2. Definition
Wilm’s Tumor is also known as Nephroblastoma. It is
a highly malignant embryonal neoplasm.
It may involve one or both kidney.
3. INCIDENCE AND ETIOLOGY
Usually the tumor is unilateral, but in 5% cases it
may be bilateral.
The tumor involves left kidney more than right
kidney.
It affects children between 3-5 year of age.
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4. CONT……………..
The disease occur in about 1 out of 2-2.5 lakh
children.
The exact cause of tumor is unknown, but it has
been identified that tumor suppressor gene acts to
promote normal kidney development. This gene
may be absent or missing in wilm’s tumor.
5.
6. PATHOPHYSIOLOGY
Mostly wilm’s tumor is unilateral but it can be bilateral in
5% of cases
Nephroblastoma are generally large and rapidly growing.
Tumor generally start growing in renal parenchyma or at the
tip of kidney.
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7. Cont………….
It causes suppression of normal tissue remaining
Majority of tumors present a as single encapsulated mass, that
separates the normal kidney and tumor.
Although the tumor is encapsulated but the membrane may be very
thin and get easily torn
Rupture of tumor put patient at the risk of hemmorhage and
dissemination of tumor
8. CLINICAL FEATURES
Presence of abdominal
mass.
Pain ,if tumor is enlarging
Anorexia
Hematuria
Nausea and vomiting
Urinary tract infection
9. STAGES OF WILM’S TUMOR
STAGE I (43% CASES ) - Tumor limited to kidney and
completely resectable.
STAGE II (23% CASES) - Tumor extend beyond kidney , into
nearby fatty tissue, but it is resectable.
STAGE III (23% CASES) – Non hematogenous spread in
abdomen, like spread to lymph nodes in abdomen or pelvis,
but this stage tumor is not completely resectable.
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10. CONT…………………
STAGE IV (10% CASES) - Hematogenous metastatis
to lungs and liver.
STAGE V (5% CASES)- Bilateral renal involvement.
12. DIAGNOSTIC EVALUATION
History- The child may have positive family history.
Physical examination reveals presence of abdominal mass.
Urinanalysis reveals presence of blood in urine.
Abdominal x-ray
13. Cont………….
Ultrasound
Chest X- ray to detect metastasis to lungs.
Increased Blood urea nitrogen, creatinine values.
14. MANAGEMENT
The management of children with wilm’s tumor include
:-
Radiation therapy
Chemothrapy
Surgical management
15. Radiation Therapy
Wilm’s tumor may be bilateral or large in size ,
may be inoperable, for such cases radiation
therapy may be used to reduce the size of
tumor, so that surgery can be performed.
16. CHEMOTHERAPY
The objective of chemotherapy is to treat any
metastatic lesions that may exist and destroy
any cells in blood stream, before they get
implanted.
The drugs used for chemotherapy are
Actinomycin D, Doxorubicin and Vincristine.
17. SURGICAL MANAGEMENT
Partial or complete nephrectomy is done for
unilateral and for bilateral partial nephrectomy is
done.
After surgical management, chemotherapy and
radiation therapy is given if indicated.
18. TREATMENT
Treatment for wilm’s tumor is based mainly on the
stages of the cancer:-
STAGE I- These tumor are still only in the kidney.
Standard treatment starts with surgery to remove the
part of kidney containing tumor. These children needs
to be watched closely because the chances of
recurrence are higher.The chemotherapy is given for
18 weeks.
19. cont…………..
STAGE III- Treatment is usually surgery followed by
radiation therapy to the abdomen over several days .
This is followed by chemotherapy for about 6
months.
STAGE IV- These tumors are already spread to distant
parts of the body at the time of diagnosis, so
standard treatment id surgery followed by radiation
and chemotherapy.
20. Cont…………
STAGE V- In this stage usually tumor is bilaterally
present, standard treatment involves surgery,
radiation and chemotherapy repeatedly until normal
kidney tissue left behind. In case if not enough
kidney tissue is left after surgery that child may need
to place on dialysis. If there is no evidence of any
cancer after year or two, a donor kidney transplant
may be done.
21. NURSING MANAGEMENT
PREOPERATIVE CARE
Prepare the parents and child for surgery.
Expalin parents not to palpate the abdomen of the
child.
Explain child about post operative care , if he or she is
old enough.
Caution should be taken while turning and handling
the child.
22. POST OPERATIVE CARE
Monitor vitals signs.
Monitor renal functioning by monitoring
weight, intake output and KFT values.
Obsesrve for the signs of functioning.
Use aseptic techniques while doing dressing
Explain parents about follow up and
continuing treatment at the time of discharge.
23. Current Research
Research on diagnostic procedures and treatment
for Wilms tumor is ongoing. The following advances
may still be under investigation in clinical trials and
may not be approved or available at this time.
Always discuss all diagnostic and treatment options
with your child’s doctor.
24. New tumor markers. A tumor marker, also called a serum
marker or biomarker, is a substance found in a person's
urine, or body tissue. Doctors in the United Kingdom recently
discovered that children with Wilms tumor who have a
recurrence all have active genes on one area of a specific
chromosome (the 1q area). Children can be tested to see if
their 1q genes are active, which can help doctors plan
treatment. Once the specific genes are identified, it is
that new tests may be developed to predict which children
likely to have a recurrence.
25. Genetics. A new Wilms tumor gene (WTX) has been
identified on the X chromosome and plays a role in
normal kidney development. This gene is not active in
approximately one-third of children with Wilms tumor.
Studies on the importance of this gene are ongoing.
New therapy combinations. Researchers are looking at
whether patients with a favorable histology Wilms tumor
who have metastases in the lungs need radiation
to the lungs if the lung tumors go away after six weeks
chemotherapy.
