Wilms tumor (also called Wilms' tumor or nephroblastoma) is a type of childhood cancer that starts in the kidneys. It is the most common type of kidney cancer in children. About 9 of 10 kidney cancers in children are Wilms tumors.
2. INTRODUCTION
Wilms' tumor is a malignant renal tumor and is the
most common renal neoplasm in children.
It constitutes approximately 6% of all childhood
tumors.
Wilms' tumor, also known as nephroblastoma,
It is named after Max Wilms, the German
surgeon (1867–1918) who first described it.
3. INCIDENCE
Incidence is 7.6 cases per million for children
younger than age 15.
Seventy-five percent of cases occur before the child
is age 5.
Most commonly a unilateral disease, but in 5% to
10%, both kidneys are involved
4. CAUSES
It's not clear what causes Wilms' tumor, but in
rare cases, heredity may play a role.
Cancer begins when cells develop errors in their
DNA.
The errors allow the cells to grow and divide
uncontrollably and to go on living when other
cells would die.
The accumulating cells form a tumor. In Wilms'
tumor, this process occurs in the kidney cells.
5. RISK FACTORS
Factors that may increase the risk of Wilms' tumor include:
African-American race. In the United States, African-
American children have a slightly higher risk of
developing Wilms' tumor than children of other races.
Asian-American children appear to have a lower risk than
children of other races.
Family history of Wilms' tumor. If someone in child's
family has had Wilms' tumor, then child has an increased
risk of developing the disease.
6. Conti..
Wilms' tumor occurs more frequently
in children with certain
abnormalities or syndromes
present at birth, including:
Aniridia. In aniridia the iris — the
colored portion of the eye —
forms only partially or not at all.
Hemihypertrophy. Hemihypertro
phy means one side of the body or
a part of the body is noticeably
larger than the other side.
Aniridia
7. Wilms' tumor can occur as part of rare
syndromes, including:
WAGR syndrome. This syndrome includes
Wilms' tumor, aniridia, genital and urinary system
abnormalities, and intellectual disabilities.
Denys-Drash syndrome. This syndrome includes
Wilms' tumor, kidney disease and male
pseudohermaphroditism, in which a boy is born
with testicles but may exhibit female
characteristics.
8. Conti..
Beckwith-Wiedemann syndrome.
Children with this syndrome tend to be significantly larger
than average (macrosomia).
Other signs may include abdominal organs that jut out into
the base of the umbilical cord, a large tongue
(macroglossia), enlarged internal organs and ear
abnormalities.
There is also an increased risk of tumors, including a type
of liver cancer called hepatoblastoma
9. Types of Wilms Tumors
There are two kinds of Wilms tumors, divided by
how the cells look under a microscope.
TYPES
Favorable
histology
Unfavorable
or anaplastic
histology
10. CONTI..
Favorable histology. More than 9 out of 10
Wilms tumors fall into this group. It means there
isn’t a lot of difference among the cancer cells.
Children with this type have a good chance of
being cured.
Unfavorable or anaplastic histology. This type
has a variety of deformed cancer cells. It can be
much harder to cure.
11. 5 Stages of wilm’s tumor
• Tumor confined to kidney and completely
excised.
STAGE I
• Tumor extends beyond kidney but
completely excised.
STAGE II
• Tumor infiltrates renal fat; residual tumor
after surgery. Lymph node involvement of
hilum, para-aortic region or beyond.
STAGE III
12. Conti..
• Metastasis in lung or
liver, rarely bone and
brain..
STAGE IV
• Bilateral renal involvement at
time of initial DIAGNOSIS
STAGE V
13. Clinical Manifestation
A firm, nontender upper quadrant abdominal mass is
usually the presenting sign; it may be on either side.
(It is usually observed by the parents.)
Abdominal pain, which is related to rapid growth of
the tumor, may occur.
As the tumor enlarges, pressure may cause
constipation, vomiting, abdominal distress, anorexia,
weight loss, and dyspnea.
