Introduction Risk factors pathophysiology sign and symtoms staging

1. WILM'S TUMOR
OR
NEPHROBLASTOMA
PRESENTED BY- Miss.Jyotsna Kamble
Nursing tutor
V.M.P. College of Nursing
& Medical research Institute, Akluj

2. INTRODUCTION
 Wilm's tumor is the most common type of renal
cancer in children.
 Wilm's tumor or nephroblastoma is a highly malignant
embryonal neoplasm affecting one or both kidneys.
 It is named after Max Wilms, a German doctor who
wrote one of the first medical articles about the
disease in 1899.
 It is the fifth most common neoplasm in children
affecting 6.5% of childhood malignancies.
 80% of children develop Wilm's tumor before 5 years
of age.

3. WILM'S TUMOR

4. RISK FACTORS/ETIOLOGY
 Age: Most common in young children with the average age being about 3-4
years.
 Gender Girls have a slightly higher risk of Wilms tumor than boys.
 Family History : About 1- 2% of children with Wilms tumors have one or more
relatives with the same cancer.
 Certain genetic syndromes: WAGR syndrome (Wilm's tumor, Aniridia,
Genitourinary tract abnormalities, Mental Retardation.
 Denys-Drash syndrome and Frasier syndrome. • Beckwith-Wiedemann
syndrome.
 Certain birth defects: Aniridia (complete or partial lack of the iris of the eyes),
cryptorchidism (a condition in which one or both of the testes fail to descend
from the abdomen into the scrotum)., and hypospadias (a congenital condition
in males in which the opening of the urethra is on the underside of the penis).
 Genetic: WT1 gene localized at chromosome 11 p13
 Unknown.

5. CONT…
 Denys-Drash syndrome is characterized by kidney disease
that begins within the first few months of life. Affected
individuals have a condition called diffuse glomerulosclerosis,
in which scar tissue forms throughout glomeruli, which are the
tiny blood vessels in the kidneys that filter waste from blood.
 Frasier syndrome is characterized by kidney disease that
begins in early childhood. Affected individuals have a
condition called focal segmental glomerulosclerosis, in which
scar tissue forms in some glomeruli, which are the tiny blood
vessels in the kidneys that filter waste from blood.
 Beckwith-Wiedemann syndrome is a condition that affects
many parts of the body. It is classified as an
overgrowth syndrome, which means that affected infants are
considerably larger than normal (macrosomia) and tend to be
taller than their peers during childhood.

6. PATHOPHYSIOLOGY
 Wilm's tumor rapidly increases in size before it is
diagnosed.
 It develops in renal parenchyma in the central or polar
location.
 The entire renal parenchyma is occupied with tumor
leaving only a rim of compressed normal tissue.
 The lesions are vascular, soft, gelatinous, and necrotic.
 The tumor appears as a single expanding mass
surrounded by a pseudocapsule of connective tissue
which seperates the kidney and tumor.
 The membrane encapsulating the tumor is very thin and
can easily be torn.

8. CLINICAL FEATURES
 Asymptomatic.
 Large abdominal mass.
 Hematuria.
 Hypertension.
 Abdominal pain.
 Fever.
 Anorexia and vomiting.
 Anemia, thrombocytosis.
 Polycythemia.

9. CLINICAL STAGING OF WILM'S TUMOR
ACCORDING TO CHILDREN'S ONCOLOGY GROUP STAGING SYSTEM:
 Stage I: The tumor was contained within one kidney and was
removed completely by surgery. About 40% to 45% of all
Wilms tumors are stage I.
 Stage II: The tumor has grown beyond the kidney, either into
nearby fatty tissue or into blood vessels in or near the kidney,
but it can be removed completely by surgery About 20% of all
Wilms tumors are stage II.
 Stage III: Spread to lymph nodes in the abdomen or pelvis.
Deposits of tumor (tumor implants) are found along the inner
lining of the abdominal space after the surgery. About 20% to
25% of all Wilm's tumors are stage III.

10. CONT…
 Stage IV: Tumor spread through the blood to
organs away from the kidneys such as the lungs,
liver, brain, or bones, or to lymph nodes far away
from the kidneys. About 10% of all Wilm's tumors
are stage IV.
 Stage V: Bilateral kidney involvement. About 5% of
all Wilms tumors are stage V.

11. DIAGNOSIS
 Abdominal X-ray: Shows soft tissue opacity in the site
of kidney.
 Ultrasonography and Doppler: Identify the organ of
origin, involvement of contralateral kidney.
 CT scan: Identifies tumor extension to adjacent
structures.
 Chest X-ray: Identifies pulmonary metastasis.
 Intravenous pyelography: Shows distortion of calyces
by a mass within the kidney.
 Fine needle cytology: For confirmation before
chemotherapy.

12. TREATMENT
 Overall, about 9-10 children with Wilms tumor are cured.
 The main types of treatment for Wilms tumor are surgery,
chemotherapy and radiation therapy.
 Most children will get more than one type of treatment.
 1. Surgery: Radical nephrectomy is done in most of the
cases.
 2. Chemotherapy: The chemotherapy drugs used most often
are actinomycin D (dactinomycin) and vincristine.
 In tumors with unfavorable histology, drugs such as
doxorubicin (Adriamycin) and cyclophosphamide may also be
used.
 Preoperative chemotherapy is indicated to diminish the size
of the tumor.
 Stage I and II tumors are treated with actinomycin D and
vincristine postoperatively

13. CONT,…
 3. Radiotherapy:
 It is usually used for more advanced Wilms tumors
(stages III, IV, and V) and for some earlier stage
tumors with unfavorable histology.
 External beam radiation therapy is given 5 days a
week for a couple of weeks.
 Abdominal radiation is given in stage III tumor and
pulmonary radiation is given for pulmonary metastasis.
 For advanced tumor combination of chemotherapy of
vincristine, actinomycin D, and adriamycin with
abdominal radiation are treatment of choice.

14. NURSING MANAGEMENT
 Assessment
 Inspect for abdominal distention.
 Assess for abdominal pain, fever, anorexia, and
vomiting.

15. NURSING INTERVENTION
Preoprative Care
 Explain the parents not to palpate the abdomen of their
child as it can rupture.
 Place a sign 'DO NOT PALPATE THE ABDOMEN' near
the bed side of the child.
 Prepare the child and family for surgery with age
appropriate explanation.
 Monitor vital signs regularly.
 Provide emotional support to the family.
 Allow the parents to express their concern and queries
regarding child's prognosis.
 Prepare for the side effects of chemotherapy and
radiotherapy.

16. CONT…
 Postoperative Care
 Monitor the child for GI activity, bowel sounds, stool
production, signs of infection and hemorrhage. Monitor vital
signs 2-4 hourly.
 Monitor urine output for kidney function.
 If NG tube is placed NG output should be measured every 4
hrs.
 Administer replacement fluid with potassium as gastric
contents are potassium rich.
 Monitor serum electrolyte level frequently.
 Administer antibiotics as prescribed.
 Administer chemotherapy drugs via large vein as they cause
soft tissue burns.
 Monitor for side effects of chemotherapy and radio-therapy.

17. NEUROBLASTOMA

45. THANK YOU