Wilms Tumor Also known as NEPHROBLASTOMA Its common name is ANEPONYM, referring to Dr.

r. Max Wilms, the German surgeon (18671918) who first described this kind of tumor diagnosed only when the tumor(s) is/are already big, but before the cancer metastasize (spreads throughout the body) cancer of the kidneys that typically occurs in children, rarely in adults usually strikes when a child is about 3 years old risk of developing Wilms' tumor is slightly higher in girls than in boys Stages: o Stage I - cancer is only found in the kidney o Stage II - cancer has spread deeper into other parts of the kidney and to nearby blood vessels, but was completely removed by surgery. No cancer cells are found at the edges of the area where the tumor was removed. o Stage III - cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen o Stage IV - cancer has spread to distant structures such as the lungs, liver or brain o Stage V - cancer cells affect both kidneys Etiology: Due to loss or inactivation of a tumor suppressor gene called WT1 on chromosome 11. Tumor suppressor genes usually suppress the growth of tumors and control cell growth. Incidence: According to the National Cancer Institute (2007), the highest incidence of kidney cancer occurs in the United States, Canada, Northern Europe, Australia, and New Zealand. The lowest incidence is found in Thailand, China, and the Philippines. In Philippines, Childhood cancers are the #1 disease killer of children - more than asthma, cystic fibrosis, diabetes, and pediatric AIDS combined. According to DOH, Wilms Tumor compromises 3.4% of all childhood cancers General Signs and Symptoms: A firm, non-tender mass in the upper quadrant of the abdomen is usually the presenting sign. It may be on either side. Abdominal pain which is related to rapid growth of the tumor.

 As the tumor enlarges, pressure may cause constipation, vomiting, abdominal distress, anorexia, weight loss and dyspnea. BODY SYSTEM Endocrine System SIGNS & SYMPTOMS > fever > cannot stimulate the kidney for the production of erythropoietin Lymphatic System > decrease production of RBC in the bone marrow RATIONALE > unknown reason > due to the kidney cannot perform its function well > due to the decrease or absence of erythropoietin hormone which helps the bone marrow in the production of RBC > accumulation of fluid in the peritoneal cavity due to excessive fluid in the body > because any fever may cause temporary loss of appetite and all the nutrient that suppose to be for the body is being eaten by the tumor cells > due to infants who are still exclusively breastfed may go 7 days without a stool (for infant patients) and also due to the fluid imbalance in the digestive system > due to uremia the body compensate by excreting the toxins through vomiting

> ascites Digestive System > loss of appetite

> constipation

> nausea and vomiting Urinary System > hematuria (blood in the urine) > ischemia > indicates that blood has passed through the tubule > due to its increasing tumor cells, compressing the surrounding tissue > due to blockage of tumor cells in the urine way the urine output decreases

> GU defects (in terms of

excretory) Reproductive System > GU defects (in terms of genital) > the genital decreases the urine output due to the CREA which is suppose to be excreted is reabsorb again and goes into the circulation. > occur only when metabolic process is affected > due to the CREA that is not excreted, can expel out by perspiration which is very irritating > due to urochrome retention > pallor > edema >due to Na and water retention from reduced glomerular filtration rate > may be associated with hyperphosphatemia that results from renal failure > due to Na and water retention from reduced glomerular filtration rate > results from excessive fluid in the body that reaches in the lungs > that results from pulmonary edema > due to electrolyte imbalance

Skeletal System Integumentary System

> pain at the joint > itching

>pruritus Cardiovascular System > high Blood Pressure

Respiratory System > pulmonary edema

> dyspnea Nervous System > decrease level of consciousness > lethargy Muscular System Hematologic System > muscle weakness > anemia

> mental and physical sluggishness > due to electrolyte imbalance, atrophy and collection of uremic toxins > due to erythropoietin are not produce which helps bone marrow to make RBC > presence of abnormal levels of urea and

> Uremia

other nitrogenous products in the blood due to the kidney cannot excrete it

Diagnostic Evaluation: Abdominal ultrasound detects the tumor and assesses the status of the opposite kidney Chest X-ray and CT scan may be done to identify metastasis MRI or CT scan of the abdomen may be done to evaluate local spread to lymph nodes Urine specimens show hematuria; no increase in vanillylmandelic acid and homovanillic acid levels as occurs with neuroblastoma Complete blood count, blood chemistries, especially serum electrolytes, uric acid, renal function tests, and liver functions tests, are done for baseline measurement and to detect metastasis Nursing Interventions: Instruct patient/SO to practice strict hand washing before and after eating or before and after using the toilet to prevent the spread of infection among the family members and to reduce the risk of wound infection. Encourage patient to wear mask to prevent inhaling microorganisms because patient is immunocompromised. Instruct patients mother to keep patients fingernails clean and short to prevent from scratching that results in skin injury. Instruct patient /SO to clean thoroughly fresh fruits before eating to avoid from ingestion of pathogens and any microorganisms. Instruct SO to provide a smoke free and dust free environment for the patient to avoid nasal irritation that might aggravate patients condition. Instruct SO to change bed linens thrice a week to prevent to prevent dwelling of microbes, dust and viruses in the linens. Clean skin surface with running water and mild soap after change of dressing to reduce skin contaminants. If patient cannot take a bath, offer him regular sponge bathing when client verbalized feeling of discomfort to promote comfort and well-being. Tell the mother to make sure that her child avoid vigorous exercise or activities and exhaustion and always have rest periods. Instruct patient to limit body movements, especially bending his back. Avoid prodding or pushing on the child's belly area to avoid injury to the tumor site.

