Wilm's Tumor : Pediatric Oncology Oneliners
1. MC malignant tumor of children less than 5 years = Wilm's Tumor.
2. MC malignant tumor of kidney in children = Wilm's Tumor (6-7% of all childhood malign.)
3. U/L >> B/L. But B/L Wilm's more comminly seen in = Familial Wilm's.
4. Origin of Wilm's = Nephrogenic rests.
5. MC manifestation of Wilm's Tumor = Asymptomatic Abdominal Mass (80% cases at presentation) >> Abdominal pain(30%) or Hematuria (10-25%)
6. Acquired von Willebrand disease seen at the time of diagnosis of Wilm's tumor = in 5-10% cases.
7. Clotting factor deficiency seen = factor VII
8. First/most important investigation = Renal USG.
9. Investigation to know tumor extension or pulmonary mets = CT scan
10. B/L renal involvement at the time of diagnosis = Stage V wilm's.
11. Most sensitive imaging modality for IVC invasion= MRI
12. Best characterized WT gene = WT1
13. Syndrome asso. with Wilm's showing WT2 mutation = Beckwith Wiedemann Syndrome.
[Mnemonic : Note it has Two W's => Hence WT2.] [Rest all are WT1 mutation associated]
14. Organomegaly + Macroglossia + Omphalocele + Hemihypertrophy in presence of Wilm's = Beckwith Wiedemann Syndrome.
[Mn : He is a Wide Man with everything large and umbilicus wide open.]
15. Most Important progonostic factors of Wilm's = Histology (presence of anaplasia is a bad marker) > Stage and histology > loss of heterozygosity at 1p and/or 16q.
16. Mental Retardation is a feature in which syndrome asso. with Wilm's = WAGR syndrome.
17. Rx is based on = staging & histology.
Cure rate with modern Rx = 85-90%.
18. Abdominal radiation is used in which stage Wilm's = stage lll.
Stage l & ll Rx = Surgery followed by chemotherapy with vincristine, Actinomycin D, Adriamycin.
Ref : Ghai Essential Pediatrics 8/e.