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Wilms TumorBy: Feniel Alquizar
Aldin Piamonte
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Definition
Wilms'tumor or nephroblastoma is cancer ofthe kidneys that typically occurs in children,
rarely in adults.
Its common name is aneponym.
Referring to Dr. Max Wilms, the German
surgeon (18671918) who first described this
kind of tumor.
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Wilms tumor grows rapidly and may be very
large at the time of diagnosis. A fragile thin
capsule that may be easily torn or broken
usually encloses the tumor, which may be
present in one or both kidneys. There are two
categories to describe the histology of a
Wilms tumor, favorable and unfavorable.
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Favorable- tumors are more responsive to
therapy and, as the name implies, have a
favorable prognosis; whereas,
Unfavorable- tumors have poorer prognosis
(prediction of course and outcome of a
disease).
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Pathophysiology
The pathophysiology ofWilms tumor is
characterized by an abnormal proliferation of
the metanephric blastema cells, which are
believed to be primitive embryologic cells of
the kidney.
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arises from the developing kidney by genetic
and epigenetic changes
abnormal proliferation of the metanephricblastema cells
(primitive embryologic cells of the kidney)
Wilms Tumor
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Diagnostic Tests
Plain abdominal radiograph often shows displacementof abdominal organs and occasionally the presence ofcalcification (
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CT scan shows the tumor within the kidney, oftenwith a rim of normal kidney tissue around theedge and distortion of the urine collecting systemof the kidney and displacement of the kidneytoward the center of the body.
Also defines the Wilms tumor as being within the
kidney; identifies the presence of enlarged andsuspicious lymph nodes; evaluates the possiblepresence of a second Wilms tumor in theopposite kidney; assesses involvement of the
tumor into the veins leaving the kidney and goingback to the heart (renal vein, inferior vena cava,right atrium), and determines if the patient hasspread of tumor to the liver.
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Chest CT is obtained to evaluate for the
presence of tumor spread to the lungs.A CTscan cannot determine whether the tumor
can be removed, however, because this can be
assessed accurately only at the time of
surgery.
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Arteriograms (injection of dye into the
arteries so that they can be seen on x-rays) are
rarely helpful in the preoperative evaluation of
Wilms tumor, and for most patients this test
is rarely necessary.
Magnetic resonance imaging (MRI)may be of
help in patients with suspected inferior vena
cava or right atrial tumor or in patients withWilms tumor on both sides.
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The role ofultrasound to evaluate infants and
children with a risk for Wilms tumor. It is
successful in identifying these children atearlier stages of disease.
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Screening does increase the proportion
of children in whom the tumor can be
removed while keeping part of thekidney. Current recommendations are for
ultrasound every 3 months until 6 to 8
years of age. For children with Beckwith-Wiedemann syndrome and
hemihypertrophy, the adrenal glands and
liver should be evaluated in addition tothe kidney because this group is also at
risk for liver tumors (hepatoblastoma)
and neuroblastoma.
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Therapeutic Management
Surgical removal of the tumor and effected
kidney (nephrectomy) is the treatment of
choice and also allows for accurate staging
and assessment of tumor spread.
Radiation or chemotherapy may be
administered either before or after surgery.
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Sequence depends on the stage of the disease
at the time of diagnosis
If without metastasis
surgery (nephrectomy)
chemotherapy (actinomycin D; Vincristine)
ionizing radiation (cobalt/deep x-ray for 24exposure days)
If with metastasis
radiation to both lung fields chemotherapy (actinomycin)
surgery (thoracotomy)
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Nursing Management
It is important to prepare parents and the
child for the extent of the incision, which is
considerable.
General nursing measures for the comfort ofthe patient are carried out. One factor
pertinent to this condition is that all
unnecessary handling of the abdomen is to beavoided because it can cause the tumor to
spread.
STRICTLY NO ABDOMINAL PALPATION.
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Support child when urinating.
Treatment regimens by stage
The treatment your child undergoes depends
onthe stage of the cancer, the type of cancer
cell,and the child's age and general health.
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Stage I or II cancer. If the cancer is restricted tothe kidney or nearby structures and the cell type
is not aggressive, your child will undergo removal ofthe affected kidney and tissues and some of the
lymph nodes near the kidney, followed by
chemotherapy. Some stage II cancers are also treated
with radiation.
Stage III or IV cancer. If the cancer has spreadwithin the abdomen and can't be completely
removed without jeopardizing structures such as
major blood vessels radiation will be added to
surgery and chemotherapy. Your child may undergo
chemotherapy before surgery to shrink the tumor.
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Stage V cancer. If the tumor cells are in
both kidneys part of the cancer from
both kidneys will be removed duringsurgery and neighboring lymph nodes
taken to see if they contain tumor cells.
Chemotherapy is used to shrink theremaining tumor. Surgery is repeated to
remove as much tumor as possible while
leaving functioning kidney tissue. Morechemotherapy and radiation therapy may
follow.
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Children react differently to therapies, so
adjustments may be necessary. Discuss
your child's treatment plan with his or
her doctor and make certain that you
understand the benefits and risks before
giving your consent. Ask the doctor
about side effects of treatments and
when to report them, and what can be
done to ease them.
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Nursing Diagnosis
1. ANXIETY
2. RISK FOR INJURY
3. FAMILY PROCESSES, INTERRUPTED
Reference
http://emedicine.medscape.com/article/453076-overview
http://en.wikipedia.org/wiki/Wilms%27_tumor
http://www.geneimprint.com/media/pdfs/14987361_fulltext.pdf
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Thank You!!!
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