WILMS TUMOR

Question 1

wilms tumor (nephroblastoma)

Answer 1

cancer of kidneys, typically occur in children as a renal tumor
- MC in children w/no associated genetic syndromes, some children have congenital abnormality

Question 2

Sx

Answer 2

• painless palpable abdominal mass
• loss of appetite
• abd pain
• fever
• n/v
• HEMATURIA
• HTN
• rarely variocele

Question 3

tumor stages
1-4

Answer 3

stage 1- tumor 7 cm
stage 2- tumor >7 cm
stage 3- infiltrating gerota’s fascia and vena cava
stage 4- infiltrating to other organs and LNs

Question 4

patho- nephrogenic rests

Answer 4

nephrogenic rests are precursor lesions—> clusters of embryonic/immature kidney cells that didn’t develop normally during fetal growth and eventually may lead to wilms tumor development

• found in 40% of unilat tumors, >90% bilat tumors

Question 5

2 main types of nephrogenic rests

Answer 5

perilobar v. intralobar
- perilobar: in children <1 yo, inc risk of developing contralat wilms tumor

Question 6

• tumor characteristics and location
• common metastasizes where

Answer 6

• encapsulated and vascularized
• do not cross midline of abdomen
• metastasis usually to lung

Question 7

tumor rupture complications

Answer 7

• risk of bleeding and peritoneal dissemination of tumor
• surgical intervention needed

Question 8

triphasic nephroblastoma- 3 pathologic elements

Answer 8

contains derivatives of metanephric:
- blastema (sheets of small blue round cells)
- mesenchyme (stroma) (fibroblastic)
- epithelium (tubules)

Question 9

imaging
- intial, confirmatory
- which test not usually done immediately

Answer 9

• US to confirm presence of intrarenal mass
• CT MRI for detailed imaging
• confirm Dx with tissue sample
• most cases no Bx done first, bc risk of ca cells spreading during procedure

Question 10

definitive dx

Answer 10

pathological exam of nephrectomy specimen

Question 11

tx

Answer 11

nephrectomy (north america) or chemo followed by nephrectomy (europe)