estimates suggest 6-9 cases per million person-years in whites, 3-4 cases per
million person-years in East Asians and more than 10 cases per million person-
years among black populations.
• Among patients with unilateral Wilms tumor enrolled in all NWTSG protocols, the
male-to-female ratio was 0.92:1.

Causes
Wilms tumor is thought to be caused by alterations of genes responsible for normal
genitourinary development.

• WT1 gene. WT1, the first Wilms tumor suppressor gene at chromosomal band
11p13, was identified as a direct result of the study of children with Wilms tumor
who also had aniridia, genitourinary anomalies, and mental retardation (WAGR
syndrome).
• Additional genetic loci. A second gene that predisposes individuals to develop
the Wilms tumor has been identified (but has not yet been cloned) telomeric of
WT1, at 11p15; this locus was proposed on the basis of studies in patients with
both Wilms tumor and Beckwith-Wiedemann syndrome (BWS), another congenital
Wilms-tumor predisposition syndrome linked to chromosomal band 11p15.

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Clinical Manifestations
The child with Wilms tumor exhibits the following:

• Abdominal mass. The most common manifestation of Wilms tumor is an

asymptomatic abdominal mass; an abdominal mass occurs in 80% of children at
presentation.
• Abdominal pain. Abdominal pain or hematuria occurs in 25%.
• Tumor hemorrhage. A few patients with hemorrhage into their tumor may
present with hypotension, anemia, and fever.

Assessment and Diagnostic Findings

The following studies are indicated in patients with Wilms tumor:

• Laboratory studies. Complete blood count, chemistry profile, urinalysis,

coagulation studies, and cytogenic studies are made to determine Wilms tumor;
results may reveal an 11p13 deletion, as in WAGR syndrome, or a duplication of
the paternal allele 11p15, as in Beckwith-Wiedemann syndrome (BWS).
• Renal ultrasonography. Renal ultrasonography is often the initial study because
it does not expose children to the detrimental effects of radiation.
• CT scanning. Abdominal computed tomography (CT) scanning helps in
determining the origin of the tumor, involvement of the lymph nodes, bilateral
kidney involvement, invasion into major vessels (eg, inferior vena cava), and liver
metastases.
• MRI scanning. Abdominal magnetic resonance imaging (MRI) is reportedly the
most sensitive imaging modality for determination of caval patency and may be
important in determining whether the inferior vena cava is directly invaded by the
tumor.

Medical Management
Treatment consists of surgical removal as soon as possible, after the growth is discovered,
combined with radiation and chemotherapy.

• Focal anaplastic stage I-III Wilms tumors and diffuse anaplastic stage I
Wilms tumors. Nephrectomy followed by vincristine, actinomycin-D,
and doxorubicin in addition to local radiotherapy.

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 • Focal anaplastic stage IV Wilms tumors and diffuse anaplastic stage II-
III tumors. Patients undergo the same treatment, with the addition
of cyclophosphamide, etoposide, and carboplatin.
• Stage IV diffuse anaplastic Wilms tumors. More aggressive treatment is
delivered; nephrectomy is followed by initial irinotecan and vincristine
administration, which in turn is followed by actinomycin-D, doxorubicin,
cyclophosphamide, carboplatin, etoposide, and radiotherapy.
• Activity.No precautions regarding activity are advised, although the patient and
his or her parents should be aware that the patient will have only 1 kidney after
therapy; activities that carry an inherent risk of kidney injuries, such as boxing
and hockey, should be avoided.

Pharmacologic Management
As previously stated, several cytotoxic agents may cause liver damage in patients treated
for Wilms tumor.

• Antineoplastics. These chemotherapeutic agents used to treat patients with

Wilms tumor depend on the stage and histology of disease; commonly used agents
include dactinomycin, vincristine, doxorubicin, cyclophosphamide, etoposide, and
carboplatin; the dosage depends on the particular stage of the disease and on the
child.

Nursing Management
Nursing management of a child with Wilms tumor include the following:

Nursing Assessment
Assessment of the child with Wilms tumor include:

• Assess for bleeding. Assess for bleeding from any site and febrile episodes;
Monitor WBC, platelet count, hematocrit, absolute neutrophil count.
• Assess the oral cavity. Assess oral cavity for pain ulcers, lesions, gingivitis,
mucositis or stomatitis and effect on the ability to ingest food and fluids.
• Assess for anxiety. Assess source and level of anxiety and need for information
and support that will relieve it.

Nursing Diagnoses
Based on the assessment data, the major nursing diagnoses are:

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 • Ineffective protection related to antineoplastic agents, radiation therapy, or
leukopenia.
• Impaired oral mucous membrane related to chemotherapy.
• Anxiety related to change in health status and threat of death.
• Risk for injury related to side effects of medications and complications.

Nursing Care Planning and Goals

The major nursing care planning goals for a child with Wilms tumor are:

• Child will be protected from illness or injury.

• Child will be free of oral mucous membrane irritation.
• Clients will experience decreased anxiety.
• Child will not experience injury.

Nursing Interventions
Nursing interventions appropriate for the child are:

• Prevent transfer of microorganism. Perform handwashing prior

giving care, utilize mask and gown when needed, provide a private room, monitor
for any signs and symptoms of infection.
• Prevent oral trauma. Instruct the use of a soft-sponge toothbrush or sponge
toothette or gauze when rinsing the mouth; instruct to avoid foods which are hot,
spicy, or high in ascorbic acid (vitamin C); provide oral hygiene 30 minutes prior
or after meals; instruct to refrain from eating or drinking for 30 minutes after
completion of oral hygiene; and offer moist, soft, bland foods.
• Prevent anxiety. Have the parents to stay with the child or encourage open
visitation, provide a telephone number to call for information; explain all
procedures and care in simple, direct, honest terms and repeat as often as
necessary; reinforce physician information if needed and provide specific
information as needed; and provide consistent nurse assignment with the same
personnel; encourage parents to participate in care.
• Prevent injury. Avoid any palpation of abdominal mass; post sign on bed stating
not to palpate preoperatively; assess incision site for redness, swelling, drainage,
intactness, and healing and change dressing when soiled or wet; assess oral
and perineal area; and encourage parents to appropriately dress child based on
weather conditions and to refrain from participating on rough activities or sports.

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Evaluation
Goals are met as evidenced by:

• Child is protected from illness or injury.

• Child is free of oral mucous membrane irritation.
• Clients experienced decreased anxiety.
• Child did not experience injury.

Documentation Guidelines
Documentation in a child with Wilms tumor include:

• Cultural and religious beliefs, and expectations.

• Plan of care.
• Teaching plan.
• Responses to interventions, teaching, and actions performed.
• Postoperative care.
• Modifications to the plan of care.
• Attainment or progress toward desired outcomes.