WILMS’ TUMOR

“NEPHROBLASTOMA”

PRESENTED BY: GROUP 2

MACKY JESSA GLENN DENN JILLIANE

INTRODUCTION

• Wilms' tumor is a rare kidney cancer that primarily affects children.

• also known as “Nephroblastoma”• It is named for Max Wilms, a German surgeon (1867-1918)• Having certain genetic conditions or birth defects can

increase the risk of getting it. • Children that are at risk should be screened for Wilms'

tumor every three months until they turn eight.• Wilms tumor is a type of kidney cancer that occurs in

children.• Is a malignant tumor.

• It accounts for 6% of all childhood cancers.• It generally grows to a large size before it is

diagnosed, usually before the child reaches age 5.

• The tumor expands the renal parenchyma, and the capsule of the kidney becomes stretched over the surface of the tumor.

• The tumor may metastasize to the lymph nodes, lungs, liver, and brain.

CAUSE: UNKNOWN

ASSESSMENT

• 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.

• Less common manifestation are hypertension, fever, hematuria, and anemia.

• Associated anomalies includes aniridia (absence of the iris), hemihypertrophy of the vertebrae, and genitourinary anomalies.

SYMPTOMS

• Abdominal swelling• An abdominal mass you can feel• Abdominal pain• Fever• Blood in the urine

RISK FACTORS• Factors that may increase the risk of Wilms'

tumor include:• Age (Common in young children with an

average age of about 3-4 years old)• Race (Slightly higher in African-american than

white children and lowest among Asian-american children.)

• Having a family history of Wilms' tumor• GENDER (Higher in girls than boys)

• Wilms' tumor occurs more frequently in children with certain abnormalities present at birth, including:

Aniridia Hemihypertrophy

Undescended testicles Hypospadias

Wilms' tumor can occur as part of rare syndromes, including:

• WAGR syndrome. This syndrome includes Wilms' tumor, aniridia, abnormalities of the genitals and urinary system, and mental retardation.

• Denys-Drash syndrome. This syndrome includes Wilms' tumor, kidney disease and male pseudohermaphroditism, in which a boy is born with testicles but may exhibit female characteristics.

• Beckwith-Wiedemann syndrome. Symptoms of this syndrome include abdominal organs that protrude into the base of the umbilical cord, a large tongue (macroglossia) and enlarged internal organs.

Denys-Drash syndrome

Beckwith-Wiedemann

syndrome

STAGES OF WILMS’ TUMOR

• Stage I.– The cancer is found only in one kidney, and generally can be

completely removed with surgery.

• Stage II. – The cancer has spread to the tissues and structures near the affected

kidney, such as fat or blood vessels, but it can still be completely removed by surgery.

• Stage III. – The cancer has spread beyond the kidney area to nearby lymph nodes

or other structures within the abdomen, and it may not be completely removed by surgery.

• Stage IV. – The cancer has spread to distant structures, such

as the lungs, liver, bones or brain.

• Stage V. – Cancer cells are found in both kidneys.

STAGES OF TUMOR

ANATOMY AND PHYSIOLOGY

PATHOPHYSIOLOGY

PREDISPOSING FACTORS:-AGE: 3-5-CONGENITAL ANOMALIES-FAMILIAL DISPOSITION TO CANCER

PRECIPITATING FACTORS:-2ND HAND SMOKING-EXPOSURE TO ASBESTOS OR HEAVY METALS

DESTRUCTION OF STRUCTURE OF

CHROMOSOME 11

IN THE NUMBER OF WT1

UNREGULATED AND SUPPRESSED GROWTH OF CELLS AND TISSUES

HEMIHYPERTHROPYCRYPTHORCHIDISM

CELLULAR MUTATION IN THE KIDNEY

AFFECTS THE RENAL CORTEX

TUMOR PRODUCTION

CORTICAL AND MEDULLARY INFILTRATION AND

PROLIFERATION

SUPPRESSION OF FUNCTION OF

NEPHRONS

WILMS’ TUMOR

DIAGNOSTIC EXAMINATION

• Abdominal untrasound detects the tumor and assesses the status of the opposite kidney.

• Chest X-ray and CT scan may be done to identify matastasis.

• MRI or CT scan of the abdomen may be done to evaluate local spread to lymph nodes.

• 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.

• SURGERY-Surgical removal of kidney tissue is called nephrectomy.

• The various types of nephrectomy include:

• Simple nephrectomy. In this surgery, the surgeon removes the entire kidney. The remaining kidney can increase its capacity and take over the entire job of filtering the blood.

• Partial nephrectomy. This involves removal of the tumor and part of the kidney tissue surrounding it. It's usually performed when the other kidney is damaged or has already been removed.

• Radical nephrectomy. In this type of surgery, doctors remove the kidney and surrounding tissues, including the ureter and adrenal gland. Neighboring lymph nodes also may be removed

CHEMOTHERAPHY• Chemotherapy uses medication to kill cancer

cells throughout the body. This treatment affects rapidly dividing cells; thus, normal cells with fast turnover — such as hair follicles, cells in the gastrointestinal tract and bone marrow, the tissue at the core of bone that manufactures blood cells — are affected as well as cancer cells.

RADIATION THERAPY• Radiation therapy uses X-rays or other sources

of high-energy rays to kill cancer cells. It's usually started within a few days after surgery. If your child is very young, he or she may need a sedative to remain still during treatment.

TREATMENT REGIMENS BY STAGE

• The treatment your child undergoes depends on the stage of the cancer, the type of cancer cell, and the child's age and general health.

