NEPHROBLASTOMA NephroBlastoma (Wilms tumor) referring to Dr.

Max Wilms, the German surgeon (18671918) who first described this kind of tumor. A malignant tumor of the kidney found in children, usually below the age of three and rarely over the age of eight. In some cases it involves both kidneys. The most obvious symptom is an abdominal swelling. Treatment is by nephrectomy followed by chemotherapy. Considerable improvement in the results of treatment has occurred in recent years since the use of cytotoxic drugs, such as dactinomycin and vincristine, as a routine. The number of children that survive at least five years after diagnosis is improving, being currently around 75%. In some children the tumor is associated with an abnormality if chromosome number 13; in these cases other features, such as absence of the iris in the eyes and hemihyperthropy are present. Nephrobastoma is a rare embryonal tumor of the kidney arising from undifferentiated mesoderm and represent 5% to 6% of childhood cancers in the United states. Its incidence remains constant in the United States, with 7.8 c cases per 1 million population ages 1 to 14 years. Approximately 500 children are diagnosed each year in the United States, most common tumor occurring in children. Paternal preconception toxin exposure (hydrocarbons and heavy metals) may be associated with increased risk in offspring. Nephrobastoma is slightly more common in black children than in white children. PATOGENESIS - Nephroblastoma has both sporadic and inherited origins. The sporadic form occurs in children with no known genetic predisposition. Inherited cases, which are relatively rare, are transmitted in an autosomal dominant fashion. Nephroblastoma has been linked to mutation of several tumor suppressor genes (i.e., WT1 mutations). Eighteen percent of children who have nephroblastoma also have a number of congenital anomalies. The anomalies associated with nephroblastoma are aniridia (lack of and iris in th eye), hemi hypertrophy (an asymmetry of the body), and genitourinary malformations (i.e., horseshoe kidneys, hypospadias, urethral duplication, polycystic kidneys). Children with both congenital anomalies and nephroblastoma are more likely to have the inherited bilateral form of the disease. CLINICAL MANIFESTATION - Most nephroblastoma usually present as enlarging asymptomatic abdominal masses before the age of 5 years. Many tumors are actually discovered by the childs parent, who feels or notices an abdominal swelling, usually while dressing or bathing the child. The child appears healthy and thriving. Other presenting complaints including vague abdominal pain (37%), hematuria (18%), and fever (22%). Hypertension also may be present. In 25% to 63 % of cases, there may be excess renin secretion. Nephroblastoma may occur in any part of the kidney and varies greatly in size at the time of diagnosis. The tumor genrally appears as a solitary mass surrounded by a smooth, fibrous external capsule and also may contain cystic or hemorrhagic areas. A pseudocapsule generally separates the tumor from the renal parenchyma.

EVALUATION AND TREATMENT On physical examination, the tumor feels firm, not tender, smooth, and is generally confined to one side of the abdomen. If the tumor is palpable past the midline of the abdomen. If the tumor is palpable past the midline of the abdomen, it may be large or arising from a horseshoe or ectopic kidney, once an abdominal mass is detected, an abdominal ultrasound may be the initial means of study. Abdominal computed tomography (CT) scan of MRI also may be obtained before biopsy and surgical removal of the tumor. Diagnosis is based on surgical biopsy. Additional laboratory and radiologic studies are used to evaluate the presence or absence of metastasis. The most common sites of metastasis are regional lymph nodes and the lings. Metastasis also occurs in the liver, brain, and bone. Several staging system for nephroblastoma have been developed. The most widely accepted system was developed by the National Wilms Tumor Study Group). Primarily treatment is usually surgical exploration and resection of chemotherapy and then surgical resection, survival approaches 90% for localized disease and 70% for metastatic disease. STAGING OF NEPHROBLASTOMA TUMOR Tumor characteristics Tumor limited to the kidney; can be completely resected. Tumor ascending beyond the kidney but is totally resected. Residual nonhematogenous tumor confined to the abdomen Hematogenous metastases to organs such as lung, liver, bone, or brain. Bilateral disease either at diagnosis or later, then staged for each kidney.

Stage I. II. III. IV. V.

