Didi's Files

8/8/2019 Didi's Files

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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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