Seminar on Nephroblastoma

(Willms Tumor)

PRESENTING BY: NARESH KUMAR SONI

MSC. NURSING 2ND YEAR
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General objective
At the end of class all students will be able
to understand the topic Wilms tumor its
stages and apply this knowledge in
nursing practice.

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Introduction
Wilms tumor, orNephroblastomaiscancer of
thekidneys that typically occurs inchildren rarely in
adult is named after DrMax Wilms the German surgeon
(18671918) who first describedabout Wilms tumor.
Most Nephroblastoma are unilateral, being bilateral in
less than 5% of cases, They tend to be encapsulated
and vascularized tumors that do not cross the midline of
the abdomen. In cases of metastasis it is usually to the
lung. A rupture of Wilms' tumor puts the patient at risk
of hemorrhage and peritoneal dissemination of the
tumor. In such cases, surgical intervention by a surgeon
who is experienced in the removal of such a fragile
tumor is imperative.
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Incidences in India And

Uttrakhand
The annual incidence rate in India is about 7 cases
per 500 Children from 0 to 15 years old.
Nephroblastoma incidence is less in Uttrakhand
and utter Pradesh . It is a young child cancer,
starting between 1 and 5, with 98% of the cases
occurring before the age of 7. Neonatal forms are
rare. More than 200 cases have occurred in adults,
sometimes even elderly adults.

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Causes
Wilms tumor is the most common form of childhood kidney
cancer In most cases, Wilms tumor cannot be linked to a specific
cause.
Studies are under way to determine whether environmental
factors, such as drugs, chemicals, or infectious agents, can
cause Wilms tumor.
Some children have genetic factors that increase their risk of
developing the disease, including certain syndromes that are
associated with Wilms tumor.
One such condition is Beckwith-Wiedemann syndrome, which
is a growth disorder that causes body and organ enlargement.
Other abnormalities associated with this cancer include a
missing iris of the eye (aniridia) is a birth defect that is
sometimes associated with Wilms tumor.
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Cont..
Other birth defects linked to this type of kidney
cancer include certain urinary tract problems and
swelling of one side of the body, a condition
called hemi hypertrophy.
About 1.5 percent of children with this cancer
have a relative who is also affected by the
disease.
Siblings and twins are also more commonly
diagnosed, which point to genetic factors as a
cause of the cancer

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Patho-physiology
Wilms tumor (WT) is an abnormal proliferation of
the mesanephric layer with no differentiation into
tubules or glomeruli and usually arises from three
tissue elements: Blastema microscopically undifferentiated
small round blue cells arranged in a diffuse or
organoid pattern

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 Epithelium- epithelial structures [tubules or

glomeruli] develop out of condensations of
blastema and may simulate the nephrogenic
development of the kidney, however larger
capillary formations unlike normal developing
kidney may also be present while heterologous
squamous or mucinous elements may also be
identified

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 Stroma- this is usually manifest as immature

spindled cells, but heterologous differentiation
may result in the presence of skeletal muscle,
osteoid(organic portion of the bone matrix that
forms prior to the maturation ofbone tissue), or
fat.

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SIGN AND SYMPTOMS

The disease usually has no symptoms that are
obvious to the child or parent.
Instead, The hard mass of the tumor is most often
felt by the physicians hands during a routine
exam of the abdomen.
Parents might also find a lump in the belly.
Symptoms of Wilms tumor are similar to those of
other childhood ailments, so it is important to see
your doctor for an accurate diagnosis. Children
with Wilms tumor might display the following
symptoms:
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Typical symptoms are:

An abnormally large abdomen

abdominal pain
fever
blood in the urine (in about 20% of cases) or
discoloration of urine
increase and growth of one side of body

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Diagnosis
Unlike most cancers, Wilms tumor does not generally
interfere with a childs health even though it may
become very large.
The average weight of a newly found tumor is one half
pound(226gms) thats four times larger than a
kidney of the average three-year-old.
Though large, the Wilms tumor cells do not tend to
spread quickly. Most are found during the childs
annual physical exam when the doctor presses deeply
into the childs stomach area, above the navel.
Several tests are used to diagnose Wilms tumor. They
are:
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 Ultrasound:Sound waves directed at the tumor help make a

picture of the tumor.
CT Scan(computed tomographic scan): Similar to an x-ray, this
machine produces pictures of soft tissues that will show irregular
swellings and lumps possible tumors in the kidney.
CT Scan of chest: This is used to look for possible spread of the
tumor.
MRI(magnetic resonance imaging): This uses magnetic waves to
make a picture of the kidney.
Chest and bone x-rays: These may be taken to determine if the
Wilms tumor cells have spread to sites beyond the kidney.

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 Biopsy and surgical removal: If any of the

procedures above show abnormal tissue, the
doctor will usually try to surgically diagnose it. In
some cancers, biopsies are done first, but with
most cases of Wilms tumor, the surgeon attempts
to take out all of the tumor instead of removing
just a portion to examine under a microscope.

