Nephroblastoma
Introduction
🞂 Nephroblastoma is a type of cancer which develops in the
kidneys.
🞂 This cancer is also called Wilms tumor, and is the most
common type of kidney cancer to develop in children.
🞂 In the 1950s and 1960s, most children with nephroblastoma
had a very poor prognosis, but treatment for the cancer has
advanced significantly in the intervening decades.
🞂 The success rate for Wilms tumor treatment is now
approximately 90%, largely due to the practice of combining
surgery with radiotherapy or chemotherapy.
C ont…
🞂 Wilms' tumour was named after Dr. Max Wilms, who
first described it as a type of kidney cancer.
🞂 It's thought to come from very specialised cells in the embryo
known as metanephric blastema.
🞂 These cells are involved in the development of the child's
kidneys while they're in the womb.
🞂 These cells usually disappear at birth, but in many children
with Wilms' tumour, cells called nephrogenic rests can still be
found.
Definition
🞂 A Wilms tumor is a malignant tumor (cancer) which
means it has the ability to grow and spread. The places
it usually spreads to include the lungs, liver and nearby
lymph nodes.
Causes & Incidence
🞂 In most children the causes of Wilms' tumour are unknown.
🞂 In 1 in 100 people with Wilms' tumour, another family
member will also have Wilms' tumour.
🞂 Nephroblastoma has a global incidence rate of 0.8 cases
per 100,000 people.
🞂 Most children with this cancer are diagnosed between three
and four years of age. Children commonly have tumors in
one kidney only; in around 6% of cases tumors grow in
both
kidneys.
Staging of Nephroblastoma
🞂 Stage I
🞂 Tumor is limited to the kidney and is completely
excised.
🞂 The surface of the renal capsule is intact.
🞂 Treatment: Nephrectomy +/- 18 weeks of
chemotherapy
…depending on age of patient and weight of tumor.
Cont…
Staging of Nephroblastoma(cont’d)
🞂 Stage II
🞂 Tumor extends beyond the kidney but is completely
excised.
🞂 Tumor involvement of the blood vessels of the renal sinus
and/or outside the renal parenchyma.
🞂 Treatment: Nephrectomy + abdominal radiation + 24
weeks of chemotherapy
Cont…
Staging of Nephroblastoma(cont’d)
🞂 Stage III
🞂 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.
🞂 Treatment: Abdominal radiation + 24 weeks of
chemotherapy + nephrectomy after tumor shrinkage
Cont…
Staging of Nephroblastoma(cont’d)
🞂 Stage IV
🞂 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.
🞂 Treatment: Nephrectomy + abdominal radiation + 24
weeks of chemotherapy + radiation of metastatic site as
appropriate
Cont…
Staging of Nephroblastoma(cont’d)
🞂 Stage V
🞂 bilateral renal involvement at the time of initial
diagnosis
🞂 Treatment: Palliative care + Individualized therapy based
on tumor burden
Signs & Symptoms
🞂 Abdominal pain
🞂 Abdominal mass you can feel
🞂 Unexplained weight loss
🞂 Distended abdomen (abdominal swelling)
🞂 Fever
🞂 Blood in urine (hematuria)
Diagnosis
🞂 History taking and physical assessment
🞂 Urinalysis:-
🞂 blood, sugar, protein and bacteria infection
🞂 Blood tests:-
🞂 Full Blood Count,
🞂 Radiology studies
🞂 Ultrasound
🞂 CT scan
🞂 X-ray of the abdomen
🞂 Biopsy
Treatment
🞂 In general, this type of cancer is curable. If the tumor is only in
the
kidney (typical), it can be removed along with the whole kidney
(a nephrectomy). During the operation, the surgeon checks if the
other kidney has a tumor.
🞂 If there are tumors in both kidneys, a piece of the tumor will be
removed. After the surgery, the child is given some chemotherapy
drugs like Dactinomycin (trade name Cosmegen).
Cont…
Treatment (cont’d)
🞂 Children 16 years old or older have higher mortality
rates
within their stages. This is due to them being treated less
aggressively and consistently.
🞂 Adjuvant chemotherapy is sometimes used.
Cont…
Treatment (cont’d)
🞂 Surgery
🞂 Chemotherapy
🞂 Radiotherapy
🞂 Stem cell
transplant
Surgery
🞂 Nephrectomy (surgical
removal of the kidneys)
Chemotherapy
🞂 Vincristine
🞂 Actinomycin-D
…are recommended for all stages
Radiotherapy
🞂 Depending on the stage of the tumour at diagnosis,
radiotherapy may also be given to the area of the affected
kidney or to the whole abdomen.
🞂 Radiotherapy treats cancer by using high-energy rays to
destroy the cancer cells, while doing as little harm as possible
to normal cell.
Stem Cell Transplant
🞂 Children with high-risk neuroblastoma may receive a transplant
using their own blood stem cells (autologous stem cell
transplant). The bone marrow produces stem cells, which mature
and develop into the red and white cells and platelets that make
up the blood.
🞂 Child undergoes a procedure that filters and collects stem cells
from his or her blood. Then high doses of chemotherapy are used to
kill any remaining cancer cells in child's body. child's stem
cells are then injected into child's body, where they can form new,
healthy blood cells.
Side Effects
🞂 feeling sick (nausea) and being sick (vomiting),
🞂 hair loss,
🞂 bruising and bleeding,
🞂 tiredness,
🞂 diarrhoea and
🞂 an increased risk of infection.
Nursing Management
🞂 Reassure child’s mother (parent) to allay fear and anxiety
🞂 Explain procedures to mother (parent) and child, including
risks and importance of surgery
🞂 Provide diversionary therapy by using magazine or television
on the part of mother and toys or cartoons on the part of the
baby
🞂 Assess and manage pain of child by employing diversionary
therapy and administering prescribed pain medications
🞂 Ensure consent form is signed by child’s mother (parent) and
countersigned by surgeon before surgery.
Prognosis
🞂 Effective diagnosis, staging, and multimodality therapy has
dramatically reduced the morbidity and mortality in children
with nephroblastoma
🞂 Poor prognostic factors in children include the presence
of bone metastases and tumor spillage during surgery
END OF
PRESENTATION
THANK YOU FOR YOUR COOPERATION
