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Wilms tumor (Nephroblastoma) Q

  1. Most common abdominal malignancy in children>1 yrs (<1yr neuroblastoma)
  2. Peak age incidence 3-4 yr.
  3. Arise from embryonic nephrogenic tissue from abnormal proliferation of metanephric blastema. Q
  4. Consists of mixture of epithelial and stromal elements (Multicentric) Q
  5. Associated with dilation of short arm chromosome 11.
  6. More prevalent with associated anomalies as WAGR syndrome C wilm’s tumor, anidredia, genitourinary anomalies (Horseshoe kidney and mental retardation)  and hemihypertrophy, neurofibromatosis and back with syndrome.
  7. M.C presentation is painless smoth abdominal lumpQ.
  8. Triad of symptoms will be.
  9. Lump, Fever, Hematuria
  10. Hypertension is also accompanyingQ
  11. Usually U/L but in 5-10% may be B/LQ
  12. Metastasize hematogenously to lung. Q
  13. Prognostic factor is its histology. Q
  14. Staging
    I – Tumour limited to kidney and completely resected
    II- Tumour extends byonds the kidney but still resected completely.
    III – Residual nonhematogenous tumor confirmed to abdomen after resection positive lymph node and massive tumour spillage.
    IV - Hematogenous metastases.
    V – B/L renal involvement
  15. Patient having a predominantly epithelial type
  16. of tumour has favoured history.
  17. Egg shell pattern calcification on x-ray.
  18. USG and IVP helps in diagnosis.
  19. Preoperative biopsy not done. Q
  20. Surgery is choce of therapy (Nephrectomy + lymph node sampling chemo-and radiotherapy in end stage of disease.
  21. Actinomycetin D is drug of choiceQ
  22. Bilateral wilms tumour (10-14%) is treated by partial resection and chemotherapy.
  23. Botryoid willm’s tumour arises from metanephric blastema remnants of renal pelvis and treatment of choice is nephroureterectomy. Q

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