Wilms tumor

Wilms tumor (also called Wilms' tumor or nephroblastoma) is a type of childhood cancer that starts in the kidneys. It is the most common type of kidney cancer in children. About 9 of 10 kidney cancers in children are Wilms tumors.

1. By, Mr. Abhijit Bhoyar M Sc. Nursing Child Health WILM’S TUMOR

2. INTRODUCTION Wilms ' tumor is a malignant renal tumor and is the most common renal neoplasm in children. It constitutes approximately 6% of all childhood tumors. Wilms '  tumor , also known as nephroblastoma , It is named after Max  Wilms , the German surgeon (1867–1918) who first described it.

3. INCIDENCE Incidence is 7.6 cases per million for children younger than age 15. Seventy-five percent of cases occur before the child is age 5. Most commonly a unilateral disease, but in 5% to 10%, both kidneys are involved

4. CAUSES It's not clear what causes Wilms ' tumor, but in rare cases, heredity may play a role. Cancer begins when cells develop errors in their DNA. The errors allow the cells to grow and divide uncontrollably and to go on living when other cells would die. The accumulating cells form a tumor. In Wilms ' tumor, this process occurs in the kidney cells.

5. RISK FACTORS Factors that may increase the risk of Wilms ' tumor include: African-American race.  In the United States, African-American children have a slightly higher risk of developing Wilms ' tumor than children of other races. Asian-American children appear to have a lower risk than children of other races. Family history of Wilms ' tumor.  If someone in child's family has had Wilms ' tumor, then child has an increased risk of developing the disease.

6. Conti.. Wilms ' tumor occurs more frequently in children with certain abnormalities or syndromes present at birth, including: Aniridia .  In aniridia the iris — the colored portion of the eye — forms only partially or not at all. Hemihypertrophy .   Hemihypertrophy means one side of the body or a part of the body is noticeably larger than the other side. Aniridia

7. Wilms ' tumor can occur as part of rare syndromes, including: WAGR syndrome.  This syndrome includes Wilms ' tumor, aniridia , genital and urinary system abnormalities, and intellectual disabilities. 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.

8. Conti.. Beckwith- Wiedemann syndrome.   Children with this syndrome tend to be significantly larger than average ( macrosomia ). Other signs may include abdominal organs that jut out into the base of the umbilical cord, a large tongue ( macroglossia ), enlarged internal organs and ear abnormalities. There is also an increased risk of tumors, including a type of liver cancer called hepatoblastoma

9. Types of Wilms Tumors There are two kinds of Wilms tumors, divided by how the cells look under a microscope.

10. CONTI.. Favorable histology.  More than 9 out of 10 Wilms tumors fall into this group. It means there isn’t a lot of difference among the cancer cells. Children with this type have a good chance of being cured. Unfavorable or anaplastic histology.  This type has a variety of deformed cancer cells. It can be much harder to cure.

11. 5 Stages of wilm’s tumor

12. Conti..

13. Clinical Manifestation A firm, nontender upper quadrant abdominal mass is usually the presenting sign; it may be on either side. (It is usually observed by the parents.) Abdominal pain, which is related to rapid growth of the tumor, may occur. As the tumor enlarges, pressure may cause constipation, vomiting, abdominal distress, anorexia, weight loss, and dyspnea .

14. Conti.. Less common are hypertension, fever, hematuria , and anemia. Associated anomalies. Hemihypertrophy Aniridia (without the iris) GU anomalies Overgrowth syndromes ( ie , Beckwith- Wiedemann syndrome)

15. Diagnostic evaluation Abdominal ultrasound - to show the tumor and to assess the status of the opposite kidney. Radiography of chest to identify metastases. CBC and peripheral smear - for baseline data. Urinalysis for hematuria .

16. Conti.. Blood chemistries , especially serum electrolytes, uric acid, renal function tests (blood urea nitrogen and creatinine ), and liver function tests ( bilirubin , alanine aminotransferase , aspartate aminotransferase , lactate dehydrogenase [LD], total protein, albumin, and alkaline phosphatase ) may show abnormalities

17. Conti.. Urinary VMA and HVA to distinguish from neuroblastoma . MRI or CT scan of the abdomen to evaluate local spread to lymph nodes or adjacent organs. Real-time ultrasonography . The Vanillylmandelic (VMA) test is primarilly used to detect and rule out tumors called neuroblastoma in children with an abdominal mass or other symptoms suggestive of the disease. Homovanillic acid (HVA) measurement in urine is used for the screening children for catecholamine- secreating tumors such as neuroblastoma

18. Management Surgery If doctor operates, they may do one of these procedures: Partial nephrectomy .  This removes the tumor and some healthy tissue around it. Radical nephrectomy .  This removes the affected kidney, the ureter , the adrenal gland on top of the kidney, and nearby tissue.

19. Conti.. Removal of both kidneys.  In some cases, the doctor will need to take out both kidneys. Child would then need to have dialysis, using a machine to filter waste out of their blood. Once they’re healthy enough, they might have a kidney transplant.