 Encourage walking soon after surgery for early return bowel function, promotes effective breathing, mobilizes secretions, improves circulation, prevents stiffness of joints and relieves pressure Inform the mother to inspect surgical site/size, noting characteristics and integrity to recognize early or delayed healing/developing complication may prevent a more serious situation such as infection. Monitor surgical dressing, placement, and bleeding to make appropriate referrals if there are significant findings such as hypovolemic shock. Monitor the vital signs especially the temperature because patient might have infection already. Measure and record the abdominal girth of the patient because shet has higher risk of having spleenomegaly and haepatomegaly due to the condition. Prepare favorite foods to gain weight for upcoming chemotherapy because her appetite may decrease Avoid legumes because it is high in purines which produce uric acid that can deposit in the kidneys in the form of urate stones. Instruct the mother to follow the medication program carefully. The drugs prescribed to patient are designed to prevent various complications and to treat the condition. Strictly instruct the mother not to give any medicines, over-the-counter drugs, vitamins, herbs, or food supplements to her child without first talking to physician. Teach the SO on how to administer the medication, its correct route, dosage, and timing. Medical Management: Surgery Surgery is the main treatment approach used in patients with Wilms' tumor in the USA. In Europe, the patient undergoes surgery after they receive chemotherapy. Surgical removal of the kidney tissue is called nepherectomy. There are four types of nepherectomy performed in patients with Wilms tumor: o Simple nepherectomy. During this surgery, the doctor removes the entire kidney where the tumor is present. o Partial nepherectomy. During this surgery, the doctor removes the tumor together with the surrounding kidney tissue. o Radical nephetectomy. During this surgery, the doctor removes the tumor together with the entire kidney, the ureters (thin tube that carries the urine from the kidney to the urine bladder), the adrenal gland, the fat tissue that capsules the kidney, and sometimes with the adjacent lymph nodes. o Nephron-sparing surgery. During this surgery, the doctor tries to remove only the tumor with as little damage to the kidney tissue as possible.

 Chemotherapy Chemotherapy is a systemic treatment (affects cells throughout the entire body) that uses drugs either to stop the abnormal growth and dividing process of the cancer cells, or to kill the cancer cells. This treatment can be administrated as pills, through injections, or through a catheter placed in a blood vessel. Radiation Therapy This is a treatment approach which uses high-energy rays or particles to destroy cancerous cells. The most common type of radiation therapy used in patients with Wilms' tumor is external beam therapy, which uses an external device (linear accelerator) to generate high-energy rays that focus on the tumor. This type of treatment is often use along with surgery in advanced stages of Wilms' tumor patients (stage III, V, and VI) or in early stages (stage I a1nd II) if the tumor has an unfavorable histology.

PATHOPHYSIOLOGY of WILMS TUMOR

Predisposing Factors 1.Age: Wilms Tumor diagnosed at the age of 3-5 y.o. Our client is 4 y.o. 2.Congenital anomalies 3.Heredo-familial Predisposition to cancer Precipitating Factors 1.Second Hand Smoking: The father or our client is smoking 6-8 cigarette stick/day in their house 2. Exposure to asbestos, heavy metal

Destruction of the structure of chromosome 11 Hemihypertrophy Cryptorchidism Decrease in the number of WTI Unregulated and unsuppressed growth of cells and tissues Cellular mutation in the kidney Affects the renal cortex Tumor production Cortical and medullary infiltration and proliferation

Suppression of function of nephrons

Wilm's tumor
Cancer is confined only in the kidney (Stage I)

With medical-surgical intervention: Good prognosis Prevent metastasis

Without medical-surgical intervention: Tumor progression

Spread beyond the kidney into a nearby fat or tissue (Stage II)

With medical-surgical intervention: Good prognosis Prevention of metastasis

Without medical-surgical intervention: Tumor progression

Abdominal mass Constipation Abdominal pain (minimal) Loss of appetite Fever(low grade)

Spread to lymph nodes from the abdominal cavity and adjacent vital structures (Stage III)

With medical-surgical intervention: Good prognosis Prevention of metastasis Abdominal mass Constipation Abdominal pain(moderate) Loss of appetite Fever(high grade)

Without medical-surgical intervention: Tumor progression

Cancer enters blood stream and affects other organs (Stage IV)

With medical-surgical intervention: Good prognosis Prevention of metastasis

Without medical-surgical intervention: Tumor progression

Cancer is present on both sides of the kidney

Decrease production of Vit. D Decreased absorption of calcium in the intestines

Altered RAA mechanism

Decreased erythropoietin production Decreased number of RBC

Altered waste production Decreased urine formation Urinary retention

Altered Acid Excretion

Hypertension Urinary Retention

Decreased catabolism of proteins Acidity

Anemia Hypocalcemia Edema