14. Conti..
Less common are hypertension, fever, hematuria,
and anemia.
Associated anomalies.
– Hemihypertrophy
– Aniridia (without the iris)
– GU anomalies
– Overgrowth syndromes (ie, Beckwith-Wiedemann
syndrome)
15. Diagnostic evaluation
Abdominal ultrasound- to show the tumor and
to assess the status of the opposite kidney.
Radiography of chest to identify metastases.
CBC and peripheral smear - for baseline data.
Urinalysis for hematuria.
16. Conti..
Blood chemistries, especially serum electrolytes,
uric acid, renal function tests (blood urea nitrogen
and creatinine), and liver function tests (bilirubin,
alanine aminotransferase, aspartate
aminotransferase, lactate dehydrogenase [LD], total
protein, albumin, and alkaline phosphatase) may
show abnormalities
17. Conti..
Urinary VMA and HVA to distinguish from
neuroblastoma.
MRI or CT scan of the abdomen to evaluate local
spread to lymph nodes or adjacent organs.
Real-time ultrasonography.
The Vanillylmandelic (VMA) test is
primarilly used to detect and rule
out tumors called neuroblastoma in
children with an abdominal mass or
other symptoms suggestive of the
disease.
Homovanillic acid (HVA)
measurement in urine is used
for the screening children for
catecholamine-secreating
tumors such as neuroblastoma
18. Management
Surgery
If doctor operates, they may do one of these
procedures:
Partial nephrectomy. This removes the
tumor and some healthy tissue around it.
Radical nephrectomy. This removes the
affected kidney, the ureter, the adrenal gland
on top of the kidney, and nearby tissue.
19. Conti..
Removal of both kidneys. In some cases,
the doctor will need to take out both kidneys.
Child would then need to have dialysis, using
a machine to filter waste out of their blood.
Once they’re healthy enough, they might
have a kidney transplant.
20. Chemotherapy
Certain medications can fight or kill cancer cells
inside child’s body.
Most children who have Wilms tumors will get chemo
at some point during treatment.
These drugs can also affect healthy cells, leading to
side effects including:
Hair loss
Fatigue
Mouth sores
21. Conti..
Loss of appetite
Nausea and vomiting
Diarrhea or constipation
Bruising or bleeding easier than usual
Higher chances of infection
22. Radiation
Strong radiation can also kill cancer cells. A
machine focuses it onto the cancer.
Doctors tend to use radiation for tumors that
are stage III and above. But it can also have
short-term and long-term side effects,
including tissue damage, so they’ll use as
little as possible.
23. COMPLICATIONS
Metastasis to the lungs, lymph nodes, liver, bone, and
brain.
Complications from radiation therapy include bowel
obstruction, hepatic damage, nephritis, sterility in girls,
interstitial pneumonia, scoliosis.
Cumulative incidence of second malignancy is 1.6%
after 15 years with radiation being the greatest risk
factor
24. NURSING ASSESSMENT
Obtain a history.
– Ask how tumor was first discovered.
– Ask whether the child has history of other GU
anomalies or whether there is a family history of
cancer.
– Determine whether the child has had hematuria,
dysuria, constipation, abdominal pain, decreased
appetite, or fever before hospitalization and ask
how these were treated.
25. Perform a physical examination
that includes:
– Assessment for associated anomalies: aniridia,
hemihypertrophy of the spine, or cryptorchidism.
– Palpation of lymph nodes for enlargement,
tenderness, and mobility.
– Palpation of the liver and spleen for enlargement.
26. Conti..
– Palpation of the abdomen to determine the size
and location of the tumor.
– Auscultation of the lungs to assess breath sounds
or abnormality because of spread of tumor.
Assess coping, resources, and emotional
state of the family.