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Staging of Wilms tumor

Once Wilms tumor is found, tests are done to
determine the extent to which the cancer cells
have spread from their point of origin to
surrounding tissues, or to other parts of the body.
This is called staging the cancer. Staging is an
important step because it helps the childs doctor
recommend the most effective treatment
approach. The stages of Wilms Tumor are:

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Stage I (43% of patients)

For stage I Wilms' tumor, all of the following criteria
must be met:
Tumor is limited to the kidney and is completely
excised.
The surface of the renal capsule is intact.
The tumor is not ruptured or biopsied (open or needle)
prior to removal.
No involvement of extra renal or renal
sinus lymph-vascular spaces
No residual tumor apparent beyond
the margins of excision.
Metastasis of tumor to lymph nodes not identified.
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Stage II (23% of patients)

For Stage II Wilms' tumor, 1 or more of the following criteria
must be met:
Tumor extends beyond the kidney but is completely excised.
No residual tumor apparent at or beyond the margins of
excision.
Any of the following conditions may also exist:
Tumor involvement of the blood vessels of the
renal sinus and/or outside the renal parenchyma.
The tumor has been biopsied prior to
removal or there is local spillage of tumor
during surgery, confined to the flank.
Extensive tumor involvement of renal
sinus soft tissue.
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Stage III (23% of patients)

For Stage III Wilms' tumor, 1 or more of the
following criteria must be met:
Unresectable primary tumor.
Lymph node metastasis.
Tumor is present at surgical margins.
Tumor spillage involving peritoneal surfaces
either before or during surgery, or transected
tumor thrombus.

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Stage IV (10% of patients)

Stage IV Wilms' tumor is defined as the presence

of hematogenous metastases (lung, liver, bone,
or brain), or lymph node metastases outside the
abdomenopelvic region.

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Stage V (5% of patients)

Stage V Wilms tumor is defined as bilateral renal
involvement at the time of initial diagnosis. Note:
For patients with bilateral involvement, an
attempt should be made to stage each side
according to the above criteria (stage I to III) on
the basis of extent of disease prior to biopsy.

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Treatment
Treatment for Wilms tumor depends on the stage
of the disease, the cell type (histology), and the
age and overall health of the child. Whatever the
stage, all patients with Wilms tumor have
treatment options available to them. They consist
of the following approaches, used singly, serially
or in combination, depending on the location,
stage and biological characteristics of the cancer
cells.

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Partial nephrectomy
The cancer and part of the kidney nearest the
cancer are removed. This operation is usually
performed only in special cases, such as when
the other kidney is damaged or has already been
removed.

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Nephrectomy
A whole kidney is removed. The remaining
kidney on the other side of the body performs the
work of filtering the blood. In children, the
remaining kidney will grow an additional ten or 20
percent to accommodate this extra work. A
nephrectomy is the most common surgery for
Wilms tumor.

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Chemotherapy
This method relies on powerful anti-cancer drugs
that are usually injected into a vein. It is a
systemic treatment meaning it treats the
whole body, or system, because the circulating
blood carries the drugs throughout the entire
body. Chemotherapy given after the cancer has
been surgically removed is called adjuvant
chemotherapy.

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High-dose chemotherapy
In some instances, very high doses of
chemotherapy drugs will be used to kill cancer
cells. A possible side-effect of this dose is
destruction of the bone marrow, which is the bloodforming tissue inside the bones. To mitigate this
effect, doctors can withdraw portions of the
patients own bone marrow before therapy begins
and freeze it. After the high-dose chemotherapy is
over, the patients bone marrow will be
reintroduced into the body by a needle in a vein.
This is called an autologous bone marrow
reinfusion.
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Radiation therapy
This is used to kill cancer cells and shrink the size
of the tumor. Radiation relies on the use of x-rays
and other high-energy rays generated by a
machine outside of the body.

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Treatment
According to
stages of
Nephroblastoma
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Stage I Wilms tumor

If the child has a favorable-histology tumor, or
has anaplastic Wilms tumor, treatment will
probably be surgery to remove the cancer
followed by chemotherapy. If the child has either
clear cellsarcoma of the kidney or rhabdoid
tumor, treatment will probably be surgery to
remove the cancer followed by radiation therapy
plus chemotherapy.

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Stage II Wilms tumor

If the child has a favorable-histology tumor,
treatment will probably be surgery to remove the
cancer followed by chemotherapy. If the child has
an unfavorable histology tumor (anaplasia, clear
cellsarcoma, or rhabdoid tumor of the kidney),
treatment will probably be surgery to remove the
cancer followed by radiation therapy plus
chemotherapy.

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Stage III Wilms tumor

If the child has a favorable- or an unfavorable-histology

tumor (anaplasia, clear cellsarcoma, or rhabdoid tumor),
treatment will probably be surgery to remove the cancer
followed by radiation therapy plus chemotherapy.
Sometimes the cancer cannot be removed during surgery
because it is too close to important organs or blood
vessels or because it is too large to remove. In this case,
the doctor may only perform a biopsy and then give
chemotherapy with or without radiation therapy. Once
the cancer has become smaller, surgery can be
performed, followed by additional chemotherapy and
radiation therapy.
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Stage IV Wilms tumor

If the child has a favorable- or an unfavorablehistology tumor (anaplasia, clear cellsarcoma, or
rhabdoid tumor), treatment will probably be
surgery to remove the cancer followed by
radiation therapy with chemotherapy. Patients
whose cancer has metastasized to the lungs will
receive additional radiation therapy.

