Askep pada anak dengan Wilms

Tumor
By :Alfonsa Reni Oktavia
 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.
 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.
 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.
 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).
 Stages

• 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.
 Clinical Manifestations
The child with Wilms tumor exhibits the following:

1. Abdominal mass. The most common

manifestation of Wilms tumor is an
asymptomatic abdominal mass; an abdominal
mass occurs in 80% of children at presentation.
2. Abdominal pain. Abdominal pain or hematuria
occurs in 25%.
3. Tumor hemorrhage. A few patients with
hemorrhage into their tumor may present with
hypotension, anemia, and fever.
 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.
Pathophysiology
 Assessment and Diagnostic Findings
1. 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).
2. Renal ultrasonography. Renal ultrasonography is often the initial study
because it does not expose children to the detrimental effects of
radiation.
3. 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.
4. 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
1. 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.
2. 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.
3. 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.
4. 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.

1. 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:

1. Assess for bleeding. Assess for bleeding from any site and febrile
episodes; Monitor WBC, platelet count, hematocrit, absolute
neutrophil count.
2. 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.
3. 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:

1. Ineffective protection related to antineoplastic

agents, radiation therapy, or leukopenia.
2. Impaired oral mucous membrane related to
chemotherapy.
3. Anxiety related to change in health status and threat
of death.
4. 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:

1. Child will be protected from illness or injury.

2. Child will be free of oral mucous membrane
irritation.
3. Clients will experience decreased anxiety.
4. Child will not experience injury.
 Nursing Interventions
Nursing interventions appropriate for the child are:

1. 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.
2. 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.
 Nursing Interventions
Nursing interventions appropriate for the child are:
3. 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.
4. 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:

1. Child is protected from illness or injury.

2. Child is free of oral mucous membrane
irritation.
3. Clients experienced decreased anxiety.
4. Child did not experience injury.
 Documentation Guidelines
Documentation in a child with Wilms tumor
include:

1. Cultural and religious beliefs, and expectations.

2. Plan of care.
3. Teaching plan.
4. Responses to interventions, teaching, and
actions performed.
5. Postoperative care.
6. Modifications to the plan of care.
7. Attainment or progress toward desired
outcomes.