Wilm's tumour: Oneliners(Very high yield)

Wilm's Tumor : 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 cell 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 prognostic 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.