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Wilm`s tumor

Wilm`s tumor . Also called Nephroblastoma; Kidney tumor Is a type of kidney cancer that occurs in children . Pathophysiology . is characterized by an abnormal proliferation of the metanephric blastoma cells, which are believed to be primitive embryologic cells of the kidney. . Symptoms.

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Wilm`s tumor

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  1. Wilm`s tumor • Also called Nephroblastoma; Kidney tumor • Is a type of kidney cancer that occurs in children

  2. Pathophysiology • is characterized by an abnormal proliferation of the metanephric blastoma cells, which are believed to be primitive embryologic cells of the kidney.

  3. Symptoms • Abdominal pain • Constipation • Fever • General discomfort or uneasiness (malaise) • High blood pressure • Increased growth on only one side of the body • Loss of appetite • Nausea • Swelling in the abdomen (abdominal hernia or mass) • Vomiting • Note: Abnormal urine color may also be associated with this disease.

  4. Exams and Tests • History and physical exam. • Family history of cancer and look for associated birth defects in the child. • A physical examination reveals an abdominal mass. High blood pressure may also be present. • Blood in the urine occurs in less than 25% of children. Tests : • Abdominal ultrasound • Abdominal x-ray • Complete blood count (may show anemia) • Creatinine • CT scan of the abdomen • Intravenous pyelogram • Urinalysis

  5. Staging of Wilm`s tumor • 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 • No residual tumor apparent beyond the margins of excision. Treatment: Nephrectomy +/- 18 weeks of chemotherapy depending on age of patient and weight of tumor. Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic

  6. 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 sinus and/or outside the renal parenchyma. Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy • Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic

  7. Stage III (23% of patients) • For Stage III Wilms' tumor, 1 or more of the following criteria must be met: • Lymph node metastasis. • Residual tumor after surgery. Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage • Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic

  8. Stage IV (10% of patients) • Defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdomenopelvic region. • 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

  9. Stage V (5% of patients) • Defined as bilateral renal involvement at the time of initial diagnosis. • For patients with bilateral involvement, an attempt should be made to stage each side according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy. • Treatment: Individualized therapy based on tumor burden

  10. Differential diagnosis • Hydronephrosis • Multicystic kidney • Lymphoma

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