• Stage I or II cancer. If the cancer is restricted to the kidney or nearby structures — and the cell type is not aggressive, your child will undergo removal of the 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 spread within 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.

• Stage V cancer. If the tumor cells are in both kidneys — part of the cancer from both kidneys will be removed during surgery and neighboring lymph nodes taken to see if they contain tumor cells. Chemotherapy is used to shrink the remaining tumor. Surgery is repeated to remove as much tumor as possible while leaving functioning kidney tissue.

MEDICAL MANAGEMENT

• If your child is diagnosed with this condition, avoid prodding or pushing on the child's belly area, and use care during bathing and handling to avoid injury to the tumor site.

• The first step in treatment is to stage the tumor. Staging helps doctors determine how far the cancer has spread and to plan for the best treatment. Surgery to remove the tumor is scheduled as soon as possible. Surrounding tissues and organs may also need to be removed if the tumor has spread.

• Radiation therapy and chemotherapy will often be started after surgery, depending on the stage of the tumor.

PHARMACOLOGICAL TREATMENT

• The goal of these is to cure as many children as possible while limiting side effects by giving as little treatment as is necessary.– Dactinomycin– Vincristine– Doxurubicin– Etoposide– Cyclophosphamide– Carboplatin– Mesna

• Complementary methods:

• art therapy or play therapy to reduce stress;• acupuncture to help relieve pain • peppermint tea to relieve nausea. • Some complementary methods are known to

help, while others have not been tested. Some have been proven not to be helpful, and a few have even been found harmful.

NURSING INTERVENTIONS

• PREOPERATIVE CARE

• Report abnormal laboratory values to the surgeon.-Bacteriuria, blood coagulation abnormalities, or other significant abnormal values may affect surgery and postoperative care.

• Discuss operative and postoperative expectations as indicated, including the location of the incision and anticipated tubes, stents, and drains.- Preoperative teaching about postoperative expectations reduces anxiety for the client and family during the early postoperative period.

POSTOPERATIVE CARE

• Frequently assess urine color, amount, and character, noting any hematuria, pyuria, or sediment. Promptly report oliguria or anuria, as well as changes in urine color or clarity.

• Note the placement, status, and drainage from ureteral catheters, stents, nephrostomy tubes, or drains. Label each clearly. Maintain gravity drainage; irrigate only as ordered.

• Support the grieving process and adjustment to the loss of a kidney.

• Provide the following home care instructions for the client and family.

• Teach the importance of protecting the remaining kidney by preventing UTI, renal calculi, and trauma.

• Maintain a fluid intake of 2000 to 2500 mL per day.

• Gradually increase exercise to tolerance, avoiding heavy lifting for a year after surgery.

• Teach care of the incision and any remaining drainage tubes, catheters, or stents.

• Instruct to report signs and symptoms to the physician, including manifestations of UTI (dysuria, frequency, urgency, nocturia, cloudy, malodorous urine) or systemic infection (fever, general malaise, fatigue), redness, swelling, pain, or drainage from the incision or any catheter or drain tube site.

• NURSING DIAGNOSIS

1. Risk for infection r/t surgical incision/ presence of tubes, catheters

• Monitor for the following signs of infection:-Redness, swelling, increased pain, or purulent drainage at incisions, injured sites, exit sites of tubes, drains, or catheters.-Elevated temperature.-Color of respiratory secretions.-Appearance of urine.

• Assess nutritional status, including weight, history of weight loss, and serum albumin.

• Maintain or teach asepsis for dressing changes and wound care, catheter care and handling, and peripheral IV and central venous access management.

• Wash hands and teach other caregivers to wash hands before contact with patient, and between procedures with patient.

• Limit visitors.• Encourage intake of protein- and calorie-rich foods.• Encourage fluid intake of 2000 ml to 3000 ml of

water per day (unless contraindicated).• Encourage coughing and deep breathing; consider

use of incentive spirometer.• Administer or teach use of antimicrobial

(antibiotic) drugs as ordered.• Recommend the use of soft-bristled toothbrushes

and stool softeners to protect mucous membranes.

• CONTINUITY OF CARE

• Teach patient or caregiver to wash hands often, especially after toileting, before meals, and before and after administering self-care.

• Teach patient the importance of avoiding contact with those who have infections, colds, or other things.

• Teach family members and caregivers about protecting susceptible patient from themselves and others with infections or colds.

• Teach patient, family, and caregivers the purpose and proper technique for maintaining isolation.

• 2.Dysfunctional grieving r/t loss of body organ

• Assess stage of grieving being experienced by patient or significant others: denial, anger, bargaining, depression, and acceptance.

• Assess the influence of the following factors on coping: past problem-solving abilities, socioeconomic background, educational preparation, cultural beliefs, and spiritual beliefs.

• Assess whether the patient and significant others differ in their stage of grieving.

• Identify available support systems, such as the following: family, peer support, primary physician, consulting physician, nursing staff, clergy, therapist or counselor, and professional or lay support group.

• Identify potential for pathological grieving response.• Evaluate need for referral to social security representatives, legal

consultants, or support groups.

• Observe nonverbal communication.• Establish rapport with patient and significant others; try to

maintain continuity in care providers. Listen and encourage patient or significant others to verbalize feelings.

• Recognize stages of grief; apply nursing measures aimed at that specific stage.

• Provide safe environment for expression of grief.• Minimize environmental stresses or stimuli. Provide the

mourners with a quiet, private environment with no interruptions.

• Remain with patient throughout difficult times. This may require the presence of the care provider during procedures, difficult discussions, conferences with other family members or other members of the health care team.