 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. Staging if from I (limited to kidney) to IV (matastasis) and stage V, which indicates bilateral involvement (rare). The tumor may metastasize to the lymph nodes, lungs, liver, and brain. Assessment 1. A firm, non-tender mass in the upper quadrant of the abdomen is usually the presenting sign. It may be on either side. 2. Abdominal pain which is related to rapid growth of the tumor. 3. As the tumor enlarges, pressure may cause constipation, vomiting, abdominal distress, anorexia, weight loss and dyspnea. 4. Less common manifestation are hypertension, fever, hematuria, and anemia. 5. Associated anomalies includes aniridia (absence of the iris), hemihypertrophy of the vertebrae, and genitourinary anomalies. Diagnostic Evaluation 1. Abdominal untrasound detects the tumor and assesses the status of the opposite kidney. 2. Chest X-ray and CT scan may be done to identify matastasis. 3. MRI or CT scan of the abdomen may be done to evaluate local spread to lymph nodes. 4. Urine specimens show hematuria; no increase in vanillylmandelic acid and homovanillic acid levels as occurs with neuroblastoma. 5. 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. Pharmacologic Interventions 1. Chemotherapy as initiated postoperatively to achieve maximal killing of tumor cells. Drug combinations include vincristine and doxorubicin or vincristine and actinomycin D, depending on stage. Surgical Intervention 1. Surgery is the gold standard of therapy. Accurate staging by the type of the tumor and the extent of invasiveness is performed. Nursing Interventions Observe the surgical incision for erythema, drainage or separation. Report any of theses changes. Monitor for elevated temperature or sign of infection post-operatively. Monitor I.V. fluid therapy and intake and output carefully, including nasogastric (NG) drainage. Encourage the parents to ask questions and to understand fully the risk and benefits of surgery. Prepare the child for surgery, explain the procedures at the appropriate developmental level. Continue supporting the parents during the postoperative period. Insert NG tube as ordered. Many children require gastric suction postoperatively to prevent distention or vomiting. When bowel sounds have returned, begin administering small amounts of clear fluids. Administer pain control medications as ordered in the immediate postoperative period. Allow the child to participate in the selection of foods.

 As the child recovers, encourage child to eat progressively larger meals. If unable to eat because of radiation and chemotherapy provide I.V. fluids, hyperalimentation, or tube feedings as indicated. Prepare child and family for fatigue during recovery from surgery and with radiation treatments. Plan frequently rest periods between daily activities. Prepare the child and parents for loss of hair associated with chemotherapy and encourage use of hat as desired. Reassure the hair will grow back.

Patients profile Name: R.C Age: 1 Year old Address: Novaliches Q.C Date admitted: April 30, 2011 Admitting Diagnosis: T/C Wilms Tumor History of present illness -

PHYSICAL ASSESSMENT

BODY PART

NORMAL FINDING

ACTUAL FINDING

INTERPRETATION

ABDOMEN

abdominal skin is unblemished, no scars, color is uniform with t he body color, abdomen is rounded with symmetric movements caused by respiration; umbilicus is concave

21 inches size of abdomen Not tender, painful to touch, smooth, kidneys are enlarged

The abdomen is getting bigger due to the of the abdomen due to the enlargement of the kidney.

Medical management Date ordered 4/30 Intravenous fluid PNSS 350 X8 Cbc, adc blood typing, Na + Cl Urine function test (SGPT,SGOT) Creatinine May do CT scan of the whole abdomen with plan while awaiting scent of the creatinine Bilirubin, ALD,IPAG,AFP

D5LMB 350 ml 8 x 3 doses

5/1/11

To secure 1 unit PRBC properly typed and x-matched For transfusion of PRBC 105 CC X 40 ;6 hours a[art x 3 doses properly typed and x matched. RPI, CBC, with ABC IOH,PT,PT1

5/04/11

Treatments: Monitor vs q4 and record pls Monitor at 6 hrs. Secure abdominal scan and as recorded. BMA T3,T4, TSH 5/5 5/6 PRBC 30 cc x 2 hrs.for doses 6 hrs apart properly typed and xmatched.

X ray results

Radiological findings There are haziness in the right inner lung zones the heart is normal in size. Aorta is unremarkable. Both hemidiaphrams costophrenic sulci and visualized bones are intact, soft tissue mass density in the upper abdomen. Impression: consider pneumonia, both inner lung zones soft tissue mass density in the upper abdomen which may be due to enlargement liver.

Report of ultrasound examination April 29, 2011 OPD, EAMMC Hepato biliary and pancreatic sonography; liver is enlargement and nodular. Gall bladder is undistended with normal anechoic lumen. Biliary ducts are not dilated. Pancreas is obscured, remainder is unremarkable. Impression: Granulomatous vs. neoplastic process, liver Nodular cirrhosis also considers.

Coalugation report Componet: Prothrombin: PT % activity INR Control 10.8 208 0.71 13.4 11.9-14.0 secs % Secs Result Normal values SI units

Activated Partial thromboplastine time APTT Control 30.8 27.3 26.31 Secs secs

Tumor markers

474.3 ng/ml

13-8.1 ng/ml

Peripheral blood smear result Smear pleura microcytic hypo chromic red blood cells w/ heavy anisocytosis and poikilocytosis white blood cell is increased in count with the predominance of neutrophils. Platelets are increased .no immature cells seen. Reticulocytocount = 26x10 -3/L NV (5-15 x 10 -3/L Test Name AST ALT Result SI 92 High 39 Unit U/L U/L Normal values 15.00 37.0 30-65 Result conv. 92 39 Unit U/L U/L Normal values 15.00 37.0 65

Test Name Alkaline phosphate Total protein Albumin Globulin A/G ratio Total bilirubin Direct bilirubin Indirect bilirubin

Result 354.9 62.9 31.6 31.3 1.010 w 21.3 high 12.88 high 8.41

Unit U/L g/L g/L g/L umol/L umol/L umol/L

Normal values 136.0 82 50 32 1.6 17.1 5.00 12.1