Renal tumors Flashcards Preview

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Flashcards in Renal tumors Deck (11):
1

Wilms tumor - % syndromic - associated syndromes and risk

- 10% Overgrowth related: - Beckwith-Wiedemann (WT2), 10% risk - Perlman syndrome - Sotos, 2-3% risk - Simpson-Golahbi-Behmel, 7.5% risk - 9q22 microdeletion (PTCH1 deletion) - isolated hemihyperplasia - CLOVES Non-overgrowth: - WAGR (15% risk), - Denys-DrashFrasier (90% risk) WT1 - Bloom - LiFraumeni -FA - Trisomy 18

2

List a differential diagnosis for renal tumors

Wilms tumor (nephroblastoma) Renal cell carcinoma Rhabdoid tumor of the kidney Clear cell sarcoma of the kidney Nephrobastomatosis Congenital mesoblastic nephroma Renal medullary carcinoma Neuroblastoma

3

RCC predisposition syndromes

VHL (3p26) Tuberous sclerosis Familial RCC Renal medullary carcinoma (sickle cell) Hereditary leiomyomatosis

4

Nephroblastomatosis surveillance

serial US till age 8-10

5

Congenital mesoblastic nephroma translocation

t(12;15)

6

RCC translocation gene

TFE3

7

Rhabdoid tumor mutations and staining

SMARB1/SMARCA4

INI expression is lacking

8

Key points for clear cell sarcoma of kidney

2nd most common peds renal tumor

Propensity to metastasize - brain is most common site

Associated with BCOR mutations

All stages get Doxo and Rads

Very poor outcome for advanced stage

9

Prognostic features for Wilm's tumor

  1.  Age (adolescents do worse)
  2. Stage
  3. Size (<550g in <24m FH Stage 1 can get surgery alone)
  4. Histology - FH vs anaplasia
  5. Molecular - LOH of 1p/16q and gain of 1q

 

10

What determines whole lung rads in Stage IV Wilms?

Response of lung nodules to chemo at 6weeks. - omit if CR

11

Which CMN patients may benefit from chemotherapy?

Stage III, cellular histology, older than 3 months at diagnosis