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Wilms tumor


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renal tumor

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Wilms tumor

  1. 1. Wilms Tumor
  2. 2. • Wilms' tumor or nephroblastoma is malignant tumor of the kidneys that typically occurs in children. Dr. Max Wilms , the German surgeon (1867–1918) first described this kind of tumor. • Wilms tumor is the fifth most common pediatric malignancy (7% of all childhood tumors).
  3. 3. • Incidence is 1 in 10,000, girls are slightly more affected • 70% of cases occur before the child is 5 years of age. • Most commonly unilateral, but in 5% - 10% both kidneys are involved.
  4. 4. • Approx 500 cases are diagnosed in the U.S. annually. • The majority (75%) occurs in otherwise normal children; a minority (25%) is associated with other developmental abnormalities. • It is highly responsive to treatment, with about 90% of patients surviving at least five years.
  5. 5. Etiology • Unknown • Genetic abnormalities WT1 gene; dominant oncogene (at chromosome 11p13), WT2 gene (at chromosome 11p15)
  6. 6. It is believe that tumor begins to grow as a fetus develops in the womb, with some cells that are destined to form into the kidneys malfunctioning and forming a tumor. The exact etiology of the tumor are still being investigated.
  7. 7. • Tumor is exceedingly vascular, soft, mushy, or gelatinous in character. • Wilms tumor has capacity for rapid growth, usually grows to a large size.
  8. 8. • Tumor is usually uniform, well demarcated by a pseudocapsule of compressed renal tissue. • Tumor develops from primitive renal tissue and can have epithelial ( tubules and glomeruli), stromal (fat, skeletal muscles, cartilage) and blastemal elements (An immature material from which cells and tissues develop).
  9. 9. Wilms tumor may be associated with • Hemihypertrophy-one side of body is larger than other • Aniridia (complete loss of iris) and • Genitourinary anomalies.
  10. 10. Pathophysiology • WT1 gene; dominant oncogene (at chromosome 11p13), WT2 gene (at chromosome 11p15) (a tissue-specific gene for renal blastemal cells and glomerular epithelium) • Leads to abnormal proliferation of the metanephric blastemal cells (primitive embryologic cells of the kidney). • promote changes that may lead to the formation of Wilms tumor.
  11. 11. Clinical manifestations • Wilms tumor is diagnosed at a mean age of 3.5 years. • The most common feature is an upper quadrant abdominal mass (firm, nontender)
  12. 12. • Abdominal pain occurs in 30%-40% of cases, related to rapid growth of tumor. • Urethral obstruction due to compression • Constipation, vomiting, abdominal ditress, anorexia, weight loss and dyspnea due to enlargement of tumor.
  13. 13. • Other signs and symptoms of Wilms tumor include hypertension, fever caused by tumor necrosis, hematuria, and anemia.
  14. 14. • The neoplasm metastasize either by direct extension or by bloodstream. They may invade perirenal tissues, lymph nodes, the liver, the diaphragm, abdominal muscles and the lungs. • Invasion of bone and brain are less common.
  15. 15. Diagnostic evaluation Laboratory studies: • CBC with differential for baseline data • Platelet count:Coagulation abnormalities • Urinalysis for hematuria and urine culture • Liver function tests • Renal function tests • Blood chemistry; sr. electrolytes, uric acid
  16. 16. Imaging Studies • Ultrasonography – Initial diagnosis of a renal or abdominal mass, – Possible renal vein or inferior vena cava (IVC) thrombus (Doppler flow study may be helpful in the setting of vascular invasion.) – Information regarding liver and other kidney
  17. 17. • CT scanning of the chest and abdomen – Differential diagnosis of a kidney tumor versus adrenal tumor (neuroblastoma) – Liver metastases – Status of opposite kidney – Lymph node assessment – Status of chest with respect to metastases
  18. 18. • IVP • Chest radiography - As a baseline for pulmonary metastases • Bone scan • Magnetic resonance imaging
