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Nephroblastoma Rahul Dhaker
M.Sc. N. II year
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.
Cont….
Wilms' tumour was named after Dr Max Wilms, who first
described it. It's 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
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.
Signs & Symptoms
Signs and symptoms of neuroblastoma vary depending on
what part of the body is affected.
Neuroblastoma in the abdomen
Neuroblastoma in the chest
Cont…
Neuroblastoma in the
abdomen :-
Abdominal pain
A mass under the skin that
isn't tender when touched
Changes in bowel habits,
such as diarrhea
Swelling in the legs
• Neuroblastoma in the
chest :-
• Wheezing
• Chest pain
• Changes to the eyes,
including drooping
eyelids and unequal
pupil size
Other signs and symptoms that may indicate neuroblastoma include
Lumps of tissue under the skin
Eyeballs that seem to protrude from the sockets
(proptosis)
Dark circles, similar to bruises, around the eyes
Back pain
Fever
Unexplained weight loss
Bone pain
history and physical
Urinalysis:-
blood, sugar, protein and bacteria infection
Blood tests:-
Radiology studies
Ultrasound
CT scan
X-ray of the abdomen
Biopsy
Diagnosis
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…
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.
Surgery
Chemotherapy
Radiotherapy
Stem cell transplant
Surgery
vincristine and actinomycin D are recommended for all stages
Chemotherapy
Stage
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.
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
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
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
Stage V
bilateral renal involvement at the time of initial diagnosis
Treatment: Individualized therapy based on tumor burden
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.
Radiotherapy
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.
Stem cell transplant
Side effects feeling sick (nausea) and being sick (vomiting),
hair loss,
bruising and bleeding,
tiredness,
diarrhoea and
an increased risk of infection.
Nursing Management
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