20. Chemotherapy Certain medications can fight or kill cancer cells inside child’s body. Most children who have Wilms tumors will get chemo at some point during treatment. These drugs can also affect healthy cells, leading to side effects including: Hair loss Fatigue Mouth sores

21. Conti.. Loss of appetite Nausea and vomiting Diarrhea or constipation Bruising or bleeding easier than usual Higher chances of infection

22. Radiation Strong radiation can also kill cancer cells. A machine focuses it onto the cancer. Doctors tend to use radiation for tumors that are stage III and above. But it can also have short-term and long-term side effects, including tissue damage, so they’ll use as little as possible.

23. COMPLICATIONS Metastasis to the lungs, lymph nodes, liver, bone, and brain. Complications from radiation therapy include bowel obstruction, hepatic damage, nephritis, sterility in girls, interstitial pneumonia, scoliosis. Cumulative incidence of second malignancy is 1.6% after 15 years with radiation being the greatest risk factor

24. NURSING ASSESSMENT Obtain a history. Ask how tumor was first discovered. Ask whether the child has history of other GU anomalies or whether there is a family history of cancer. Determine whether the child has had hematuria , dysuria , constipation, abdominal pain, decreased appetite, or fever before hospitalization and ask how these were treated.

25. Perform a physical examination that includes: Assessment for associated anomalies: aniridia , hemihypertrophy of the spine, or cryptorchidism . Palpation of lymph nodes for enlargement, tenderness, and mobility. Palpation of the liver and spleen for enlargement.

26. Conti.. Palpation of the abdomen to determine the size and location of the tumor. Auscultation of the lungs to assess breath sounds or abnormality because of spread of tumor. Assess coping, resources, and emotional state of the family.

27. NURSING ALERT Avoid indiscriminate manipulation of the abdomen preoperatively and postoperatively to decrease the danger of metastasis. Because the tumor is soft and highly vascular, seeding may occur with excessive palpation or handling of the child's abdomen

28. NURSING DIAGNOSIS Anxiety of parents related to learning of diagnosis Anxiety of child related to surgery and diagnostic tests Risk for Deficient Fluid Volume postoperatively Acute Pain related to surgery and possible progression of the disease

29. Conti.. Imbalanced Nutrition: Less Than Body Requirements related to anemia, anorexia, nausea, vomiting, and mucosal ulceration secondary to chemotherapy or radiation Disturbed Body Image related to alopecia associated with chemotherapy Activity Intolerance related to fatigue that results from the size of the tumor and treatment Risk for Infection and hemorrhage related to bone marrow suppression caused by chemotherapy

30. Nursing Interventions Be available to the parents when they want to discuss their feelings. Offer kindness, concern, consideration, and sincerity toward the child and parents; be a source of consolation. Contact the family's clergyman or the hospital chaplain Goal - Reducing Parents' Anxiety

31. Conti.. Obtain the services of a social worker, as appropriate, to help the family use appropriate community resources. Offer hope that therapy will be effective and will prolong life. Have parents speak with parents of a child currently on therapy

32. Conti.. Encourage parents to participate in activities of daily living to help them feel a part of their child's care. Assess family dynamics and coping mechanisms and plan interventions accordingly. Help the parents to deal with anticipatory grief.

33. Conti.. Help the parents to deal with other family members, especially siblings and grandparents, and friends. Encourage the parents to discuss concerns about limiting their child's activities, protecting child from infection, disciplining child, and having anxieties about the illness. Facilitate communication with the clinic nurse or clinical specialist who may interact with the child during the entire course of illness

34. Goal-Reducing Child's Anxiety Provide for continuity of care. Encourage family-centered care (see page 1382). Facilitate play activities for the child and use opportunities to communicate through play. Maintain some discipline, placing calm limitations on unacceptable behavior.

35. Conti… Provide appropriate diversional activities. Encourage independence and provide opportunities that allow the child to control his environment. Explain the diagnosis and treatment in age-appropriate terms.

36. Goal - Preventing Fluid Volume Deficit and Other Complications Insert a nasogastric tube as ordered. Many children require gastric suction postoperatively to prevent distention or vomiting. Monitor gastric output accurately and replace it with the appropriate I.V. fluids as ordered. When bowel sounds have returned, begin with small amounts of clear fluids.

37. Conti.. Keep accurate intake and output record. Monitor vital signs as the child's condition warrants and check the surgical dressing frequently for drainage.

38. Goal- Controlling Pain Position the child for comfort. Water beds and bean bag chairs are usually helpful. Administer drugs on a preventive schedule before pain becomes intense. Continuous infusion pumps for opioid administration are used. Manipulate the environment as necessary to increase the child's comfort and to minimize unnecessary exertion.

39. Conti.. Prepare the child for treatment and diagnostic procedures. Use knowledge of growth and development to prepare the child for such procedures as bone marrow aspirations, spinal taps, blood transfusions, and chemotherapy. Provide a means for talking about the experience. Play, storytelling, or role-playing may be helpful. Convey to the child an acceptance of fears and anger.

40. Family Education and Health Maintenance Teach parents that children who have only one kidney should not play rough contact sports, to avoid injuring the remaining kidney. Advise parents to call health care provider if child has a fever of more than 101° F (38.3° C), bleeding, signs of infections, or exposure to chickenpox if the child has not had it.

41. Conti.. Teach measures to prevent infection, such as hand washing and isolation from children with communicable disease. Refer families to resources such as Candle lighters