  19. 19. Histologic Findings • Favorable histology (90% of cases) is characterized by all 3 histological elements, without any anaplastic features. The cure rate in these cases is close to 90%.
  20. 20. • Unfavorable histology (10% of cases) is characterized by the presence of anaplasia. • Anaplasia is defined as nuclear enlargement, and abnormal mitoses.
  21. 21. If tumor reveals sarcoma type of tumor then • Bone scan • Bone marrow aspirate • Biopsy
  22. 22. Staging and treatment • Staging is determined by combination of imaging studies and pathology findings. • Treatment strategy is determined by the stage.
  23. 23. Stage I (43% of patients) • For stage I Wilms' tumor, 1 or more of the following criteria must be met: • Tumor is limited to the kidney and is completely excised. • The surface of the renal capsule is intact. • The tumor is not ruptured or biopsied (open or needle) prior to removal. • No involvement of extrarenal or renal sinus lymph- vascular spaces • No residual tumor apparent beyond the margins of excision. • Metastasis of tumor to lymph nodes not identified.
  24. 24. Treatment: • Nephrectomy +/- 18 weeks of chemotherapy depending on age of patient and weight of tumor. EG: less than 2 years old and less than 550g only requires Nephrectomy with observation. • Outcome: 98% 4-year survival; 85% 4- year survival if anaplastic
  25. 25. Stage II (23% of patients) For Stage II Wilms' tumor, 1 or more of the following criteria must be met: • Tumor extends beyond the kidney but is completely excised. • No residual tumor apparent at or beyond the margins of excision. • Any of the following conditions may also exist: – Tumor involvement of the blood vessels of the renal and/or outside the renal parenchyma. – The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank. – Extensive tumor involvement of renal soft tissue.
  26. 26. Treatment: • Nephrectomy + abdominal radiation + 24 weeks of chemotherapy • Outcome: 96% 4-year survival; 70% 4- year survival if anaplastic
  27. 27. Stage III (23% of patients) For Stage III Wilms' tumor, 1 or more of the following criteria must be met: • Unresectable primary tumor. • Lymph node metastasis. • Tumor is present at surgical margins. • Tumor spillage involving peritoneal surfaces either before or during surgery.
  28. 28. Treatment: • Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage • Outcome: 95% 4-year survival; 56% 4- year survival if anaplastic
  29. 29. Stage IV (10% of patients) • Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdomenopelvic region.
  30. 30. Treatment: • Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate. • Outcome: 90% 4-year survival; 17% 4- year survival if anaplastic
  31. 31. Stage V (5% of patients) • Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis. • The 4-year survival in 94% patients with stage I or stage II; 76% for with stage III. • Treatment: Individualized therapy based on tumor burden
  32. 32. Management • Surgical ( partial/complete nephrectomy) • Radiation therapy • Chemotherapy
  33. 33. Chemotherapy • Actinomycin D; 0.06 - 0.12 mg/kg, IV • Doxorubicin ( adriamycin); 1.25 – 1.9 mg/ kg • Vincristine; 0.125 – 0.05 mg/kg
  34. 34. • Stage I actinomycin and vincristine for 11- 15 weeks • Stage II actinomycin and vincristine for 15 months • Stage III actinonycin, vincristine and adriamycin for 15 months,
  35. 35. • Stage IV actinomycin, vincristine, and adriamycin for 15 months. If response is slow then cyclophosphamide is added
  36. 36. • Stage V (bilateral wilms tumor) 1. Excision of tumor 2. If not possible, confirmatory biopsies then treated with vincristine and actinomycin D for 3-6 months. If no satisfactory resolution seen after 3 months, radiation is added to both kidneys
  37. 37. • A second look surgery is planned after 6 months of therapy, if not possible chemotherapy may be tried for an additional 9 months
  38. 38. • In totally inoperable cases with no metastatic disease bilateral nephrectomy and renal transplantation may be only viable operation.
  39. 39. Nursing Management