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Wilms Tumor (Nephroblastoma)


Nina, a 4-year old, complained of abdominal pain to her mother. As Nina’a mother touched her abdomen, she was alarmed to have felt a small protrusion inside. Nina was brought to the nearest pediatric clinic for an examination, and after several tests and imaging scans, Nina was diagnosed with Wilms tumor.

Description


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.
  • 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.

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.
  • 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:

  • 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

Main Article: 4 Wilms Tumor (Nephroblastoma) Nursing Care Plans

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.

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.

Practice Quiz: Wilms Tumor (Nephroblastoma)


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1. A child is admitted to the hospital with a diagnosis of Wilms tumor, stage II. Which of the following statements most accurately describes this stage?

A. The tumor is less than 3 cm. in size and requires no chemotherapy.
B. The tumor did not extend beyond the kidney and was completely resected.
C. The tumor extended beyond the kidney but was completely resected.
D. The tumor has spread into the abdominal cavity and cannot be resected.

1. Answer: C. The tumor extended beyond the kidney but was completely resected.

  • Option C: The staging of Wilms tumor is confirmed at surgery as stage II, the tumor extends beyond the kidney but is completely resected.
  • Options A, B, D: Are incorrect because they belong to the different stages of Wilms tumor.

2. A child is diagnosed with Wilms’ tumor. During assessment, the nurse in charge expects to detect:

A. Gross hematuria.
B. Dysuria.
C. Nausea and vomiting.
D. An abdominal mass.

2. Answer: D. An abdominal mass.

  • Option D: The most common sign of Wilms’ tumor is a painless, palpable abdominal mass, sometimes accompanied by an increase in abdominal girth.
  • Option A: Gross hematuria is uncommon, although microscopic hematuria may be present.
  • Option B: Dysuria is not associated with Wilms’ tumor.
  • Option C: Nausea and vomiting are rare in children with Wilms’ tumor.

3. Which of the following would be inappropriate when administering chemotherapy to a child?

A. Monitoring the child for both general and specific adverse effects.
B. Observing the child for 10 minutes to note for signs of anaphylaxis.
C. Administering medication through a free-flowing intravenous line.
D. Assessing for signs of infusion infiltration and irritation.

3. Answer: B. Observing the child for 10 minutes to note for signs of anaphylaxis.

  • Option B: When administering chemotherapy, the nurse should observe for an anaphylactic reaction for 20 minutes and stop the medication if one is suspected.
  • Options A, C, D: Chemotherapy is associated with both general and specific adverse effects, therefore close monitoring for them is important.

4. David, age 15 months, is recovering from surgery to remove Wilms’ tumor. Which findings best indicates that the child is free from pain?

A. Decreased appetite.
B. Increased heart rate.
C. Decreased urine output.
D. Increased interest in play.

4. Answer D. Increased interest in play.

  • Option D: One of the most valuable clues to pain is a behavior change: A child whose pain-free likes to play.
  • Option A: A child in pain is less likely to consume food or fluids.
  • Option B: An increased heart rate may indicate increased pain.
  • Option C: An increased heart rate may indicate increased pain; decreased urine output may signify dehydration.

5. Which of the following is the best method for performing a physical examination on a toddler?

A. From head to toe.
B. Distally to proximally.
C. From abdomen to toes, the to head.
D. From least to most intrusive.

5. Answer: D. From least to most intrusive.

  • Option D: When examining a toddler or any small child, the best way to perform the exam is from least to most intrusive.
  • Option A: Starting at the head or abdomen is intrusive and should be avoided.
  • Option B: Proceeding from distal to proximal is inappropriate at any age.
  • Option C: Starting from the abdomen is very incorrect, especially in children with Wilms tumor.

See Also


Related topics to this study guide:

Further Reading


Recommended resources and books for pediatric nursing:
  1. PedsNotes: Nurse's Clinical Pocket Guide (Nurse's Clinical Pocket Guides)
  2. Pediatric Nursing Made Incredibly Easy
  3. Wong's Essentials of Pediatric Nursing
  4. Pediatric Nursing: The Critical Components of Nursing Care

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