27. NURSING ALERT
Avoid indiscriminate manipulation of
the abdomen preoperatively and
postoperatively to decrease the
danger of metastasis. Because the
tumor is soft and highly vascular,
seeding may occur with excessive
28. NURSING DIAGNOSIS
Anxiety of parents related to learning of
diagnosis
Anxiety of child related to surgery and
diagnostic tests
Risk for Deficient Fluid Volume
postoperatively
Acute Pain related to surgery and possible
progression of the disease
29. Conti..
Imbalanced Nutrition: Less Than Body Requirements
related to anemia, anorexia, nausea, vomiting, and
mucosal ulceration secondary to chemotherapy or
radiation
Disturbed Body Image related to alopecia associated
with chemotherapy
Activity Intolerance related to fatigue that results
from the size of the tumor and treatment
Risk for Infection and hemorrhage related to bone
marrow suppression caused by chemotherapy
30. Nursing Interventions
Be available to the parents when they want
to discuss their feelings.
Offer kindness, concern, consideration, and
sincerity toward the child and parents; be a
source of consolation.
Contact the family's clergyman or the
hospital chaplain
Goal - Reducing Parents' Anxiety
31. Conti..
Obtain the services of a social worker, as
appropriate, to help the family use
appropriate community resources.
Offer hope that therapy will be effective and
will prolong life.
Have parents speak with parents of a child
currently on therapy
32. Conti..
Encourage parents to participate in activities
of daily living to help them feel a part of their
child's care.
Assess family dynamics and coping
mechanisms and plan interventions
accordingly.
Help the parents to deal with anticipatory
grief.
33. Conti..
Help the parents to deal with other family members,
especially siblings and grandparents, and friends.
Encourage the parents to discuss concerns about
limiting their child's activities, protecting child from
infection, disciplining child, and having anxieties
about the illness.
Facilitate communication with the clinic nurse or
clinical specialist who may interact with the child
during the entire course of illness
34. Goal-Reducing Child's Anxiety
Provide for continuity of care.
Encourage family-centered care (see page
1382).
Facilitate play activities for the child and use
opportunities to communicate through play.
Maintain some discipline, placing calm
limitations on unacceptable behavior.
35. Conti…
Provide appropriate diversional activities.
Encourage independence and provide
opportunities that allow the child to control
his environment.
Explain the diagnosis and treatment in age-
appropriate terms.
36. Goal- Preventing Fluid Volume
Deficit and Other Complications
Insert a nasogastric tube as ordered. Many
children require gastric suction
postoperatively to prevent distention or
vomiting.
Monitor gastric output accurately and replace
it with the appropriate I.V. fluids as ordered.
When bowel sounds have returned, begin
with small amounts of clear fluids.
37. Conti..
Keep accurate intake and output record.
Monitor vital signs as the child's condition
warrants and check the surgical dressing
frequently for drainage.
38. Goal- Controlling Pain
Position the child for comfort. Water beds
and bean bag chairs are usually helpful.
Administer drugs on a preventive schedule
before pain becomes intense. Continuous
infusion pumps for opioid administration are
used.
Manipulate the environment as necessary to
increase the child's comfort and to minimize
unnecessary exertion.
39. Conti..
Prepare the child for treatment and
diagnostic procedures.
– Use knowledge of growth and development to
prepare the child for such procedures as bone
marrow aspirations, spinal taps, blood
transfusions, and chemotherapy.
– Provide a means for talking about the experience.
Play, storytelling, or role-playing may be helpful.
– Convey to the child an acceptance of fears and
anger.
40. Family Education and Health
Maintenance
Teach parents that children who have only
one kidney should not play rough contact
sports, to avoid injuring the remaining kidney.
Advise parents to call health care provider if
child has a fever of more than 101° F
(38.3° C), bleeding, signs of infections, or
exposure to chickenpox if the child has not
had it.
41. Conti..
Teach measures to prevent infection, such
as hand washing and isolation from children
with communicable disease.
Refer families to resources such as Candle
lighters