Malignancy Flashcards
malignant childhood renal tumours
nephroblastoma /Wilms renal cell cancer clear cell sarcoma rhabdoid renal tumour neuroblastoma
benign childhood renal
mesoblastic nephroma
angiomyolipoma
haemangioma
cystic nephroma
DDx of chidlhood abdominal mass
massive HN renal tumour renal cysts bowel duplication neuroblastoma hepatic tumour soft tissue sarcoma
conditions predisposing to Wilms
Denys Drash
WAGR
Beckwith Wiedemann
WAGR
wilms
aniridia
GU malformation
mental retardation
bilateral disease Wilms
5%
peak age presentation
when do wilms present
male vs female wilms
bilateral disease
3-4 years
majority wilms present under age 5 75%
M and F equally affected
5%
neuroblastoma urine test
urine VMA vanillylmandelic acid
staging wilms tumour
1 = completely confined to kidney excised
2 =out of capsule completely excised
3= local invasion, incomplete resection, positive LN, tumour spill not confined to flank
4= metastatic disease, usually lung
5= bilateral
prognosis wilms low risk histology 5 year survival
1/2 = >90 III = 70-80 IV= 60-80% V= 70-80%
poor prognosis histology types
nephroblastoma blastemal type
diffuse anaplasia type
clear cell sarcoma kidney
rhabdoid tumour kidney
survival 5 year bad histology
clear cell sarcoma = 70-80%
rhabdoi 20%
anaplastic wilms 60%
triphasic histology of Wilms
stromal
blastemal
epithelial / tubules
preop chemo regimen
vincristine
actinomycin D
for 4 weeks then repeat imaging preop us doppler and MRI
post op histology risk stratification
low risk stage 1 no further treatment
most other VA for 27 weeks
stage III need RT
High risk add doxorubicin
mesoblastic nephroma
benign tumour under 6 months
homogenous non enhacing solid mass needs just primary nephrectomy
most common tumour neanatal period