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Stage V Wilms tumor

Because both kidneys contain cancer, it is not
usually possible to remove both kidneys. A piece
of the cancer in both kidneys will probably be
removed, along with some of the lymph nodes
around the kidney to see whether they contain
cancer. Following surgery, chemotherapy is given
to shrink the cancer. A second operation is then
performed to remove as much of the cancer as
possible, while leaving as much of the kidneys as
possible. Surgery may be followed by more
chemotherapy and/or radiation therapy.
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Recurrent Wilms tumor

If the childs cancer comes back (recurs),
treatment depends on the treatment the child
received before, how much time has passed since
the first cancer was treated, the histology of the
cancer, and where the cancer came back.
Depending on these factors, treatment may be
surgery to remove the cancer, plus radiation
therapy and chemotherapy.

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Possible Complications
The tumor may become quite large, but usually
remains self-enclosed. Spread of the tumor to the
lungs, liver, bone, or brain is the most worrisome
complication.
High blood pressure and kidney damage may
occur as the result of the tumor or its treatment.
Removal of Wilms tumor from both kidneys may
affect kidney function.

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Nursing Management
Nursing Interventions
Observe the surgical incision for erythema, drainage
or separation. Report any of theses changes.
Monitor for elevated temperature or sign of infection
post-operatively.
Monitor I.V. fluid therapy and intake and output
carefully, including nasogastric (NG) drainage.
Encourage the parents to ask questions and to
understand fully the risk and benefits of surgery.
Prepare the child for surgery, explain the procedures
at the appropriate developmental level.
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Cont..
Continue supporting the parents during the
postoperative period.
Insert NG tube as ordered. Many children require gastric
suction postoperatively to prevent distention or
vomiting.
When bowel sounds have returned, begin administering
small amounts of clear fluids.
Administer pain control medications as ordered in the
immediate postoperative period.
Allow the child to participate in the selection of foods.
As the child recovers, encourage child to eat
progressively larger meals.
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Cont..
If unable to eat because of radiation and
chemotherapy provide I.V. fluids,
hyperalimentation, or tube feedings as indicated.
Prepare child and family for fatigue during recovery
from surgery and with radiation treatments. Plan
frequently rest periods between daily activities.
Prepare the child and parents for loss of hair
associated with chemotherapy and encourage use
of hat as desired. Reassure the hair will grow back.

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Research Article
Daily Life Physical Activity in LongTerm Survivors of Nephroblastoma
and Neuroblastoma

van Waas, Marjolein PhD*; Wijnen, Mark BSc*; Hartman, Annelies

PhD*,; de Vries, Andrica C.H. MD, PhD*; Pieters, Rob MD, PhD*;
Neggers, Sebastian J.C.M.M. MD, PhD*,; van den Heuvel-Eibrink,
Marry M. MD, PhD

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Research Statement :Daily Life Physical Activity in Long-Term Survivors of Nephroblastoma and
Neuroblastoma
Research objective : The aim of the study was to assess daily life physical activity in Dutch adult
long-term nephroblastoma and neuroblastoma survivors.
Research tool : We used the Short QUestionnaire to ASsess Health-enhancing physical activity
(SQUASH), which was developed and validated to assess daily life physical activity in the Dutch
adult population.
Population : Dutch adult population.
Sample: (163) Sixty-seven nephroblastoma and 36 neuroblastoma survivors (median age, 30 y;
range, 18 to 51 y) and 60 sociodemographically similar healthy control subjects (median age, 32 y;
range, 18 to 62 y) were asked to complete the SQUASH during their regular follow-up visit.
Result : The adjusted mean physical activity score in male neuroblastoma survivors (mean,
7155;P=0.004) was significantly lower than in male controls (mean, 10,574), whereas it was not
significantly lower in male nephroblastoma survivors (mean, 9122;P=0.108). The risk of metabolic
late effects after childhood cancer, such as obesity, hypertension, and diabetes, can be positively
influenced by a healthy lifestyle with sufficient physical activity. Nevertheless, studies on physical
activity in adult survivors of childhood cancer are scarce and involve different and often
nonvalidated questionnaires. Adjusted means for physical activity scores in females were not
different from their controls.
Conclusion : In conclusions, male neuroblastoma survivors were identified as performing less
daily physical activity.

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Bibliography
Bibliography
http://en.wikipedia.org/wiki/Wilms'_tu
mor
http://www.childrenscancer.org/main
/wilms_tumor_nephroblastoma/
http://www.nlm.nih.gov/medlineplus/e
ncy/article/001575.htm
http://ghr.nlm.nih.gov/glossary=neph
roblastoma
http://www.gustaveroussy.fr/?p_m=
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